M4F2 CT disorders
Arthralgias and non-erosive arthritis PIPs and MCPs are the most commonly symptomatic, followed by knees and wrists.
MSS Symptoms of SLE: one of the MC clinical features.
no underlying disorder, symmetrical
Raynaud's Phenomenon (primary disease)
Calcium channel blockers
Treatment for Raynaud's Phenomenon (first line)
corticosteroids
treat pleuritis with
Raynaud's Phenomenon
Abrupt onset of triphasic color response. 1. White: vasoconstriction or spasm of digital arteries 2. Blue: blood in capillaries becomes deoxygenated producing a cyanotic appearance. 3. Red: on rewarming, blood flow is increased producing reactive hyperemia
Systemic Lupus Erythematosus (SLE)
Autoimmune disease that affects multiple organ systems, including skin, joints, kidney, CNS, CVS, Serosal membranes, hematologic system, and immune system.
Calcium Salts Raynauds Esophageal Sclerodactyly Telengactias
CREST assoc with Limited Cutaneous Systemic Sclerosis
PERICARDITIS - Substernal CP, relieved by leaning forward - Responds to corticosteroids. - May hear pericardial rub on auscultation.
CV Symptoms of SLE: MC cardiac manifestation:
FATIGUE, headaches, weight loss, and fevers are common along with generalized arthralgias, myalgias, and lymphadenopathy. Most patients have spontaneous flares and remissions. Careful monitoring for major organ disease is important because it can occur at anytime. → should send pt to rheumatologist!
Constitutional Symptoms of SLE
waxing and waning, but can demonstrate a chronic pattern.
Course of Systemic Lupus Erythematosus (SLE)
Malar rash (butterfly rash) Discoid Skin Lesions→ may be associated with alopecia. Not always round in shape. Lupus Panniculitis Mucosal Ulcerations: of buccal mucosa and upper palate Arteriolar spasms or infarcts in nail folds Livedo Reticularis Petechial-purpuric or urticarial lesions on the extremities. Exacerbated by sun exposure.
Cutaneous Symptoms of SLE
Careful history and physical exam*** ⇒ ask about drugs (SHP, INH, minocycline) and Family Hx (strong genetic component)
Diagnosis of SLE
Gi manifestations are uncommon Vasculitis of GI tract can result in bowel necrosis and post prandial pain (increased blood flow to gut during eating) Peritonitis: peritoneal effusion and abdominal pain. Pancreatitis due to vascular pathology.
GI manifestations in SLE
Anemia
Heme in SLE: ______ is present in about 50% of patients with Lupus
Leukopenia (specific lymphopenia) Idiopathic thrombocytopenic purpura (ITP) - Can be an early manifestation of SLE. - Can lead to life-threatening hemorrhage. Ab to clot factors can impair clot formation and hemorrhage. → too much or not enough clotting
Heme symptoms in SLE
non-Hodgkin's Lymphoma
Increased ____________ in patients with SLE.
⅓ of patients will develop lymphadenopathy during the course of disease, lymphoma should always be in the differential Splenomegaly sometimes Increased non-Hodgkin's Lymphoma in patients with SLE.
Lymphadenopathy in SLE
Cognitive dysfunction - inability to concentrate Psychosis more likely due to steroid treatment. Cerebrovascular disease stroke Seizures Headaches
MC neuropsychiatric manifestations (CNS) in SLE
difficult need a careful Hx and PE, analysis of CSF and imaging.
MC neuropsychiatric manifestations (CNS) in SLE - diagnosis can be
- Sensorimotor neuropathies→ damage to small nerve fibers - CN and ocular involvement vasculopathy and ischemia. - Transvere myelitis assoc with APL.--> inflammation of spinal cord, mat cause dif with arms and legs, and bowel and bladder incontinence.
MC neuropsychiatric manifestations (PNS) in SLE
Arthralgias and non-erosive arthritis is one of the MC clinical features. PIPs and MCPs are the most commonly symptomatic, followed by knees and wrists. Jaccoud's arthropathy→ - Specific for lupus due to -- Subluxation (partial dislocation) at the MCP joints and swan neck deformities of fingers. -- Deformities are reducible. Osteoporosis and osteonecrosis
MSS Symptoms of SLE
Parenchymal Disease (less common); tissue itself is inflamed, less common but more problematic. May involve alveolar hemorrhage. Pulmonary embolism: secondary to peripheral thrombosis Pulmonary hypertension: increased pulmonary resistance and impaired diffusion capability.
Other Pulm Symptoms of SLE:
- Limit exposure to cold: entire body not just hands. - Avoid situations that are stressful - Avoid smoking (it ruins vasculature) - Avoid beta-blocking medications. → we dont give beta blockers for any type of vasospastic disease.
Preventative measures for Raynaud's Phenomenon
Systemic Lupus Erythematosus (SLE)
Production of autoantibodies, immune complexes and immune system cells causes inflammation and tissue damage.
