PULM CCM

What are the most common causes of pleural effusion in the United States? Which one causes COMPLETE OPACIFICATION?

They are congestive heart failure, pneumonia, malignancy, and pulmonary embolism Patients with malignant pleural effusions typically present with dyspnea Malignancy is the most common cause of massive pleural effusions that cause complete opacification of one hemithorax

A 53-year-old woman with a known history of collagen vascular disease has had worsening shortness of breath over the last 2 years. Her diffusing capacity has been stable at 65% predicted, and she has no evidence of airflow obstruction on spirometry. Her total lung capacity has slowly decreased over the last 5 years, from an initial value of 84% predicted to a current value of 55% predicted. Her chest radiograph shows small lung volumes, but no clear evidence of interstitial infiltrates or scarring. Which of the following collagen vascular diseases might explain these findings? Idiopathic pulmonary fibrosis Rheumatoid arthritis Sarcoidosis Scleroderma Systemic lupus erythematosus

"The Shrinking lung syndrome of LUPUS!" occurs in some patients with systemic lupus erythematosus. It is characterized by small lung volumes and relatively normal lung parenchyma. The cause for the progressive decline in lung volumes is not entirely clear, but is thought to be caused by a combination of recurrent pleurisy and possible diaphragmatic weakness.

Both the size and side(s) of a pleural effusion provide clues to its etiology 1. A pleural effusion filling more than half of the hemithorax is likely caused by what? 2. An effusion that is bilateral and with a normal heart is generally caused by what? 2b. An effusion that is bilateral and with an enlarged heart is generally caused by what? 4. Right-sided effusions suggest what? 5. Left-sided effusions?

1. Greater than 1/2 of lung: The 3 T's: Trauma, Tumor, Tuberculosis Hepatic hydrothorax (in patients with cirrhosis and ascites-- RX like ascites--with Diuretics) and Chylothorax (caused by thoracic duct obstruction or disruption) often by Lymphoma 2. Bilateral with Normal Heart: Tumor Connective tissue disease Viral infection and Congestive heart failure (less often) 2b--HF 4. Right-sided effusions suggest: Congestive heart failure (again!) Hepatic hydrothorax and Meigs syndrome (in patients with benign ovarian tumors or fibroids) 4. Left-sided effusions suggest Lousy/bad things outside the lung!: Aortic aneurysm dissection Boerhaave syndrome (esophageal rupture) Pancreatitis Splenic rupture or infarction

DLCO is decreased in what 3 disorders that decrease pulmonary capillary blood volume and DLCO is increased in which other?

1. Interstitial lung diseases 2. Emphysema 3. Pulmonary vascular disease including Pulmonary HTN NOTE: Normal DLCO makes clinically important interstitial lung disease (ILD) unlikely--which is great to use as a rule out Pulmonary Hemorrhage increases DLCO spuriously by measurement method.

Treatment of Pmeumothorax is in part determined by the extent of the pneumothorax. If the pneumothorax involves less than what PERCENT would support conservative care?

15% of the lung volume, administration of 100% oxygen and observation are appropriate. Larger pneumothoraces should be treated with either a chest tube or placement of a catheter to withdraw air.

Pleural Dx Effusion and Air as SPT

16% cancer and many with HF. Lymphatics drain fluid. Get DOE, Cough, Pleuritic CP, Fevers, NS, wtloss. Travel, exposures, PMHx. Dullness and dec BS. Key PE: Distended neck veins, rales, edema. Calf/thigh swelling, cord with DVT/PE infarction with effusion. RV Heave. ascites and inc LN. CXR and US to see if flows and size.

osmolar gap equation (OG)

2 x NA + 1.15 * GLU/18 + BUN/2.8 : calculated osmolality(2). OG = MO - CO : osmolar gap

Lymphangioleiomyomatosis LAM Affects women in their 30s and 40s. Associated with spontaneous pneumothorax and chylous effusions. Chest CT shows cystic disease. Young lady with Lam and swiss sandwhich is Air and Mayo on the side Chronic eosinophilic pneumonia CEP Chest radiograph shows "radiographic negative" heart failure, with peripheral alveolar infiltrates predominating. Other findings may include peripheral blood eosinophilia and eosinophilia on bronchoalveolar lavage. Pulmonary alveolar proteinosis Median age of 39 years, and males predominate among smokers but not in nonsmokers. Diagnosed using bronchoalveolar lavage, which shows proteinaceous material in and around alveolar macrophages. Chest CT shows "crazy paving" pattern.

3 ILDs to learn and remember....

MTX Lung Dx

<5% of all patients Unpredictable to presentation. Variety of presentations on CXR including Fibrosis.

busulfan --> lung toxicity

<8% 30 days to 1 Year post exposure. Variety of presentations. Steroids for Progressive Dx..

Nodule Vs. Mass and what size is 75% likely to be Cancer?

A nodule is defined as a lesion that measures less than 4 cm in diameter. A mass is one that is greater than 4 cm in diameter. MASS lesions defined as greater than 3 cm in diameter have a greater than 75% probability of being malignant. REMEMBER >3cm = >3 Quarters Chance of Cancer

Obliterative Bronchiolitis or Bronchiolitis Obliterans (BO--means NO (markings), and Bad Oder (inhaled toxins) and No air movement (Trapping) COPD PFTs and NO response to Steriods.

A variety of associations, including postinfection (primarily viral); posttransplant (lung or bone marrow); toxin/inhalational exposure; drugs (e.g., penicillamine, gold); and connective tissue diseases. There is also an idiopathic form. Clinical Presentation The primary clinical manifestation is dyspnea. Evaluation and Diagnosis Pulmonary function tests reveal obstruction or air-trapping with high residual volumes like COPD. Chest radiographs may reveal hyperinflation or may be normal. High-resolution computed tomography shows air-trapping and mosaic perfusion accentuated on expiratory images. Lung biopsies demonstrate replacement of small airways by peribronchiolar and submucosal fibrosis (i.e., constrictive bronchiolitis), but not organizing pneumonia. Treatment The response to corticosteroid therapy is generally poor since UNLIKE BOOP which is granuloma based, this is fibrosis based.

a-a gradient

A-a (O2) = (FiO2%/100) * (Patm - 47 mmHg) - (PaCO2/0.8) - PaO2

A previously healthy 38-year-old woman who works in a pet shop presents with a 4-week illness characterized by fever, dry cough, arthralgias, and exertional dyspnea. A chest x-ray (CXR) obtained 2 weeks ago showed peripheral wedge-shaped infiltrates. Treatment for community-acquired pneumonia with a fluoroquinolone has not improved her symptoms. A repeat CXR shows that the previous infiltrates are resolving, but new ones are appearing. A chest computed tomography scan shows that these infiltrates spare the subpleural regions. She has normal vital signs and is afebrile. Physical examination reveals occasional inspiratory squeaks, but these are evanescent. Hematologic and biochemical profiles are normal. You decide to pursue a surgical lung biopsy to further evaluate the etiology of her infiltrates. What is the most likely histology? Plugs of granulation tissue in small airways and alveoli filled with cholesterol-laden macrophages vs. Thickening and obliteration of small airway walls with extension of fibrotic tissue into the interstitial space

ANSWER: BOOP aka COP vs. BO

Acute eosinophilic pneumonia vs Chronic eosinophilic pneumonia

Acute eosinophilic pneumonia (AEP) Acute presentation with infiltrates and hypoxemia with or without peripheral eosinophilia Diagnosis requires eosinophilia in bronchoalveolar lavage (BAL) fluid or from lung tissue Steroid-responsive Chronic eosinophilic pneumonia (CEP) Subacute presentation with constitutional symptoms, cough, dyspnea, and peripheral eosinophilia Chest radiograph with peripheral infiltrates (reverse pulmonary edema) Diagnosis requires BAL eosinophilia (>40% diagnostic) or lung tissue eosinophilia Steroid-responsive Patients may develop severe asthma and require chronic steroid use

A 39-year-old woman with a history of asthma presents to her primary care physician with difficulty walking. Her exam is notable for diffuse expiratory wheezing and difficulty with dorsiflexion of her left ankle. Which of the following diseases is the best explanation for her current presentation? Acute eosinophilic pneumonia Allergic angiitis and granulomatosis Allergic bronchopulmonary aspergillosis Chronic eosinophilic pneumonia Hypereosinophilic syndrome

Allergic angiitis and granulomatosis AAnG (AKA Churg-Strauss syndrome) is a syndrome defined by the constellation of asthma, eosinophilia, and systemic vasculitis. This is a systemic illness with frequent involvement of the lung (asthma with infiltrates), skin, peripheral nervous system (mononeuritis multiplex), heart, and gastrointestinal tract. Acute and chronic eosinophilic pneumonia would not be expected to cause a foot drop. Hypereosinophilic syndrome can cause both lung disease and other organ involvement but mononeuritis multiplex is not a classic finding of the disease. Allergic bronchopulmonary aspergillosis can be characterized by steroid-dependent symptoms, but does not cause neurologic disease.

A 37-year-old African American man presents with gradually worsening cough and dyspnea on exertion. His oxygen saturation is 87% on room air, he has diffuse fine crackles on auscultation of his lungs, and his chest x-ray reveals bilateral hilar adenopathy and diffuse reticulonodular infiltrates. He works as a hired hand on a dairy farm, but previously worked in the shipyards. The most likely etiology of his interstitial infiltrates is most likely which one of these: Hypersensitivity pneumonitis Sarcoidosis Silicosis Viral pneumonia None of the above

Although the potential causes of interstitial lung disease include over 100 processes, and the patterns of infiltrates are not specific in most instances, this patient's B hilar adenopathy is most suggestive of sarcoidosis.

In which of the following disease states would you expect an increased diffusing capacity for carbon monoxide (DLCO)? Emphysema Multiple pulmonary emboli Pulmonary hemorrhage Pulmonary hypertension

Alveolar Hemorrhage raises DLCO: The presence of blood in the alveolar space acts as a "sink" for the carbon monoxide used in the diffusing capacity measurement. The DLCO will be increased. All the other choices are associated with a decreased DLCO, since these disease processes reduce the surface area for gas exchange.

