2 - Restrictive/ Pneumoconioses

How does IPF appear on grossly and grossly?

"Honeycombing" fibrosis throughout lung Dense collagenous fibrosis (normal lung), relatively normal lung, and fibroblastic foci Inflammation w/ fibrotic areas

Other buzzword findings of asbestos?

"ivory white" calcified, supradiaphragmatic, and *pleural plaques*

Occupations of those with silicosis?

(The *silly egg sand*wich I *found* is *mine*!) *Sili*cosis: - *sand*blasters - *found*ries - *mine*s -Stone-cutting -Glass manufacturing

Specific CXR finding of silicosis?

*EGG*SHELL calcification of hilar lymph nodes fits w/ egg sandwich theme

What 2 diseases are associated with heavy silica exposure?

1. Acute silicosis -- proteinaceous fluid in alveoli 2. Accelerated silicosis -- fibrotic complications (sandblasters)

What are 5 examples of diseases that must be ruled out before a Dx of IPF can be made?

1. Collagen vascular disease 2. Drug toxicity (bleomycin & amiodarone) 3. Chronic hypersensitivity pneumonitis 4. Asbestosis 5. Familial idiopathic pulmonary fibrosis

(A minority/The majority) of CWP cases develop into PMF

A minority

A man who has lived in NYC his entire life has an autopsy done following a suspected MI that reveals black pigment in the lungs and hilar lymph nodes. The patient has no Hx of lung disease. What is this?

Anthracosis -- pigment without fibrosis; incidental finding

Carbon pigment without fibrosis

Anthracosis This is carbon pigment in the lung seen in many urban dwellers and tobacco smokers and is usually not symptomatic NOTE: pneumoconioses have fibrotic rxns

Environmental exposures that increase IPF risk?

CIGARETTE smoking Gastric reflux Occupational hazarding inhaling things NOTE: occupational due to metal fumes, wood dusts, farming, hair dressing, stone-polishing

Which form of asbestos is most often encountered in the US? Occupations that acquire this?

Chrysotile (curly and flexible) -more common NOTE: amphibole is much more pathogenic than chrysotile b/c gets stuck (shipbuilding, building trades, mechanics)

What is the diagnosis of pt w/ macules accumulation of carbon pigment in wall of resp bronchioles w/ fibrosis?

Coal workers pneumoconioses (simple) NOTE: not much pulm dysfunction but can see some, but can have focal emphysema due to traction 1-2 mm

What are the different kinds of pneumoconioses?

Coal workers' pneumoconiosis Silicosis Asbestosis

Clinical conditions associated w/ NSIP

Collagen vascular disease HS pneumonitis Drug-induced pneumonitis Infection Immunodeficiency including HIV infection NOTE: remember this is a disease of exclusion

Restrictive lung disease characterized by polyploid plugs of loose organizing connective tissue w/i alveolar ducts, alveoli, and bronchioles?

Cryptogenic Organizing Pneumonia (COP) (has NO honeycomb, interstitial fibrosis, or temporal heterogeneity) The swirls are the interalveolar plugs

What three things determines the amount of dust retained in the lungs?

Dose Duration of exposure Effectiveness of clearance mechanism (like smoking)

What are some sign and symptoms of diffuse interstitial disease?

End-inspiratory crackles NO WHEEZING; that is associated w/ struggling getting air out Decreased CO diffusing capacity Dyspnea Tachypnea NOTE: "IDK why i bother w/ this slide, they're pretty much all the same in lung diseases"

T or F? Mesothelioma is the most common cancer resulting from asbestos infection.

FALSE Bronchogenic carcinoma is most common!! The most common cause of mesothelioma is asbestos though

True or False IPF can be due to asbestosis

FALSE It is a Dx of exclusion so it is not the Dx if it is secondary to something else

Describe PFT changes in a restrictive lung disease

FEV1 decreases a little, FVC decreases even more -- ratio FEV1/FVC stays the same or increased

What bodies are associated with asbestos exposure?

Ferruginous bodies (golden-brown fusiform rods resembling dumbbells) Or can be blue if stained for Fe

(Aged/Freshly fractured) quartz has more free radicals on the surface and is more toxic

Freshly fractured

What is a CXR buzzword for interstitial lung disease?

Ground glass shadow, irregular lines, infiltration by small nodules

Temporal heterogeneity of fibrosis is seen with _____ whereas homogeneity is more common to _____. (use pathological terms)

Heterogeneity: usual interstitial pneumonia Homogeneity: non-specific interstitial pneumonia (or DAD)

Specific findings for idiopathic pulm fibrosis?

Honeycomb appearance due to increased collagen deposition from repeated lung damage/healing (fancy ppl eat honeycomb) Digital clubbing

Difference between IPF/NSIP and COP

IPF & NSIP are in interstitial space COP is in alveolar (therefore no honeycombing)

Form of restrictive lung disease that is a Dx of exclusion?

Idiopathic pulmonary fibrosis (IPF)

Where can the pattern of COP be seen?