PLEURITIS - affects 30% of patients - Trt with corticosteroids - Leads to pain on respiration and exudative effusions.
Pulm Symptoms of SLE: MC pulm manifestation
blood in capillaries becomes deoxygenated producing a cyanotic appearance.
Raynaud's Phenomenon (blue)
on rewarming, blood flow is increased producing reactive hyperemia
Raynaud's Phenomenon (red)
associated with or caused by other systemic illness, unilateral or few fingers
Raynaud's Phenomenon (secondary disease)
vasoconstriction or spasm of digital arteries
Raynaud's Phenomenon (white)
connective tissue diseases: scleroderma (90%) SLE (60%)
Raynaud's Phenomenon - common in patients with
cold exposure or emotional stress.
Raynaud's Phenomenon is often precipitated by
Limited Cutaneous Systemic Sclerosis
Raynaud's more associated with
74% of patients have kidney involvement; this is an indicator of poor prognosis - Renal pathology is caused by the deposition of circulating immune complexes in glomeruli (glomerulonephritis) which causes activation of complement and recruitment of inflammatory cells.
Renal Symptoms of SLE
Class I Class II
Renal Symptoms of SLE: Best Prognosis
Class IV (50% of glomeruli are involved)
Renal Symptoms of SLE: Worst Prognosis
CBC w/ diff→ anemic? leukopenia? aPTT → clotting issues? Urinalysis→ nephritis? Serum BUN and creatinine→ nephritis? ESR as an indicator of disease ANA (initial screening test for SLE), anti-ds DNA, anti Smith, anti RNP, antiRo, Anti _La C3 and C4 can be checked→ will be low during disease flare-ups. - Complement levels are REDUCED during flareups. Antiphospholipid Ab confer a higher risk for thrombi formation.
SLE Labs
C3 and C4 complement levels
SLE Labs: REDUCED during flareups.
Antiphospholipid Ab
SLE Labs: confer a higher risk for thrombi formation.
Anti ds DNA Ab
SLE Labs: may be helpful in following the activity of lupus patients with nephritis. → also useful for monitoring response to teatment.
Regular counseling and education; requires continuous f/u Advised to avoid known triggers (tobacco,sunlight, stress) Need for adequate rest. Pregnancy has to be carefully monitored.
SLE treatment
Myalgias,arthralgias, arthritis→ NSAIDs Skin Lesions → topical steroids Skin involvement + arthralgias→ Antimalarials: Hydroxychloroquine +/- NSAIDs - Associated with reduced thrombosis - Can cause retinal toxicity (macular degeneration)though→ need ophtho exams frequently.
SLE treatment (non-organ system involvement)
NSAIDs
SLE treatment (non-organ system involvement): Myalgias,arthralgias, arthritis
Hydroxychloroquine +/- NSAIDs - Associated with reduced thrombosis - Can cause retinal toxicity (macular degeneration)though→ need ophtho exams frequently.
SLE treatment (non-organ system involvement): Skin involvement + arthralgias→ Antimalarials
topical steroids
SLE treatment (non-organ system involvement): skin lesions
Corticosteroids used for more serious complications (Ex: pericarditis) - Oral prednisone for systemic problems - More susceptible to infection→ may see thrush. - S/E: osteonecrosis, osteoporosis, cataracts, diabetes, myopathy, htn, accelerated atherosclerosis, hypoadrenalism, emotional disturbances. Other trts: cyclophosphamide, azathioprine, mycophenolate mofetil.
SLE treatment (organ involvement)
- ANA (100% of patients have ANA) → its sensitive, but not specific to SLE. So cant use this alone for dx. - Anti ds DNA Ab (60%) - Anti-smith Ab (30%) - Both the Anti ds and the anti smith Ab are not sensitive, meaning not every pt will have them, but they are very specific to Lupus, so if a pt has the anti ds or anti smith they likely have Lupus. Also Anti ro/la Ab, antiphospholipid, Ab, and anti RNP Ab
Spectrum of Ab found in SLE:
15-44
Systemic Lupus Erythematosus (SLE) is usually diagnosed in females aged ______
- Virus infection (EBV) - UV light exposure - Smoking cigarettes - Drugs: "SHP" procainamide, hydralazine, sulfa drugs, treatments for RA and Hep C/hematologic malignancies - If you take a pt off these drugs, their symptoms should improve. - Immunologic Triggers: genetic and environmental trigger act on the immune system and induce autoimmunity and consequent tissue inflammation and damage.
Systemic Lupus Erythematosus (SLE) triggers
African Americans are affected 3-4x more than whites
Systemic Lupus Erythematosus (SLE): Prevalence, severity, and characteristics differ by ethnic grop→
Socioeconomic factors
Systemic Lupus Erythematosus (SLE): ____________as well contribute to disease
C1q, C2, C4A, and HLA-DR2 and DR3
Systemic Lupus Erythematosus (SLE):Genetic susceptibility, those associated with ___
Acquired connective tissue disorder, cause unknown, but linked to environmental and genetic susceptibility
Systemic Sclerosis (Scleroderma) is an
bleomycin, pentazocine, HRT, cocaine, appetite suppressants. - Once the meds trigger scleroderma, it doesnt go away, but drugs should still be stopped to stop the progression.