Tips and Treatments of COPD exacerbation

Approximately 50% are associated with lower airway bacterial infection Prescribe Antibiotics if change in sputum volume, color, and associated with dyspnea Bronchodilators by metered-dose inhaler as effective as by nebulizer in compliant patients Systemic corticosteroids shorten duration of exacerbation No benefit of more than 2 weeks of corticosteroids

Initial test of choice for upper extremity DVT evaluation, but sensitivity and specificity 80% Addition of Doppler flow or color adds little to sensitivity

B-mode compression ultrasonography (US) Visualizes noncompressable clot in proximal veins 99% sensitive and specific for SYMPTOMATIC proximal leg DVT Drops to ONLY 30 to 60% sensitive, 99% specific for ASYMPTOMIC proximal leg DVT Cannot use when Casting blocks transponder

Causes of bronchiolitis obliterans (BO) Physical findings: Inspiratory squeaks and diffuse crackles Chest may be quiet and CT CLEAR However: Don't be Tricked: Bronchiolitis obliterans (BO) should not be confused with bronchiolitis obliterans with organizing pneumonia (BOOP; also called cryptogenic organizing pneumonia, or COP), which is a different entity involving plugging of airways with granulation tissue and postobstructive chronic pneumonia. BOOP being granuloma tissue based is often responsive to corticosteroids, whereas BO (fibrotic based) is not..BO means Bad Oder, Bad Others (virus/CTD) and BO means NO (cxr findings)

BO=BAD ODER and BAD OTHERS!! BO Pts Squeek and Crackle! Think Occupational Inhalation of toxic fumes including: Methane Hydrochloric acid Chlorine Ammonia Sulfuric acid Nitric acid (silo filler's disease) Fumes and Acids! Viral infections (e.g., respiratory syncytial virus, adenovirus, and influenza) Connective tissue diseases—rheumatoid arthritis Typical presentation for BO characterized by progressive dyspnea and airflow obstruction with a CLEAR chest CT scan. The "mosaic" attenuation pattern on the expiratory chest CT scan is suggestive but may also be seen in patients with multiple pulmonary emboli, or occasionally in healthy patients on a good expiratory film. The squeaks heard on chest examination are a sign of bronchiolitis. A definitive diagnosis often requires a lung biopsy. The absence of infiltrates suggests that this is not methotrexate hypersensitivity pneumonitis or Pneumocystis jiroveci pneumonia. P. jiroveci is uncommon in patients with rheumatoid arthritis, despite common use of low-dose steroids. Anti-TNF medications can predispose to tuberculosis, but there are no lung infiltrates or fever to suggest this. Kaplan syndrome is the presence of necrobiotic lung nodules in patients with rheumatoid arthritis, most often described in association with occupational dust exposure/coal miners.

Eosinophilic Granulomatosis with Polyangiitis (Formerly Known as Churg-Strauss Syndrome) Classically described as a clinical triad of WHAT?

Basic Information Characterized by necrotizing inflammation within small arteries, veins, and capillaries Classically described as a clinical triad of asthma, hypereosinophilia, and vasculitis Clinical Presentation Typical patient presents with the recent onset of allergy or asthma, followed by development of symptoms and signs of vasculitis Asthma associated with eosinophilic granulomatosis with polyangiitis may improve as the vasculitic phase begins Typical symptoms and signs include: Sinusitis: nondestructive, in contrast to Wegener granulomatosis Wheezing Mononeuritis multiplex: neuropathy involving named motor and sensory nerves, often acute in onset, which manifests as the inability to dorsiflex the foot or to hyperextend the wrist (i.e., footdrop or wristdrop) Cutaneous nodules: necrotizing granuloma over the extensor surfaces of joints Diagnosis and Evaluation In addition to the characteristic clinical features, distinctive diagnostic features include: Fleeting pulmonary infiltrates (30% of cases) Eosinophilia (up to 60,000 eosinophils/mm3) Positive antineutrophil cytoplasmic antibody (ANCA) (50% of cases): These antibodies are against myeloperoxidase (MPO-ANCA), which is associated with a perinuclear staining pattern (p-ANCA) on immunofluorescence Biopsy may help confirm the diagnosis Treatment Usually responds dramatically to glucocorticoids alone Eosinophilia disappears with glucocorticoids; the eosinophilia associated with the idiopathic hypereosinophilic syndromes may be less steroid responsive Cyclophosphamide may be required for refractory cases

Solitary pulmonary nodules are sometimes referred to as a _____. It is a rounded opacity on chest imaging outlined by normal lung and not associated w infiltrate, atelectasis, or adenopathy. What s/s are most likely to be malignant?

Basic Information Pulmonary nodule: A single, well-defined lesion, usually rounded or slightly ovoid, surrounded by normal lung tissue Diameter must be 3 cm or less If diameter greater than 3 cm, process is called a mass, of which 90% are malignant Solitary pulmonary nodules are found in 1 of every 500 chest radiographs Nodular mimics: nipple shadows, skin moles, prominent costochondral junction 60%are benign (most common: healed granulomas secondary to tuberculosis or fungal infection) 40% malignant (most common: bronchogenic carcinoma) Doubling time defined as an increase of 28% in nodule diameter indicates doubling) of 25 to 450 days (1M to 15 Months) suggests a malignant process where as Doubling time of less than 25 days or more than 450 days suggests a benign process

High Res CT Scans (HRCT) Correlate with Histopathology and so does not often need a Bx which has risks

Best for underlying Dx Pattern AIP: Diffuse GG consolidation--chalk on side and rub gently OP: Also GG that migrate, consider CTDx, Inf, Drugs, Idiopathic IPF/UIP: Clinical Dx of Exclusion vs. Pathological Dx...often go together, but not always same. See Honey Comb Changes at periphery with think septal lines NSIP: Variable. NOT Honey Combing though, mainly GG. Resp Bronchiolitis: Central lobular nodules with GG appearance. DIP: Basilar predom. More GG opacities w/ cysts. Hypersensitivity Pneumonitits: HP, CT shows GGO upper lobes predom, with CL micronodules by Inhalation Exposure which are airway centered. Can also be chronic and progresses to fibrosis. Mid and upper lung with UIP. Traction: caused by thicking and Train Tracks Sarcoidosis: Upper lung predom. Most common is MS or Hilar LNs. Which are Upper ("sys" ending upper-HS, Sarcoid, PLH, sis are upper) vs. Lower. Get prone images.

DDX of Bilateral Lower Lobe Infiltrates

Bronchiectasis (look for "tram tracks") Aspiration (usually found in the superior or posterior basilar segments) Dermatomyositis/polymyositis--don't forget CA screening Asbestosis (always associated with crackles on examination) Scleroderma, SLE, Sjögren syndrome Sarcoidosis (pleural thickening and effusions are seldom seen) Early Hamman-Rich syndrome/IPF (pleural disease is uncommon)▪ Rheumatoid arthritis

HRCT

Bronchiectasis is confirmed (or refuted) by this imaging modality to help find underlying causes that can be treated.

A 61-year-old man is diagnosed with lung cancer after a chest x-ray reveals an alveolar infiltrate. This pattern is most consistent with which of the following pulmonary malignancies? Adenocarcinoma Bronchoalveolar cell carcinoma Mesothelioma Sarcoma Small cell carcinoma

Bronchoalveolar cell carcinoma can present as an alveolar filling process, and is often misdiagnosed and mis-treated as an infectious pneumonia. The other entities listed more commonly present as a mass.

Basic Information Most common lethal autosomal recessive disease in whites in the United States Obstruction of exocrine glands by viscous secretion Forty percent may not be diagnosed until adolescence ABPA and asthma are seen more frequently in patients with CF and complicate treatment Clinical Presentation COPD Bronchiectasis Nasal polyps Pancreatic insufficiency Hemoptysis Chronic mucoid Pseudomonas infection Diagnosis Abnormal sweat chloride test (elevated chloride) Genotyping Treatment Management of obstructive lung disease Chest physiotherapy with manual percussion, flutter valves, pneumatic vest Long-term antibiotics, including inhaled antibiotics Nutritional support and replacement of pancreatic enzymes Newer drugs that target the defective CFTR channel have shown promise

CF

A 52-year-old smoker presents with a low-grade fever and worsening cough for 3 days. A chest radiograph reveals a middle lobe infiltrate and a small to moderate right-sided pleural effusion. A thoracentesis yields yellow serous fluid with the following characteristics: glucose 60 mg/dL, protein 3.0 g/dL, and lactate dehydrogenase (LDH) 160 U/L. Serum chemistries show glucose 92 mg/dL, total protein 6.8 g/dL, and LDH 220 U/L. How should this fluid be classified? Exudate Transudate Both an exudate and a transudate Neither an exudate nor a transudate Fluid cannot be classified without additional information EXUDATE: REMEMBER: To be classified as a transudate, ALL three of the following criteria must be met: 1. ratio of pleural fluid protein to serum protein less than 0.5, 2. ratio of pleural fluid LDH to serum LDH less than 0.6, and 3. pleural fluid LDH less than two thirds of the top-normal serum LDH. If any one of these criteria is NOT met for Transudate, THEN the fluid should be classified as an exudate

Chemistries should always be obtained on pleural fluid to determine if it is a transudate or an exudate. Differentiation between these two entities is determined by the levels of protein and LDH in the fluid and in the serum. To be classified as a transudate, all three of the following criteria must be met: ratio of pleural fluid protein to serum protein less than 0.5, ratio of pleural fluid LDH to serum LDH less than 0.6, and pleural fluid LDH less than two thirds of the top-normal serum LDH. If any one of these criteria is not met, the fluid should be classified as an exudate (answer A). For this fluid, the protein ratio is 0.44, but the LDH ratio is 0.73. The upper limit of normal for serum LDH is approximately 250 U/L, so the pleural fluid LDH of 160 U/L is slightly less than two thirds of that number. Based on the LDH ratio, the fluid is classified as an exudate. The pleural fluid glucose is not used to differentiate between transudates and exudates

6 Causes of Refractory Asthma

Chronic exposure to an allergen (e.g., molds), irritant (e.g., air pollution), or sensitizing agent (e.g., toluene di-isocyanate) Use of β-blockers (e.g., timolol eye drops for glaucoma) Use of aspirin-containing drugs for Samter Syndrome patients Mucocutaneous fungal infections Allergic bronchopulmonary aspergillosis Churg-Strauss vasculitis

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) EGwP

Clinical constellation of asthma, eosinophilia, and systemic vasculitis Lung biopsy reveals eosinophilia and extravascular granulomas Asthma is present in more than 80% of patients and radiographic infiltrates are present in more than 90% of patients; 30% have pleural effusions Disease may be unmasked after tapering systemic steroids in an asthmatic This is a systemic illness, with skin (75% of patients) and peripheral nervous system (mononeuritis multiplex in 60% of cases) involvement Mononeuritis multiplex is the most common peripheral nervous system feature The heart and gastrointestinal tract may also be involved The kidneys are rarely involved Usually responsive to corticosteroids

Hypersensitivity reaction to Aspergillus manifesting as worsening asthma Patients with CF are also susceptible to allergic bronchopulmonary aspergillosis (ABPA), and this may be an important factor in rapid clinical deterioration Clinical Presentation when NOT Diagnosed: Chronic steroid-dependent asthma or rapidly progressive CF Recurrent pulmonary infiltrates Brown, black, or green sputum plugs and airway casts expectorated--gross!