In post infectious/inflammatory diseases -Viral and bacterial pneumonias -Inhaled toxins -Collagen-vascular diseases -GvH disease in bone marrow transplant

What coats asbestos fibers in ferruginous bodies?

Iron (iron prussain blue stain)

What morphology of asbestos fiber is most pathogenic?

Long and thin AMPHIBOLE (can go deeper into the lungs and penetrate epithelial cells) These are straight & stiff and get stuck in lungs

Where in the lung will you most commonly find asbestosis?

Lower lobes NOTE: contrast with CWP and silicosis (upper lung) ("Asbestos from the roof, but affects the base (lower lobes). Silica and coal from the base (earth), but affect the roof.")

How are patients with IPF treated?

Lung transplantation Mean survival without it is 3 years or less

A common genetic variation in IPF is associated with increased secretion of _____

MUC5B

Pathogenesis of CWP?

Macrophages pickup carbon particles --> inflammation & fibrosis (macrophage cages w/ coal dust)

Which lobes are most commonly affected in coal workers' pneumoconiosis?

More upper lobes (upper parts stick up)

NSIP Pt with a (cellular/fibrosing) pattern have a better prognosis than those with a (cellular/fibrosing) pattern

NSIP Pt with a CELLULAR pattern have a better prognosis than those with a FIBROSING pattern NOTE: BOTH have a better prognosis that Pt with UIP (cellular pattern is generally younger than fibrosing) NOTE: NSIP = nonspecific interstitial pneumonia

Which restrictive lung disease shows cellular and fibrosing patterns without temporal heterogeneity?

Nonspecific interstitial pneumonia (NSIP) (disease of exclusion)

IPF clinical course?

Not good... Insidious onset of increasing SOB on exertion + dry cough Hypoxemia, cyanosis, & late clubbing Mean survival 3 years or less

How is Cryptogenic Organizing Pneumonia (COP) treated?

Oral steroids (6+mo) or spontaneous recovery

What is the PAINT mnemonic for the different types of restrictive lung disease? Be thorough.

P: pleural A: alveolar I: interstitial N: neuromuscular T: thoracic/extrathoracic

What are the 6 disease processes linked to asbestos exposure?

Parenchymal interstitial fibrosis Localized pleural plaques Recurrent pleural effusions Lung cancer Malignant pleural and peritoneal mesotheliomas Laryngeal cancer

What is the most important factor that determines whether a particle will cause pneumoconiosis?

Particle size (1-5 μM in diameter the worst)

What is the pathologic and European name of IPF?

Pathologic: Usual interstitial pneumonia (UIP) European: cryptogenic fibrosing alveolitis

UIP histologically

Peripheral accentuation

Term for occupational lung diseases

Pneumoconioses (organic or inorganic particulates, fibrous reaction in lungs)

What is the best method of care for pneumoconiosis?

Prevention (engineering controls and personal protection) NOTE: once you get it, you can't get rid of it

If a patient has coal workers' pneumoconiosis for years, what can it develop into?

Progressive massive fibrosis (PMF) --- intensive fibrosis See intensely black scars w/ significant lung compromise 1-2cm+

What can PMF result in?

Pulm HTN and cor pulmonale NOTE: CWP can increase incidence of chronic bronchitis and emphysema

What plays a central role in pathogenesis of lung injury by promoting inflamm and producing ROS and cytokines?

Pulmonary alveolar macrophages

How is pneumoconiosis diagnosed (3 ways)?

Radiographic - B readings PFTs Pathologic (Bx or Autopsy)

Where can we be exposed to asbestos?

Shipbuilding (used as insulation) Building trades (installation and removal of insulation) Mechanics (brakes linings) Asbestos mining and milling

Concentric, hyalinized nodules in the lung and lymph nodes containing shiny, birefringent particle points to which pneumoconiosis?

Silicosis

What is the most common pneumoconioses in the world? What is the culprit?

Silicosis Crystalline silica

What are 3 examples of collagen vascular diseases that have pulmonary involvement and thus can exhibit fibrosis?

Systemic sclerosis (NSIP pattern) Lupus (parenchymal infiltrates) RA (abnormal PFT; chronic pleuritis, intrapulm rheumatoid nodules, pulm HTN) (all have better prognosis than IPF)

Germline loss-of-function mutations in the _____ and _____ genes are seen in some cases of IPF

TERT; TERC Telomerase mutations and defects

True or False Asbestos can also have honeycombing of sorts

TRUE Asbestos can also have honeycombing of sorts, but is not a buzzword for your purposes, hear honeycomb, go with IPF

What is seen in the pleura @asbestosis?

Thickening and plaques

Individuals with silicosis have an increased risk of what infectious disease?

Tuberculosis (also increased risk of lung cancer)

Which part of the lung are you more likely to find silicosis?

Upper lobes (fibrotic nodules) Slowly progressive, nodular, fibrosing lymph nodes also affected

FA: Pathogenesis of silicosis?

macrophages respond to silic & release fibrogenic factors --> fibrosis phagolysosomes are also disrupted --> increased risk of TB increased risk of cancer, cor pulm, and Caplan syndrome (caplan also assoc w/ CWP)