Culture of Aspergillus species from sputum Perihilar evanescent oval shadows on chest radiograph from mucoid impactions Marked eosinophilia AND Elevated serum immunoglobulin E (IgE) Positive Aspergillus skin test and serum precipitins Dilation of central airways, "gloved finger" bronchiectasis Major criteria: Asthma, Blood eosinophilia Immediate skin reactivity (IgE-dependent reaction) to Aspergillus antigens Immunoglobulin G (IgG) antibodies (type III reaction) to Aspergillus antigens Transient or fixed pulmonary infiltrates Central bronchiectasis High serum IgE titer (normal level in symptomatic patient excludes ABPA) Minor criteria: Presence of Aspergillus in sputum Expectoration of brown mucous plugs Late-phase skin test reactivity to Aspergillus antigen Treatment Corticosteroids with dose adjusted by IgE levels Oral antifungal agents may help in some cases Check for CF genotype in affected patients

Amiodarone lung fibrosis

Cummulative over time. Variable presentation from GGO to SL Nods to reticular abnormalities. Chalk on Point vs. GGO (chalk onside rubbed lightly) Stop amiodarone>> Steroid for severe cases

DPLD Details HRCT can help Dx STOP SMOKING! Looks for subacute onset and Dx based on HRCT and w/o Bx

DIP: Pure restrictive Dx CTD: When young have high prevalence so include and look for Sns/Symptoms to look for things to treat. RA: Bronchiolitis, OP, Rhurm Nodules, NSIP and UIP same with IPF, but known. PSSclerosis: Lung Dx is Leading cause of death. Often with NSIP. HRCT B LL GGO's w or w/o thicking. Can often be Dx before they develop systemic Dx. Mycophenolate is first line Rx. NSIP: Consult Rheum for Rx.

Alveolar hemorrhage Vs. Shrinking Lung Syndome

Diffuse Alveolar hemorrhage (DAH): presents with Acute fever, dyspnea, cough, with or without hemoptysis associated with Falling hematocrit with new infiltrates Diagnosed by bronchoscopy showing progressively bloody bronchial washings Auto-Immune Mediated and may be seen in the presence or absence of the antiphospholipid antibody syndrome (?DMyositis?) Shrinking lung syndrome of LUPUS!: Restrictive lung disease in the ABSCENCE of parenchymal disease (Clear CXR/CT) Etiology is debated, but possibilities include diaphragmatic weakness, phrenic nerve involvement, or pleural inflammation.

Acute Interstitial Pneumonia (AIP)

Diffuse ground-glass opacity and consolidation with air bronchograms. Linear opacities, honeycombing, and traction bronchiectasis are uncommon.

Hypereosinophilic syndrome ▪ Allergic bronchopulmonary aspergillosis (ABPA)

Defined as more than 1500 eosinophils/mm3 in peripheral blood for 6 months Primary targets include the heart, central nervous system, peripheral nervous system, and skin. The lung is less commonly involved. Treat with corticosteroids or cytotoxic agents (or both) Clinical constellation of asthma, elevated IgE levels, eosinophilia, infiltrates, and mucous plugs Central Diagnosis is made by demonstrating eosinophilia, a positive skin test or antibodies to Aspergillus, an increased total and specific IgE, and recurrent infiltrates on chest film Responsive to corticosteroids and itraconazole as treatment resistant Asthma prior to Dx.

Mounting climbing 11K feet patient found confused and iritable on acetozolamide (but stopped). High HR, Ataxic, Inc RR, SO2 applied. What to do? restart acetazolamide dexamethazone nifedipine sildenifil

Dexamethadone for acute mountain sickness with Cerebral edema/encephalopathy (HACE). >9K ft/3KM. Give Dexa and SO2 immediately and get patient down to sea level.

Idiopathic Interstitial Pneumonias IPF DIP AIP NSIP Define Compare and Contrast Each How to Dx?

Diagnosis based on clinical presentation, high-resolution computed tomography (HRCT), and lung histology AIP, Acute interstitial pneumonitis Only one w/ Acute Onset AKA Hammon Rich Syndrome. DIP, desquamative interstitial pneumonitis- Good response to Steroids IPF, idiopathic pulmonary fibrosis, DOES NOT respond to Steroids and ONLY 1 that does not Fully Recover & Completely Resolve. Most Common, No cause or Cure 2/3 have onset >age 60 Presents with DOE, NPC, Velcro Crackles, Clubbing, RV Failure. Honey Combing on HRCT, Restrictive Lung Dx on PFT's. Only RX is Lung Transplant and newly FDA approved drugs NSIP, nonspecific interstitial pneumonitis--only w/ SubAcute Onset also with Good response to Steroids

Goodpasture Syndrome

Disease affecting the lung and kidney associated with circulating antiglomerular basement membrane antibodies that may be the cause of the process (Fig. 20-3) FIGURE20-3 Immunofluorescence study demonstrating linear staining (immunoglobulin G) of alveolar walls in a patient with Goodpasture syndrome. MPO-ANCA can be positive in 10% to 40% of cases The lung and kidney are both involved in 60% to 80% of cases; in the remainder, the kidney alone is involved Clinical Presentation Hemoptysis, dyspnea, cough, and fatigue are the primary symptoms Fever, chills, and weight loss occur in <25% of patients Bilateral infiltrates are the most common chest radiographic finding Anemia is common Hematuria and proteinuria are seen in 90% of patients Anti-GBM antibodies are seen in 90% of patients Suspect when patient has the constellation of alveolar infiltrates, anemia, and renal disease Linear IgG in biopsy, or anti-GBM antibodies are diagnostic RX: Corticosteroids, cyclophosphamide, plasmapheresis

Risk Factors for Development of Chronic Obstructive Pulmonary Disease

Disease in Smokers Airways reactivity Family history of COPD Childhood lung disease Occupational dust exposures (e.g., silica, cotton dust, grain dust, chemicals) COPD, Chronic obstructive pulmonary disease.

The initial management of cirrhosis-related pleural effusion (and the accompanying ascites) is what?

Diuresis, typically with a combination of a loop diuretic, such as furosemide, and a potassium-sparing diuretic, such as spironolactone is the initial management. Look for a TRANSUDATE with The pleural fluid analysis is consistent with a transudate, as all three of Light criteria must be present: fluid/serum protein ratio less than 0.5, fluid/serum LDH ratio less than 0.6, and absolute fluid LDH less than two thirds of the top-normal serum LDH.

Eosinophilic granuloma (pulmonary Langerhans cell histiocytosis, pulmonary histiocytosis X)

Eosinophilic Granuloma is a Variant of histiocytosis X seen almost exclusively in smoking adults and AKA: Pulmonary Langerhans cell histiocytosis Presents with Cough and dyspnea in two thirds of patients with Smoking history in 90% of patients Radiographic manifestations: progression from diffuse nodules to reticulonodular pattern to diffuse cystic changes in severe disease Spares costophrenic angles NOTE: Pneumothorax is common and May have associated central diabetes insipidus and bone cysts Biopsies reveal CD1a(+), s100+ Langerhans cells on light microscopy with Birbeck granules on electron microscopy Prognosis usually good with smoking cessation

Indications for candidacy for Lung transplantation

FEV1 less than 20% predicted Age younger than 60 to 65 years Sufficient social support

Four components of asthma treatment

Four components of asthma treatment are: 1. Monitor symptoms and pulmonary function 2. Control environmental exposures 3. Educate patients regarding proper treatment and avoidance of triggers 4. Drug treatment

DPLD Diffuse parenchymal lung disease)? 5 major causes

Hypersensitivity: Look for NC Granulomas, R/O Infection. What's the offending Agents? Birds/Down exposure? Bats, Farmers. Hot tub lung: NTMac TB Pneumonitis Radiation Drugs like MTX Exposures-Pneumoconioses CTDx--ask about other systemic symptoms noting lung can present first. Then do testing RF, SCL-70, PM/DM: Key questions about prox muscl weakness. SLE: RA: Can see Honeycombing. SSclx: Ground Glass, no honey combing, chronic aspiration vs. AIDx. Scl-70 with PHTN Monitor Diffusion Capacity over time DLCO

Comparison of Pulmonary-Renal Disorders Granulomatosis with Polyangiitis Microscopic Polyangiitis Goodpasture Syndrome

Granulomatosis with Polyangiitis (GwP--meaning MANY Granulomas and Many Vasculitides)--Ears, nose,throat & Kidney involved in 85% of cases and Plasmapharesis is only indicated for severe pulmonary hemorrhage or cases with positive anti-GBM Microscopic Polyangiitis (MP--meaning very small, but MANY Vasculitides) Upper respiratory tract is involved in only 35% Kidneys are most common organ affected (90%) Cough and dyspnea Pleural effusion, subglottic/bronchial stenoses or endobronchial lesions Can involve skin, joints, eyes, and neurologic system Elevated ESR Abnormal urinalysis MPO-ANCA is most strongly correlated with MPA, but PR3-ANCA can also be positive No granulomas on biopsy; most common lung findings are pulmonary capillaritis (hence the name w/o Granulomas and small vessel inflammation): Rx Same as above Goodpasture Syndrome (GS): Hemoptysis, dyspnea, cough, and fatigue are the primary symptoms Fever, chills, and weight loss occur in <25% of patients Anemia is common Hematuria and proteinuria are seen in 90% of patients Anti-GBM antibodies are seen in 90% of patients Suspect when patient has the constellation of alveolar infiltrates, anemia, and renal disease Linear IgG in biopsy, or anti-GBM antibodies are diagnostic. RX Same as others including Plasmapheresis to get rid of the Antibodies!

Granulomatosis with Polyangiitis (Formerly Known as Wegener Granulomatosis) Basic Information Small- to medium-sized arteries, veins, and capillaries affected Clinical Presentation Typical patient is middle-aged individual with long-standing upper respiratory tract or ear complaints (often lasting months or years), who develops symptoms and signs of a systemic inflammatory illness Symptoms and signs: Skin: palpable purpura and nodules (often over elbows); nodules are identical to eosinophilic granulomatosis with polyangiitis Eye: episcleritis, scleritis, peripheral ulcerative keratitis, uveitis, and orbital pseudotumor Ear: conductive hearing loss caused by serous otitis media or granulomatous inflammation in middle ear; less commonly, sensorineural hearing loss Nose: septal perforation and saddle-nose deformity Saddle-nose deformity in granulomatosis with polyangiitis. Trachea: subglottic stenosis (can present with stridor) Lungs: sodular lesions (with a tendency to cavitate) and pulmonary hemorrhage (Fig. 44-10) Computed tomography scan demonstrating nodular and cavitary pulmonary lesions in granulomatosis with polyangiitis. Cardiac: pericarditis (rare) Kidney: segmental, necrotizing crescentic glomerulonephritis; the glomerulonephritis that occurs in ANCA-associated vasculitis is pauci-immune (i.e., characterized by the deposition of few immunoreactants, such as IgG, IgM, C3, within the kidney) CNS: chronic meningitis and cranial nerve lesions Peripheral nerve: mononeuritis multiplex Extremities: arthralgias or myalgias and frank arthritis (often rheumatoid factor positive) Diagnosis and Evaluation ▪Diagnosis based on three histopathologic hallmarks:

Granulomatous inflammation "Geographic" (extensive) necrosis Vasculitis Majority of cases associated with cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) pattern on immunofluorescence staining usually caused by antibodies to PR-3 10% to 15% of patients with granulomatosis with polyangiitis may demonstrate p-ANCA pattern, typically caused by antibodies to MPO PR-3 and MPO-ANCA never occur in the same patient Despite rigorous testing, 20% of patients with granulomatosis with polyangiitis may be ANCA negative Treatment Systemic glucocorticoids and methotrexate for limited disease; cyclophosphamide for more severe disease Disease appears to accelerate when serum creatinine begins to rise; detection of renal involvement signals medical emergency that must be treated swiftly ANCA serologies useful in making the diagnosis, but serial testing of ANCA titers not useful in predicting disease flares ▪Reflecting dramatic improvements in treatment, there is now 90% survival at 5 years

A 43-year-old man who has smoked a half pack of cigarettes per day since the age of 20 has an insidious onset of dyspnea. His pulmonary function testing shows a forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio of 0.63, an FEV1 40% of predicted, a total lung capacity 140% of predicted, a residual volume 184% of predicted, and a diffusing capacity for carbon monoxide (DLCO) 24% of predicted. A chest computed tomography scan shows predominantly lower lobe emphysema. Which of the following statements is most likely correct? What disease does he probably have based on presenation and CXR?

His oxygen saturation by pulse oximetry falls when he exercises. When the DLCO is less than 50%, about half of patients have exercise desaturation. The diagnosis of exclusion in this case is α1-antitrypsin deficiency, evidenced by the severe emphysema in a young man with a light smoking history and lower lobe predominance (suggesting panacinar pathology)

DPLD with unknown causes

IPF onset 50-70 age no cause is found after 6 months of cough and DOE. Velcro Crackles at bases, clubbing in 50%, Dx hard. HRCT is best test looking for Honey Combing with Nl CXR. If HRCT looks like UIP, then Lung Bx not needed. Mostly peripheral and basilar in location. IPF is progressive with death in 3-5 years. Variable though and many can live >15 years. Manage co morbids. R/o OSA so check PSG. Exercise rehab may help. 2 FDA approve therapies NINTEDANIB and PIRFENIDONE targeting fibroblasts. These do delay progression, but do not cure. Refer early for Lung Transplant. Consensus Guidelines recommend NOT putting pts on Vent under any circumstances since never will come off.

Causes of Pulmonary hypertension Intrinsic vs. Extrinsic

Idiopathic, heritable, left heart disease (e.g. HF), intrinsic lung disease (e.g. COPD), hypoxemic vasoconstriction (e.g. OSA), thromboembolic (e.g. PE)

The differential diagnosis of upper lobe infiltrates is generally small

Inflammatory/CTD the include ONLY Sarcoidosis, Eosinophilic granuloma (EG) Ankylosing spondylitis (AS), Hypersensitivity pneumonitis Infections: Cystic fibrosis. Old tuberculosis or histoplasmosis Exposures: Pneumoconiosis (e.g., silicosis) (NOTE that asbestos is LOWER)

Unusual Causes of Obstructive Lung Disease

Immunoglobulin deficiency with bronchiectasis from IgA and IgG subclasses 2 and 4 deficiency. Immotile cilia syndromes Kartagener syndrome with situs inversus Absence of dynein arms of cilia on electron microscopy Yellow nails syndrome (lymphadema syndrome) with bronchiectasis Pleural effusions, lymphedema, yellow nails Sarcoidosis with upper or lower airway involvement Eosinophilic granuloma (EG) (egg and swiss sandwich) LAM (lam and swiss with Air and Mayo!) Sjögren syndrome HIV with premature emphysema

Positron emission tomography (PET): Role to define metabolic activity of pulmonary nodule

In general, malignant lesions have an increased rate of label uptake compared with benign lesions or normal tissue; however, active infections, granulomatous diseases, and other inflammatory conditions also can have increased uptake (false positives) False negatives can occur with small tumors (<1 cm), tumors with low metabolic activity (e.g., bronchial carcinoid), and hyperglycemia For diagnosis of malignant solitary pulmonary nodules, sensitivity is 97%, and specificity is 82% Role in evaluating solitary pulmonary nodules is still being determined; most useful in borderline cases, with biopsy recommended if PET scan positive

A 43-year-old woman with factor V Leiden is noncompliant with her chronic anticoagulation and presents with a painfully swollen right leg with poor palpable pulses and marked new dyspnea at rest. Her blood pressure is 100/61 mm Hg with a pulse of 115 beats/min and a respiratory rate of 23 breaths/min. Her room air arterial saturation is 85%, which increases to 95% on 4 L of O2 by nasal cannula. A ventilation-perfusion (V/Q) scan shows multiple segmental defects that are ventilated but not perfused. Thrombolytic therapy would be indicated if: Her baseline systolic blood pressure is 150 mm Hg and no further improvement occurs over the next 2 hours Her room air saturation remains less than 90% despite initiation of a fluid bolus and intravenous heparin Leg studies demonstrate a right venous clot extending into the pelvis The summation of all of the perfusion defects is greater than 50% of the total perfusion for both lungs It is discovered that the patient is homozygous for factor V Leiden

Indications for thrombolytic therapy include hemodynamic instability and extensive iliofemoral deep venous thrombosis (DVT). Thrombolytic therapy for extensive DVT has been shown to significantly reduce the postphlebitic syndrome compared with heparin treatment. None of the other clinical features in this patient warrants thrombolytic therapy.

Hypersensitivity pneumonitis 3 Forms Acute after large exposure of antigens quickly w/in 12 hours. PE reveals crackles. HRCT shows, MN and GGO rechallenge confirms Chronic Exposures: Cough, Wt loss and SOB. See SL Thickening and HC changes. GGO's (results from acute inflammation and is reversible) with CNLobules that is more chronic and irreversible Remove exposure. Variable response to Steriods may try and recheck PFT's and symptoms No Immune Mod Treatment

Inhaled antigenic organic dusts: Farmer's lung (moldy hay). Humidifier lung (thermophilic bacteria). Bird-fancier's lung (avian proteins). Acute and chronic forms. Chronic disease findings: Interlobular and intralobular interstitial thickening. Honeycombing, Traction bronchiectasis. May spare costophrenic angles.

Treatment of an acute asthmatic attack

Inhaled short-acting bronchodilators (e.g., albuterol) every 20 minutes for 3 doses Inhaled ipratropium for individuals with severe airflow obstruction failing to improve after repeated short-acting β-agonist treatment Supplemental oxygen if hypoxemia is present Intravenous magnesium sulfate (2 g infused over 20 minutes) can be helpful in individuals with severe attacks If no relief, systemic corticosteroids (e.g., prednisone 60 to 120 mg orally or intravenously and repeat every 6 hours as needed) Systemic bronchodilators (e.g., subcutaneous terbutaline or intravenous aminophylline) may be used in selected cases

A 64-year-old man, who was previously in excellent health and taking no prescription medications, presents with gradually worsening dyspnea on exertion. His chest x-ray reveals bilateral pleural effusions with a cardiothoracic ratio of 17/34 cm (or about 1/2 which is normal). The most likely cause of this presentation is: Congestive heart failure Connective tissue disease Lymphoma Pancreatitis Viral infection

Lymphoma and other malignancies are the most common causes of bilateral pleural effusions in the presence of a normal heart size.

Idiopathic Interstitial Pneumonitis--Don't know what causes, usually patients is older. UIP: BB infiltrates. AIP Acute Interstitial Pneumonia (ARDS like, high mortality) BOOP/CEP: Flu like onset, looks like Infection, but the infiltrates migrate. Steriods work! Sarcoidosis: Variable onset and manifestations. Stage by CXR LN/ILD. Fibrosis is stage 4. Treat based on organ involvement. NC Granulomas. Dx of Exclusion. RARE ONES:

LAM: Women in 30-40's, SPT and Chylous effuciions. Associated with E2 and Progresterone receptors. CEP: Classic XRY: Negative of HF.. Eos in blood and tissue. DX of Exclusions esp Drug induced lung Dx PAP: Dx of youngers M>F, smokers, Dx usingn Bronh/Lavage see protein, looks milky white, Crazy paving, thicking of septa..looks like paving stones on street.

A 72-year-old man with chronic obstructive pulmonary disease (COPD) presents for routine follow-up. He has a 120 pack-year smoking history and quit smoking 4 years ago after hospitalization for respiratory failure. He complains of severe breathlessness despite completion of a pulmonary rehabilitation program. He has daily morning cough and phlegm production. Chest examination shows decreased breath sounds and distant heart sounds. There is no cyanosis, clubbing, or peripheral edema. He has a forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio of 0.43 and an FEV1 32% of predicted. The diffusing capacity for carbon monoxide (DLCO) is 28% of predicted. The total lung capacity is 120% of predicted and the residual volume is 223% of predicted. Arterial blood gases on room air show an arterial partial pressure of oxygen (Pao2) 61 mm Hg, arterial partial pressure of carbon dioxide (PaCo2) 48 mm Hg, and pH 7.41. His maximum exercise capacity on a cycle ergometer is 32% of predicted, which is interpreted as a severe impairment. His body mass index is 23 kg/m2, and he has lost 10 pounds of body weight over the past 3 years. A chest radiograph and chest computed tomography scan show emphysema that is predominantly in the upper zones of the chest. Appropriate therapy might include which of the following options:

Lung volume reduction surgery Lung volume reduction surgery is an appropriate option for patients with severe emphysema (FEV1 <40% of predicted) who are very symptomatic, have low exercise capacity despite rehabilitation, and have emphysema predominantly in the upper lung zones. This patient does not qualify for lung transplantation given his age (> 65 years) and level of lung function (> 25% of predicted FEV1--too good). He does not have sufficient hypoxemia to warrant long-term oxygen, nor does he have enough acute hypercapnia to warrant noninvasive mask ventilation. The low DLCO suggests that this patient might desaturate with exercise--check 6MWT. If testing shows this to be the case, then portable supplemental oxygen may improve exercise capacity.

This is a rare disorder; etiology is unknown Affects ONLY fertile women Excess proliferation of lymphatic smooth muscle--hence the name) Cystic lung disease with increased interstitial markings Associated in some cases with polymorphisms of tuberous sclerosis gene complex Clinical Presentation Presents with progressive dyspnea Repeated hemoptysis may occur Interstitial infiltrates and airflow obstruction May present with pneumothorax or chylothorax (Lam & Swiss with Air and Mayo!) Diagnosis Obstructive pattern on pulmonary function tests AND low DLCO Characteristic "Swiss cheese" appearance on chest CT from multiple cystic spaces Diffuse proliferation of smooth muscle within the airway walls, interstitium, and lymphatics Treatment Sirolimus (rapamycin) prevents disease progression Lung transplantation for severe disease

Lymphangioleiomyomatosis LAM (LAM & Swiss with Air and Mayo Sandwich) Chest CT with diffuse cystic spaces consistent with a "Swiss cheese" appearance is characteristic for LAM: young woman with chronic airflow obstruction. Which other disorder that can present in a nearly identical fashion to LAM? Eosinophilic Granuloma (EG--Egg and Swiss Sandwich). At times, a lung biopsy is needed to distinguish between the two disorders.

Alpha 1 Antitrypsin Phenotypes Which one produces emphysema

M, MS, MZ: All 3 have No increased risk SZ: Mild increased risk ZZ (TOP HIT MAN): Increased risk for emphysema; 10% also develop chronic liver disease Panacinar emphysema is the typical manifestation of ZZ disease Affects entire respiratory lobule uniformly as PAN ACINAR Predominates at lung BASES in contrast to Smoking with affects Upper Segments and produces centrilobular emphysema

Occupational Exposures Concerns by Patients Clinicians should request what?

MSDS or Review Data Sheet as required by OSHA for patient review when concerned about new chemical exposures. Only do CT with specific symptoms or Abn CXR Hair testing not helpful Transfer work areas if find specific issues from known exposures

NSIP

Most common DPLD associated with AID 2 forms: Cellular (better and responds to Rx) and Fibrotic (Poor prog and does not respond). Still only 15% respond. Affects younger people. HRCT shows GGO w/o Honey Combing as in IPF. Associated with SSC, SLE, SS, DM/PMS, and others. Look for AID's. common with AIDS w/o HART.

A 22-year-old college student recently diagnosed with HIV presents to the emergency room with dyspnea. His CD4 count was 185/mm 3 last month ago, and he has been started on highly active antiretroviral therapy (HAART) and trimethoprim-sulfamethoxazole. In triage, his vitals show temperature 98.9º F, pulse 92 beats/min, respirations 22 breaths/min, and O2 saturation 86%. An arterial blood gas analysis reveals a pH of 7.39, a partial pressure of carbon dioxide (Pco2) of 38 mm Hg, an arterial partial pressure of oxygen (PaO2) of 94 mm Hg, and a measured oxygen saturation of 88%. Which of the following medications is the most likely cause of the arterial blood gas readings? Abacavir Didanosine Efavirenz Trimethoprim-sulfamethoxazole

Many of the medications taken by patients with HIV can lead to complications with life-threatening consequences. This patient has a normal PaO2 but a low measured and observed oxygen saturation. These findings are most consistent with an elevated methemoglobin, which can be seen in patients taking trimethoprim-sulfamethoxazole, dapsone, or lidocaine, among others. The diagnosis can be made by sending for a methemoglobin level.

Features of Asbestosis relative to Tobacco Abuse

Mesothelioma risk does not appear to be affected by smoking and sorry (lee's Dad) there is no effective therapy RE: Lung cancer and asbestos The risk of lung cancer in an asbestos-exposed individual is increased fourfold to fivefold in nonsmokers and fiftyfold in smokers, compared with nonsmokers who have not been exposed to asbestos in mining, construction, naval shipyards and automotive services, etc.

Serologic Testing and Lung Bx to work up DPLD

Most appropriate for young and with Sx or FHx Based on H&PE. If you see parenchymal problems, get the LNs esp for Sarcoid workup

Spirometry in COPD vs. 6 Minute Walk Test

Most sensitive measure of disease presence and progression Earliest abnormalities are decreased maximal flow at low lung volumes (seen on flow-volume loops) FEV1/FVC less than 70% is a reasonable threshold for diagnosing obstructive lung disease, but this may be within the normal range in older adult patients and may underdiagnose younger patients FEV1 determines the severity (<30% predicted is very severe) vs 6MWT--most sensitive for reductions and best for following over time decline in functioning

35 y/o with chronic cough with foul sputum with pseudomonas bacteria and negative Cl Sweat Test? What to do as Has Clubbing, rhonchi and wheezes in upper lobes with Bronchietasis.

Negative first test does not r/o CF so need to Repeat Sweat Chloride Testing if there is suspicion for CF. If 2nd negative, then refer to CF center. Many will be resistant to quinolones.

Fleishner Solid Solitary Nodule Follow-up

No (Low risk patient) <6 mm No follow-up 6-8 mm CT at 6-12 months then consider CT at 18-24 months >8 mm Consider CT at 3 months, PET/CT, or tissue sampling Yes (High risk patient) < 6 mm Optional CT at 12 months 6-8 mm CT at 6-12 months then CT at 18-24 months >8 mm Consider CT at 3 months, PET/CT, or tissue sampling Data

Cryptogenic Orgnanizing Pneumonia (COP), used to be BOOP--based on what? vs. BO?

Non specific lung injury AID, Drugs, etc. Dx of Exclusion. People are treated w/ ATBX and for COPD w/o benefit. Infiltrates are migratory on serial imaging. Even Nodules may be dx as cancer. HRCT with GGO and findings of infectious pneumnia. For atypical presentations get LUNG BX. Try Steriods or RX of AIDs.

A 62-year-old man presents with a persistent, nonproductive cough. He is hypertensive and being treated with lisinopril. The medication is replaced with a thiazide diuretic, but his cough persists. His primary care physician obtains a chest radiograph that reveals bilateral upper lung field interstitial infiltrates. Which of the following processes is most likely to be the cause of his Bilateral UPPER lung field infiltrates? Asbestosis Chronic aspiration Eosinophilic granuloma Rheumatoid arthritis Scleroderma

Of the choices listed, eosinophilic granuloma (EG) is the most likely to present with UPPER lobe predominant disease. All of the other processes are more likely to present with bilateral LOWER lobe disease

Severe serotonin syndrome

Onset within 24 hours of initiation or increasing dose, gastrointestinal prodrome Agitation, clonus, ↑ reflexes, rigidity Stop inciting drug RX: Benzodiazepines Cyproheptadine Resolves in 24 hours

Workup of DPLD Onset: Usually SubAcute or Chronic SOB, NP Cough Course: Meds: Current and previous. Exposures most commonly found including hobbies. ROS: Morning Stiffness, Dry eyes, Rash, FHx helps also. PE: Raynauds, SCL, Malar Rash, TSVitis. May be nl early. Velco crackles indicate fibrosis. HF: JVD, PE, Inc S2 to suggest long standing dx impact on R Heart. Check POx resting and exercise for 4% drop. Clubbing supports IPF.

PFTs with DLCO Vast are restrictive Some of Obstructive CXR: Nl does not r/o DPLD.

Indications for hospitalization

Peak flow less than 40% of baseline after 4 to 6 hours of treatment Persistent hypoxemia Hypercapnia: Blood gas pH may normalize if severe attack and patient is becoming fatigued Altered sensorium History of previous near-fatal asthma attacks

A 70-year-old nursing home resident is admitted to the hospital with cough, fever, and confusion. On physical examination, her blood pressure is 110/70 mm Hg, heart rate is 120 beats/min, temperature is 100.9º F, and she has decreased breath sounds with dullness to percussion over the lower third of the left chest. Chest x-ray reveals a moderate left-sided effusion that does not layer on a lateral decubitus film. Thoracentesis yields yellow, nonpurulent fluid with the following features: 16,200 white blood cells (WBCs)/mm3 (70% neutrophils, 12% lymphocytes, 18% monocytes); 2100 red blood cells/mm3; glucose 30 mg/dL; lactate dehydrogenase (LDH) 1100 U/L; protein 4.6 g/dL; and pH 7.16. Gram stain of the fluid shows no organisms. What therapy should be given next? Start clindamycin and ceftazidime; no invasive intervention Start ticarcillin/clavulanate; place chest tube Start cefuroxime; follow with serial thoracenteses Start clindamycin; place chest tube Start clindamycin and ceftazidime; perform pleural biopsy

Place a CT and start Ticar/Clav. This patient has been residing in a nursing home so it is important to cover nosocomial pathogens with either a semisynthetic penicillin derivative or a third-generation cephalosporin. Answers C and D have inadequate antibiotic coverage, so they are incorrect. The pleural fluid chemistries are most consistent with a complicated parapneumonic effusion, with glucose less than 40 mg/dL and very high LDH. Whereas the pH is in the borderline range (7.00 to 7.20), the high number of WBCs with neutrophil predominance and the presence of loculations (fluid does not layer) increase the likelihood that this effusion may ultimately develop into an empyema. Thus chest tube placement is imperative. A pleural biopsy should be performed if there is concern about the possibility of tuberculous pleuritis. This most often presents with a lymphocyte-predominant effusion, though early cases of tuberculous pleuritis can show a neutrophil predominance; however, the WBC count is typically less than 5000/mm3. A bacterial (nonmycobacterial) process is the more urgent concern here, and the fluid must be drained.

Pneumothorax results from the introduction of air into the pleural space What to look for: Tachycardia is the most common finding in pneumothorax. Hyperresonance to percussion, decreased chest wall movement, decreased fremitus, and decreased or absent breath sounds common with large pneumothorax Hypotension, cyanosis, and shift of the trachea to the contralateral side seen with tension pneumothorax How to treat? Administer 100% oxygen and observe patient if pneumothorax is small (<15% of hemithorax or 2cm). This Increases rate of reabsorption of air from pleural space fourfold Recurrence rate is Similar for primary and secondary forms Thirty percent after first episode; more than 50% after second episode which requires Pleurodysis and usually occurs within 2 years after initial pneumothorax Advise patients to avoid scuba diving which could precipitate. After second pneumothorax, recommend intervention to prevent recurrence Chemical pleurodesis through chest tube Check CT to look for Blebs/bullae and If present, blebs/bullae should be surgically resected, with concomitant surgical pleurodesis Pleurodesis agents and techniques are the same as those used for persistent pleural effusions (see Pleural Effusion section)

Pneumothorax May occur spontaneously or result from trauma or iatrogenic causes (e.g., surgery, central venous access placement, thoracentesis) Primary spontaneous pneumothorax occurs when no clinically apparent lung disease is present with Male predominance (6 : 1) Typical patient is a tall, thin male smoker younger than age 40 years. Most patients have radiographically inapparent subpleural bullae (except on CT!). Secondary spontaneous pneumothorax occurs as a result of underlying lung disease (like EG below, PCJ) Obstructive lung diseases associated with secondary spontaneous pneumothorax include asthma, chronic obstructive pulmonary disease, and cystic fibrosis Associated interstitial lung diseases include idiopathic pulmonary fibrosis, eosinophilic granuloma, and lymphangioleiomyomatosis Pneumocystis jiroveci (formerly carinii) pneumonia and Marfan syndrome are also associated with secondary spontaneous pneumothorax

A 43-year-old woman has severe episodic asthma, requiring her to visit the emergency department and receive a course of oral steroids four to five times per year, and she requires oral steroids approximately half the days of the year. She uses high-dose inhaled corticosteroids twice daily. She works in an electronics assembly plant soldering components to integrated circuits. She has gained approximately 30 pounds over the past 5 years, which she attributes to her oral steroids. She has chronic severe frontal headaches for which she takes an analgesic that contains aspirin, butalbital, and caffeine approximately four times per week. She has had sinus drainage surgery with removal of obstructing nasal polyps (clue!), but this has had only minimal effect on her headaches. She has no pets, does not travel to foreign sites, and has never smoked cigarettes. A chest radiograph is normal. Pulmonary function tests show the forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio is 0.68, and her FEV1 is 72% of predicted. The most appropriate treatment for this patient would include:

Prescribe: Daily monitoring of peak expiratory flow, prescription of nasal steroids and a leukotriene receptor antagonist, and discontinuation of the current analgesic medication (ASA/NSAID) The patient has chronic severe asthma, and her history suggests that she has "Samter triad" with asthma, nasal polyps, and sensitivity to aspirin. A first step in treating this patient would be to discontinue the aspirin-containing medication and add a second controller medication, which would most appropriately be a leukotriene receptor antagonist. Monitoring of peak flow to assess for asymptomatic deterioration in lung function and institution of an asthma action plan based on peak flow and clinical symptoms may allow her to avoid emergency department visits. She may also have an element of occupational asthma, and monitoring of twice-daily peak flow will help assess whether she worsens at the end of the day or throughout the course of the workweek.

Scleroderma LDx Basic Information Most-->60% to 100% of patients with scleroderma have ILD at autopsy

Pulmonary hypertension occurs in 5% to 37% of patients and is more common with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. It can be secondary to ILD or to primary vascular involvement. Cyclophosphamide has been shown to decrease the progression of ILD for these patients.

Fleishner Follow-up of Pulmonary Sub solid Nodules

Pure ground glass <6 mm No follow-up ≥6 mm CT at 6-12 months to confirm persistence, then CT every 2 years until 5 years Part solid nodule <6 mm No follow-up ≥6 mm CT at 3-6 months to confirm persistence. If unchanged and solid component remains < 6mm, annual CT should be performed for 5 years

Rheumatoid Arthritis Although rheumatoid arthritis (RA) is more common in females, RA lung disease is more common in males (3 : 1) Symptoms from RA lung involvement precede joint disease in 20% of cases There are Multiple pulmonary manifestations of RA to know

RA Additional lung manifestations include: Rheumatoid (necrobiotic) nodules, which may be single or multiple; may cavitate! Pathology of nodules reveals histiocytic palisades specific for RA (Note Caplan syndrome: rheumatoid nodules in coal miners) Constrictive bronchiolitis (or bronchiolitis obliterans--BO) Dyspnea, dry cough; chest radiograph clear or hyperinflated Obstruction on PFTs that does not respond to bronchodilators Pathology reveals narrowing and eventual replacement of respiratory bronchioles by peribronchiolar and submucosal fibrosis Poor response to therapy Cricoarytenoid arthritis Pain, hoarseness, dyspnea, stridor, obstruction Symptoms in 25% of patients with RA

A 32-year-old man has severe orthopnea. He cannot lie flat for more than a few seconds during the physical examination. When he does lie flat, he has paradoxic inward movement of the abdomen during inspiration. Examined in the upright position, he has clear lung fields, and a normal cardiac exam. He has an estimated jugular venous pressure of 12 cm H2O and peripheral edema. He has proximal muscle weakness, testicular atrophy, and premature baldness. What is the likely pattern on his pulmonary function testing?

Restrictive lung disease and hypercapnia He has Diaphragmatic paralysis presenting with orthopnea and paradoxic abdominal movement. The baldness, proximal muscle weakness, and testicular atrophy suggest a diagnosis of myotonic dystrophy (MD) a neuromuscular disease

Less common causes pleural effusion and look alikes?

Rheumatoid pleurisy may be asymptomatic and occur in the absence of joint disease activity Classic presentation is an older man with subcutaneous rheumatoid nodules and a unilateral pleural effusion Frequently mimics a complicated parapneumonic effusion, with high lactate dehydrogenase (>1000 U/L), low glucose (<40 mg/dL), and low pH (<7.2) Lupus pleuritis is typically PAINFUL and occurs in 15% to 40% of patients with lupus at some point in the disease Small, bilateral effusions commonly seen It is the presenting feature of lupus in 5% of cases Painful Lupus pleuritis may be seen in drug-induced lupus secondary to procainamide, hydralazine, or isoniazid, among others Chylothorax manifests with (typically) unilateral pleural effusion depending upon where Lymphoma interrupts the Thoracic duct which Originates in abdomen as cisterna chyli, enters the thorax with the aorta, runs along the right anterolateral surface of the vertebrae, crosses the midline at the fourth thoracic vertebra T4, continues up along the left anterolateral vertebral surface, and empties into the junction of the left internal jugular and subclavian veins Site of interruption of thoracic duct determines side of pleural effusion (right-"below" sided if below the fourth thoracic vertebra; left-sided if above it) Lymphoma is cause in 75% of cases Patients with asbestos exposure may present with benign asbestos pleural effusion 5 to 15 years after exposure to asbestos Effusions are typically unilateral, persist for a mean of 4 months, and resolve spontaneously From Asbestosis, Patients may go on to develop pleural plaques (20 years after exposure) or mesothelioma (20 to 30 years after exposure)

The following pairs consist of findings on thoracentesis and possible etiologies. In which pair are findings on thoracentesis correctly paired with a possible etiology? Glucose less than 60 mg/dL: pancreatitis pH less than 7.20: rheumatoid pleurisy Red blood cell (RBC) count greater than 100,000: lupus pleuritis Triglycerides greater than 110 mg/dL: cirrhosis

Rheumatoid pleurisy, complicated parapneumonic effusions, esophageal rupture, and malignant pleural effusions may ALL be associated with pleural fluid pH less than 7.20 pH <7.00 is diagnostic of empyema in the proper clinical setting. Pleural fluid with an RBC count greater than 100,000 is usually seen with trauma, malignancy, or pulmonary embolus with infarction, but is not described with lupus pleuritis. A low pleural fluid glucose <60 mg/dL is seen with parapneumonic effusions, empyema, tuberculous effusions-- all 3 infectious, REMEMBER: rheumatoid pleurisy (INFLAM) & malignant effusions, but is not described with pancreatic causes. Triglycerides >110 mg/dL in the pleural fluid, which defines chylothorax, often are seen with REMEMBER: lymphoma or in general interruption of the thoracic duct, but are not described with cirrhosis (answer D).

Idiopathic Pulmonary HTN: IPAH how to w/u and Rx.

Right-heart catheterization should be performed in all patients to determine if there is a positive response to acute administration of a vasodilator (typically nitric oxide) Five percent to 10% of patients are "responders" (PA pressure declines by at least 10 mm Hg to a value less than 40 mm Hg) and should be treated with oral calcium channel blockers "Nonresponders" have an increased risk of death with calcium channel blockers and should receive other therapy (prostacyclin, endothelin receptor antagonist, or phosphodiesterase-5 inhibitor)

Heerfordt syndrome (uveoparotid fever) can Dx WHAT w/o BX?

Sarcoid: Anterior uveitis, parotiditis, fever (uveoparotid fever), and facial nerve palsy

A 27-year-old man with a 3-week history of bilateral ankle pain with lower extremity swelling and erythematous nodules on both legs presents to the emergency department for evaluation. He has been taking cephalexin orally with no improvement in symptoms. A routine chest x-ray reveals right paratracheal and bilateral hilar lymphadenopathy with no parenchymal abnormalities. The most likely diagnosis is:

Sarcoidosis Because the patient has Löfgren syndrome (sarcoidosis with the triad of hilar adenopathy, acute arthritis, and erythema nodosum) and thus has a very good prognosis, it there are no symptoms, it does NOT need to be treated. Although the differential diagnosis includes lymphoma, tuberculosis (answer E), and histoplasmosis (answer A), the presence of bilateral ankle arthritis strongly suggests sarcoidosis in this patient with hilar adenopathy and erythema nodosum.

Pink Puffer VS Blue Bloater

See PIC not Bloater has CB, Younger

A 52-year-old man who has never smoked has been confined to bed for the past year because of severe obesity (body mass index 48 kg/m2). He has hypoxemia secondary to hypoventilation and peripheral edema suggesting cor pulmonale. Which of the following sets of lung volumes are most likely present? Total lung capacity (TLC) 110% of predicted, functional residual capacity (FRC) 120% of predicted, residual volume (RV) 160% of predicted

TLC 85% of predicted, FRC 62% of predicted, RV 94% of predicted Patients with obesity have slight reductions of TLC, but they are often in the normal range. The most striking abnormality in obesity is the disproportionate reduction in end-expiratory lung volume or FRC. Remember that FRC is the balance between the chest wall's tendency to expand outward and the lung's tendency to pull inward. Obesity decreases chest wall compliance and shifts the balance toward a lower end-expiratory volume. The airways at the base of the lung may be closed during normal tidal breathing at lower end-expiratory volumes, which contributes to the poor oxygenation. This patient most likely has obesity hypoventilation syndrome as well.

Trans vs. Exudates

TRANS: HF, Cirrhosis, low albumin, on P.HD, atalectasis, rhinothorax, Pericarditis, Meig's syndrome EXUDATES: PNE, Cancer, PE, Infection and TB, Auto Immune, Asbestosis, CABG, Post MI, Yellow Nail with Chylo, Drugs Check PH, GS cultures cytology, cell counts w/ differential. High Monos/Lymphocytes with TB and Cancer and chronic effusions. High Eos from air or blood in space, also PNE, Drugs and others like EGPA, cancer and parasites. Painful Lupus Pleuritis. pH <7.3 in many including RA/Lupus pleuritis. Insert Chest Tube for pH <7.2. Complicated. Amlyase from Pancreatitis or Eso rupture. TG >110 chylothorax from TD disruption from surgery trauma or cancer.

A 45-year-old woman presents with complaints of fevers and dyspnea worsening over the last few weeks. She is concerned about the mold she has seen in her basement following recent rain storms. Her examination reveals faint crackles, with no clubbing or peripheral edema. Chest x-ray shows patchy infiltrates in the peripheral lung zones. Laboratory tests show an elevated white blood cell count of 18,600/mm3, with a differential of 60% polymorphonuclear leukocytes, 31% lymphocytes, and 9% eosinophils. What is the most likely diagnosis? Allergic bronchopulmonary aspergillus Bronchiolitis obliterans with organizing pneumonia (BOOP) Chronic eosinophilic pneumonia Churg-Strauss syndrome Idiopathic pulmonary fibrosis

The combination of peripheral eosinophilia and peripheral pulmonary infiltrates suggests chronic eosinophilic pneumonia (CEP). Bronchoalveolar lavage with eosinophilia (over 40% of the total cell count) would strongly suggest this diagnosis.

A 67-year-old active smoker presents to his primary care physician with a nonproductive cough for the last 2 months. His physician orders a chest radiograph, which shows a 1.5-cm nodule in his left upper lobe. Which of the following features of a solitary pulmonary nodule is supportive of a malignant diagnosis? Diffuse calcification throughout the nodule Doubling time of 12 months Lack of cavitation "Popcorn" calcification pattern Smooth edge, with well-defined borders

The doubling time of malignant lesions is between 25 and 450 day. Thus, a doubling time of 12 months (365 days) supports a malignant diagnosis. All of the other answers describe features more suggestive of benign lesions.

A 53-year-old man presents with progressive exertional dyspnea. Pulmonary function tests (PFTs) reveal a forced vital capacity (FVC) of 2.84 L (87% of predicted), a forced expiratory volume in 1 second (FEV1) of 1.9 L (41% of predicted), and an FEV1/FVC ratio of 0.42. A chest radiograph is clear. Which of the following diseases is most likely responsible for the PFT abnormalities? Churg-Strauss syndrome Idiopathic pulmonary fibrosis Rheumatoid arthritis Systemic lupus erythematosus Wegener granulomatosis

The pulmonary function tests suggest obstructive lung disease. Of the potential etiologies on the list, only rheumatoid arthritis and Churg-Strauss syndrome cause obstructive disease and PFTs. More than 90% of patients with Churg-Strauss syndrome have an abnormal chest radiograph, so the most likely etiology of this patient's airflow obstruction is bronchiolitis obliterans (BO) caused by rheumatoid arthritis.

A 70-year-old man who collapsed in a nursing home is brought to the emergency department. His temperature is 36.0º C, pulse is 100 beats/min, and blood pressure is initially 75/40 mm Hg. He is obtunded and requires intubation for airway protection. Initial laboratory data show a blood glucose of 800 mg/dL. His initial creatinine kinase is 300 U/L with an MB index of 5%. His urinalysis shows pyuria. A pulmonary artery (PA) catheter is placed in the intensive care unit and the following values are obtained: cardiac index, 2.3 LOW L/min/m2; pulmonary artery occlusion pressure, 6 mm Hg LOW; systemic vascular resistance (SVR) (indexed to body surface area), 3000 mm Hg min/L·m2 HIGH. These readings suggest that his condition is most consistent with: Anaphylaxis Cardiogenic shock Hypovolemic shock Septic shock

These PA catheter readings are consistent with hypovolemic shock with low normal cardiac index, low normal filling pressures, and elevated systemic vascular resistance. In a patient such as this with potential hypovolemia, sepsis, and cardiogenic shock, a PA catheter may be used to differentiate these states. This patient has predominant hypovolemic shock, likely from his hyperosmolar nonketotic state. Despite the presence of a urinary tract infection, the PA catheter readings do not show the high cardiac output and low SVR typical of septic shock (answer D). Cardiogenic shock (answer B) typically has a low cardiac output with high filling pressures and a high SVR. Treatment in this case should include aggressive fluid resuscitation.

The differential diagnosis of nodules includes: The differential diagnosis of masses includes the previously listed possibilities and is expanded to include The differential diagnosis for anterior mediastinal masses ("terrible Ts") deserves particular attention and includes:

Things that Cause Lung Nodules: Infections: Bacterial (including abscesses) Mycobacterial Fungal Parasitic (e.g., human infection with Dirofilaria immitis [dog heartworm]) Granulomata (Inflammation) Rheumatoid nodules, Granulomatosis with polyangiitis (formerly Wegener granulomatosis) Vascular malformations Bronchogenic cysts Hamartoma Primary or secondary lung neoplasms Masses add: sarcomas, fibromas, and progressive massive fibrosis (PMF) Causes of ANTERIOR MEDIASTINAL MASSES: Terrible Ts" Teratoma Thymoma Thymolipoma Thymic carcinoma/carcinoid Thymic cyst Thoracic thyroid Terrible lymphoma

A 47-year-old woman presents with 5 weeks of progressive dyspnea and intermittent fevers, not responsive to 5 days of azithromycin followed by 2 weeks of levofloxacin. Her laboratory findings are normal, except for a slightly elevated erythrocyte sedimentation rate (ESR) and white blood cell (WBC) count. A chest x-ray reveals bilateral patchy alveolar infiltrates. The most likely diagnosis is: Allergic bronchopulmonary aspergillosis (ABPA) Bronchiolitis obliterans with organizing pneumonia (BOOP) Goodpasture syndrome Idiopathic pulmonary fibrosis Rheumatoid arthritis

This case is a typical presentation for BOOP. The duration of symptoms (too short) and chest x-ray findings would be unusual for idiopathic pulmonary fibrosis or rheumatoid arthritis which progress more gradually. ABPA presents with an asthma-like picture, most commonly with fleeting infiltrates on serial chest x-ray. Goodpasture syndrome could present in this fashion, but the absence of hemoptysis and/or renal findings makes this less likely.

A 63-year-old man presents to the emergency room with progressively worsening shortness of breath over the past 3 weeks. He also reports episodic night sweats and unintentional weight loss of 10 pounds over the past 2 months. His past medical history is notable for hypertension, atrial fibrillation, and rheumatoid arthritis. He has been on a stable medication regimen for the past year, including lisinopril, amiodarone, warfarin, and methotrexate. On physical examination, his blood pressure is 135/85 mm Hg, heart rate is 76 beats/min, respiratory rate is 24 breaths/min, and temperature is 99.0º F; there are decreased breath sounds over the lower third of the right hemithorax with associated dullness to percussion and normal heart sounds with a regular rate and rhythm. Ulnar deviation of the fingers is present, but there is no active synovitis. A chest x-ray shows a right-sided pleural effusion, and this is confirmed on a contrast chest computed tomography (CT) scan. The CT scan also reveals bulky mediastinal adenopathy in the subcarinal, precarinal, and right paratracheal regions. No old radiographic studies are available for comparison, but the patient thinks he may have previously been told of "fluid around the lung" several years ago. Thoracentesis is performed and reveals milky-appearing pleural fluid with the following features: 1100 white blood cells (WBCs)/mm3 (20% neutrophils, 70% lymphocytes, 10% monocytes); 110 red blood cells/mm3; glucose 35 mg/dL; protein 4.0 g/dL; lactate dehydrogenase (LDH) 800 U/L; triglycerides 40 mg/dL; and cholesterol 320 mg/dL. Which of the following is the most likely cause of the pleural effusion? Amiodarone-induced pleural effusion Chronic rheumatoid pleurisy Chylothorax secondary to interruption of thoracic duct by non-Hodgkin lymphoma Complicated parapneumonic effusion Methotrexate-induced pleural effusion

This man presents with symptoms and signs suggestive of lymphoma, including weight loss, night sweats, and bulky mediastinal adenopathy. He also has a milky pleural effusion that raises the possibility of a chylothorax. Although lymphoma is the most common cause of a chylothorax, the pleural fluid chemistries actually indicate that this is not a Chylothorax, but a pseudochylothorax caused by an elevated cholesterol level. A chylothorax MUST have an elevated triglyceride level, and if the triglycerides are less than 50 mg/dL, it is definitively ruled out. A pseudochylothorax is usually seen in long-standing effusions with three primary causes: old tuberculosis, chronic rheumatoid pleurisy, and nephrotic syndrome. The cause of this patient's effusion is his rheumatoid arthritis. The lymphocyte predominance is found on the cell count for chronic rheumatoid pleurisy, and the exudative pleural fluid chemistries are consistent as well. In particular, the low glucose and the very high LDH are classic for rheumatoid pleurisy. A complicated parapneumonic effusion shows similar chemistries, but the WBC should be higher, with a neutrophil predominance. Methotrexate and amiodarone can each cause interstitial lung disease. Rare cases of pleural effusion have been reported with each drug, but they do not show high levels of cholesterol.

A 57-year-old woman presents with complaints of persistent cough, which she describes as worse at night and during meals. She has also noticed progressive dyspnea and weakness, and is now having difficulty walking up the 16 steps to her apartment. On examination, she has faint basilar crackles, no clubbing, and no edema. Her labs show a white blood cell count of 8500/mm3, with 65% polymorphonuclear leukocytes, 32% lymphocytes, and 3% eosinophils; hemoglobin of 13.9 g/dL; and creatine kinase (CK) of 380 U/L. Computed tomography shows patchy ground-glass infiltrates, with some bibasilar bronchiectasis. What would be the most likely diagnosis? Churg-Strauss syndrome Goodpasture syndrome Idiopathic pulmonary fibrosis Polymyositis-associated interstitial lung disease (ILD) Rheumatoid arthritis-associated ILD

This patient has increased muscle weakness, elevated CK, dyspnea, and a clinical story of coughing with eating that suggests aspiration, with the basilar bronchiectasis suggesting a component of chronic aspiration. This is concerning for a myositis-related ILD. The antisynthetase antibodies, particularly anti-Jo and anti-PL-12, are associated with this syndrome. Further workup for evidence of myositis might be warranted. Rheumatoid arthritis can be associated with bronchiectasis, but myositis is less common. A lack of eosinophilia or wheezing makes Churg-Strauss syndrome unlikely. Goodpasture syndrome is unlikely without pulmonary hemorrhage or renal disease.

A 36-year-old African American woman comes to the emergency room with leg pain. Examination is remarkable for tender, raised lesions over the anterior tibia bilaterally (clue). Her laboratory test results show mild normocytic anemia. Chest radiograph shows left hilar and right paratracheal fullness (another clue). Subsequent bronchoscopy with transbronchial needle aspiration reveals noncaseating granulomas (the clincher!). The appropriate management of this problem would be: Azathioprine Observation Prednisone Prednisone plus cyclophosphamide Trimethoprim-sulfamethoxazole

This patient has sarcoidosis, with a sarcoidosis type I chest x-ray. The triad of erythema nodosum, hilar lymphadenopathy, and arthralgia is called Lofgren syndrome, and alone can diagnosis and in general has a spontaneous remission rate above 90% to needs no treatment. In the absence of pulmonary symptoms or evidence of other organ involvement, specific therapy is seldom required.

A 33-year-old nonsmoking woman presents with 6 months of worsening dyspnea on exertion and fatigue. She denies any other symptoms and has no prior medical history. On physical examination, she has clear lung fields, a prominent P2, elevated jugular veins, and mild pedal edema. A chest x-ray shows plump pulmonary arteries bilaterally, without any other abnormalities. Which of the following test results is most likely to be found in her diagnostic workup? Echocardiography showing an estimated right ventricular systolic pressure (RVSP) of 20 mm Hg Ventilation-perfusion scanning showing delayed washout on the ventilation images High-resolution chest computed tomography (CT) scan showing patchy ground-glass infiltrates and subpleural honeycombing Pulmonary angiography demonstrating vascular webs and bands in the pulmonary arteries Pulmonary function testing demonstrating normal lung volumes and a severely decreased diffusing capacity

This patient has the signs and symptoms of pulmonary hypertension. Typically, such patients do not present until the mean pulmonary artery (PA) pressure has risen into the moderate to severe range. Answer A is incorrect because an RVSP (i.e., systolic PA pressure) of 20 mm Hg is in the normal range. Delayed washout on a ventilation scan is seen in chronic obstructive pulmonary disease (poorly emptying lung zones), and there is nothing to suggest this as the underlying etiology for this patient's pulmonary hypertension. Similarly, both her pulmonary exam and chest radiograph demonstrate clear lung fields, so one would not expect to find changes consistent with idiopathic pulmonary fibrosis on a chest CT scan. Her most likely diagnosis, given her age and gender, is idiopathic pulmonary arterial hypertension (IPAH), although further workup is needed to confirm this. Vascular webs and bands on pulmonary angiography are seen in chronic thromboembolic pulmonary hypertension, and this would be less likely than IPAH in this patient. E is the best answer, because it describes the typical findings on pulmonary function testing in most patients with pulmonary hypertension. The diffusing capacity is severely reduced in pulmonary hypertension because of pruning of the pulmonary vascular bed and a subsequent decrease in the surface area for gas exchange.

A 31-year-old white woman presents with dyspnea and fever of 4 days' duration. She also describes anterior chest pain that is worse when she is supine (clue to painful pleuritis/pericarditis). Exam is notable for dullness in the left base to percussion of the lungs, with rare crackles bilaterally. Chest radiograph reveals patchy infiltrates bilaterally, with left pleural effusion and enlarged cardiac silhouette. Chemistries and urinalysis are normal, but white blood cell (WBC) and platelet counts are slightly reduced. Which of the following is the most likely diagnosis? Acute lupus pneumonitis Bronchiolitis obliterans with organizing pneumonia (BOOP) Goodpasture syndrome Sarcoidosis Wegener granulomatosis

This patient is presenting with an acute illness that includes fever, infiltrates, pleural effusion, and pericarditis. Of the choices given, the likeliest etiology for this constellation of symptoms is acute lupus pneumonitis. Pleural involvement is uncommon in sarcoidosis , BOOP , and Goodpasture syndrome . Sarcoidosis can present with myocardial involvement, but pericardial disease is less common, and the overall picture is not very suggestive. Pleural effusions can be seen in Wegener granulomatosis, but the suggestion of pericardial disease and normal renal function makes it much less likely.

A 72-year-old former smoker presents to his primary care physician with 6 months of dry cough and progressive dyspnea on exertion. On exam he has mild clubbing (clue) and bilateral lower lobe crackles (clue). He has no active synovitis and no clear rash. His radiograph shows bilateral lower lobe interstitial thickening with peripheral cystic changes (clincher!). Which of the following medications would potentially slow the progression of this man's lung disease? Azathioprine Cyclophosphamide N-acetylcysteine Nintedanib Prednisone

This patient most likely has idiopathic pulmonary fibrosis (IPF). Immunosuppressive drugs such as azathioprine, cyclophosphamide, and prednisone have not been shown to be effective in IPF, and may in fact cause harm. N-acetylcysteine was commonly prescribed for IPF in the past but has fallen out of favor because of lack of clear benefit and the potential for long-term cardiovascular side effects. Nintedanib is a tyrosine kinase inhibitor that was recently approved for use in patients with IPF. It has been shown to potentially slow the progression of IPF, but does not reverse existing fibrosis or lead to clear symptomatic improvement. Pirfenidone is another antifibrotic drug that was recently approved for the treatment of IPF. It has a similar effect on the progression of lung function decline but a different side effect profile from nintedanib.

Simple vs. Complicated P Effusions What is the difference? How to manage?

Thoracentesis for unknown cause or > 1cm thickness to access. Use US Guidance and must drain if pH < 7.2. Other causes include HF, Pneumonia and Ca (Dx and relieve Sx) are most common causes. Do PA/LAT CXR w/ TUS. Bloody--Cancer, PE, Trauma, Pneumonia, Benign asbestos, check HCT if >50% of serum its a hemothorax. Milky check TG level. Yellow/Green: Inflmammation as with RA Pleuracy, check RF with low Glucose. Dark Green Bilirubin. Dark Bown/Black. Old blood vs fungal or Cancer.Purulant Empyema. Check Cultures at bedside. Trans vs. Exudates using Light's Criteria. on RATIO of TP 0.5 and LDH 0.6 or >2/3 ULN for serum and cholesterol >55 ratio to serum >0.3. Never want to miss an exudate. Lytic agents can be used to reduce size of PEffusion and need for surgical referral.

Management of Pneumothorax Sizea and clinical symptoms Management <2 cm or <15% on chest radiograph, minimal symptoms Admit to hospital for observation and supplemental oxygen (PSP may be managed as an outpatient if good access to medical care) >2 cm or >15% on chest radiograph, breathlessness, and chest pain Insertion of a small bore (<14 Fr) thoracostomy tube with connection to a high-volume low pressure suction system Cardiovascular compromise (hypotension, increasing breathlessness) regardless of size Emergent needle decompression followed by thoracostomy tube insertion Note: If persistent air leak (>48 hours) refer to a thoracic surgeon

Thresholds for management of Pneumothorax 2 things to focus on

Centrilobular emphysema tends to affect the upper lung zones FROM WHAT?, although WHAT deficiency presents with panacinar emphysema typically affecting the lower lung zones?.

Tobacco Centilobular/UPPER A1Antitrypsin Def PANLOBULAR/LOWER

A 32-year-old man presents for evaluation of chest discomfort and dyspnea. Symptoms were initially noted this morning and have been present all day. He denies fever or cough and has not had any recent chest trauma. His physical examination is unremarkable except for a pulse of 110 beats/min and respiratory rate of 18 breaths/min. An electrocardiogram shows sinus tachycardia. Chest x-ray shows a 25% pneumothorax of the left lung. What would the appropriate management at this point be? Have the patient return to the clinic in 1 day for follow-up appointment Hospitalize, administer 100% oxygen, and repeat chest x-ray in 6 hours Hospitalize, administer 100% oxygen, and place a chest tube Hospitalize, administer 100% oxygen, place a chest tube, and instill doxycycline

Treatment of pneumothorax is partially dependent on the size of the pneumothorax. Those that are small (i.e., <15%) may be treated with 100% oxygen and observation. Larger pneumothoraces >15% require air removal, via either a catheter or a chest tube. Most patients will require a chest tube. This patient, who had a spontaneous pneumothorax (seen in tall, young men who smoke), will require a chest tube because of the extent of his pneumothorax (25%).

TRIAD OF: Asthma, often requiring systemic corticosteroids for control Nasal polyps Aspirin (or other nonsteroidal antiinflammatory drug) sensitivity

Triad asthma (Samter syndrome) May require leukotriene antagonist for control of symptoms and stopping ASA which can make asthma worse and cause HAs

Granulomatosis with Polyangiitis GwP (Formerly Wegener Granulomatosis) Basic Information 90% of cases have antineutrophil cytoplasmic antibody (ANCA) antibodies

Two forms described Classic disease: involving the upper and lower respiratory tracts and kidney Limited disease: isolated respiratory tract involvement; up to 40% may be negative for ANCA antibodies Characteristic pathology reveals necrotizing granulomatous inflammation and small-vessel vasculitis of the upper and lower respiratory tracts and necrotizing glomerulonephritis in the kidney Majority of cases demonstrate positive PR3-ANCA

Protein C or S deficiency associated with: 1. what condition? 2. medication complication?

VTE and warfarin-induced skin necrosis

A 36-year-old woman presents to your clinic with complaints of acute-onset intermittent dyspnea, and reports that she has heard herself wheeze in association with these episodes. Her dyspnea does not wake her up at night. Her lung exam reveals scattered wheezes. A chest x-ray reveals no evidence of hyperinflation. Pulmonary function tests indicate mild obstructive lung disease without response to bronchodilators. A methacholine challenge test shows no evidence of bronchoconstriction after inhalation of a 25-mg/mL concentration of methacholine--so rules out Asthma. Flow-volume loops indicate variable extrathoracic obstruction. The most likely diagnosis is: Cystic fibrosis Early idiopathic pulmonary fibrosis Tracheal stenosis Vocal cord dysfunction

Vocal cord dysfunction often presents like asthma in terms of symptoms and physical exam and may coexist with asthma. Unlike asthma, vocal cord dysfunction rarely awakens people from sleep, and the onset of symptoms is more sudden. The absence of hyperinflation on a chest x-ray should also raise one's suspicion for an alternative diagnosis to asthma. The normal result of the methacholine challenge test also makes a diagnosis of asthma unlikely in a symptomatic patient. Flow-volume loops demonstrating variable extrathoracic obstruction suggest the diagnosis. The diagnosis can be confirmed with direct laryngoscopy and visualization of abnormal vocal cord movement. Tracheal stenosis typically presents with constant wheezing and dyspnea, rather than the intermittent symptoms associated with vocal cord dysfunction. Although familial forms of pulmonary fibrosis exist, this presentation is too early (usually >60yo) and would be atypical for idiopathic pulmonary fibrosis. The absence of cough, sputum production, and a normal chest radiograph make cystic fibrosis unlikely.

A 50-year-old man has a history of several severe episodes of pneumonia as a child, and the subsequent development of recurrent purulent sputum production. He presents with low-grade fever, increased cough, and sputum production. A chest radiograph reveals increased bilateral lower lung field markings suggestive of "tram tracks." The most likely diagnosis is: Asbestosis Aspiration pneumonia Bronchiectasis Dermatomyositis, with Jo-1-associated lung disease Sarcoidosis

While all of these processes may present with lower lung field changes radiographically, the "tram tracks" appearance is a classic finding seen with bronchiectasis. This patient's recurrent infections as a child likely led to scarring of his lower lobe bronchi which causes the "tram track" appearance. The resulting abnormal airway clearance led to recurrent infections with further airway damage.

Bleomycin Lung Injury

risk with CUM Dose like Amiodarone, risk include increased Age, and other agents. 1-6 months post exposure with rapid onset. High O2 lung exposure makes it worse. Remove drug and steriods for severe symptoms.

Osmolal gap is the difference between

calculated and measured osmolality values

Radiation pneumonitis

causes pulmonary fibrosis and presents with cough, fever, chest fullness, dyspnea, pleuritic chest pain, hemoptysis, new infiltrates even ARDS. CT shows diffuse infiltrates, effusions. HRCT with GGO in field of exposure. Well defined demarcation is pathognomonic, but not always seen. Tx: Steroids for severe Dx. Recoil pneumonitis can occur with other agents including

Silicosis SRLD

fibrotic lung disease from inhaling silica particles. Stone washing even causes it. Fracting with fine sand. 4 Types: 1. Acute Sil Proteinosis after high exposure level within weeks to a few years. Crazy paving pattern on HRCT. DOE, can be fatal BAL reveals milky fluid. 2. Chronic Simple: Low to moderate over 10 years. Upper Zone w/ Central nodules. Hilar LN 3. Chronic Complicated PMF, but get coalescent fibrosis in periphery and enlarges w/ lung contraction. 4. Accelerated Silicosis. 3-10 Yrs. All can get TB, Infection. Once it develops nothing helps. Removal will help prevent further injury. Inc Risk for Cancer esp in smokers.

Laboratory studies indicative of the need for chest tube placement include what values for pH, Glucose, LDH?

pH less than 7.00; glucose less than 40 mg/dL; LDH more than three times ULO normal. Loculated pleural fluid in a patient with an underlying pneumonia also increases the need for chest tube placement, because simple parapneumonic effusions rarely loculate. A sympathetic pleural effusion is caused by disease in a nearby structure (e.g., left-sided pleural effusion in a patient with pancreatitis).

The differential diagnosis of exudative pleural effusions includes what?

parapneumonic effusions, tuberculosis, malignancy, pulmonary embolus, rheumatoid arthritis, Painful lupus pleuritis, asbestosis and chylothorax.

nitrofurantoin lung

presents with SOB acutely with faint lines in older woman whose taken for along time. In chronic Form may be Steroids based on anecdotal data.

Pleurodysis Agents

procedure of causing the adherence of the visceral pleural in the chest with the parietal pleural of the inner chest wall in order to prevent recurrences of a pneumothorax. Talc vs. Doxycycline each with benefits/risks