Systemic Sclerosis (Scleroderma): Environmental Factors (Drugs)
silica, polyvinyl chloride, epoxy resins
Systemic Sclerosis (Scleroderma): Environmental Factors (Occupational Exposure)
CMV and Parvovirus B19
Systemic Sclerosis (Scleroderma): Environmental Factors (Virus)
women 30-50 yrs old, african americans
Systemic Sclerosis (Scleroderma): most commonly affects
family history
Systemic Sclerosis (Scleroderma): strongest identifiable risk factor
African Americans and males
_____ and _____have worse prognosis for scleroderma.
Libman Sacks nodules
___________ are inflammatory in nature and may be related to Antiphospholipid Ab in SLE.
19
_______neuropsychiatric syndromes can be associated with SLE
Female: Male ratio is 8-9:1 (increased estrogen)
are males or females affected more by Systemic Lupus Erythematosus (SLE)
beta-blocking
avoid giving ________ with Raynaud's Phenomenon
pulmonary fibrosis
diffuse Cutaneous Systemic Sclerosis
Systemic Sclerosis (Scleroderma)
fibrosis of skin and vascular dysfxn
ANA
initial screening test for SLE
pulmonary hypertension
limited Cutaneous Systemic Sclerosis
procainamide, hydralazine, sulfa drugs, treatments for RA and Hep C/hematologic malignancies
lupus like drugs *know*
secondary Raynaud's
nailfold capillaroscopy: abnormal
primary Raynaud's
nailfold capillaroscopy: normal
Valve abnormalities: nodules or valve thickening (sometimes leads to regurg) - Libman Sacks nodules are inflammatory in nature and may be related to Antiphospholipid Ab in SLE. Premature atherosclerosis due to chronic inflammation with immune system activation which leads to vascular damage. Raynaud's phenomenon affects up to 60% of patients.
other CV Symptoms of SLE:
5 yr survival rate >90% and 10 yr 85%
prognosis of SLE
- Hispanic texan african american - Lack of health insurance - Poor social support.
prognosis of SLE: Higher lupus activity predicted with the following
organ damage within first year
prognosis of SLE: Poorer survival in those with _________
rare
prognosis of SLE: prolonged remission is _____
Elevated Cr
prognosis of SLE:____________indicates worse outcome
Organ failure
scleroderma ***______ due to alterations in small blood vessels and progressive fibrosis (lungs, GI tract, kidneys and heart)
antitopoisomerase I
scleroderma Clinical Manifestations (Diffuse Cutaneous Systemic Sclerosis): Associated with _________________—> in 30% of patients.
fingers, extremities, face, and trunk. Tendency to develop tendon rubs. **TRUNK IS AFFECTED
scleroderma Clinical Manifestations (Diffuse Cutaneous Systemic Sclerosis): Skin induration
rapid
scleroderma Clinical Manifestations (Diffuse Cutaneous Systemic Sclerosis): _______ progression
Pulmonary fibrosis and acute renal failure
scleroderma Clinical Manifestations (Diffuse Cutaneous Systemic Sclerosis): _____________ and _____________ are common early in the disease.
more rapid and severe
scleroderma Clinical Manifestations (Diffuse Cutaneous Systemic Sclerosis): course is
limited to fingers and toes, distal to elbows and face. ○ Trunk spared and slow progression
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis) Skin induration
typically RAYNAUD'S PHENOMENON
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis) first manifestation
● More common ● Skin induration: limited to fingers and toes, distal to elbows and face. ○ Trunk spared and slow progression ● First manifestation: typically RAYNAUD'S PHENOMENON Often associated with CREST SYNDROME Calcinosis cutis: deposits of calcium salts in the skin and subQ Sclerodactyly; skin tightening, largely affecting the hands and fingers^ Associated with anti-centromere antibody (50% of patients) May develop pulmonary arterial hypertension later in life.
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis) is
CREST syndrome
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis) is often associated with
calcium salts
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis): Calcinosis cutis: deposits of__________in the skin and subQ
pulmonary arterial hypertension
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis): May develop ____________ later in life.
skin tightening, largely affecting the hands and fingers (the abnormal build up of fibrous tissue in the skin cause the skin to tighten so severely that the fingers curl and lose their mobility
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis): Sclerodactyly
anti-centromere antibody (50% of patients)
scleroderma Clinical Manifestations (Limited Cutaneous Systemic Sclerosis): associated with
Typical color changes→ white, blue, red Occurs in virtually all pts with scleroderma Can distinguish primary from secondary via nailfold capillaroscopy
scleroderma Clinical Manifestations (Raynauds)
nailfold capillaroscopy
scleroderma Clinical Manifestations (Raynauds) : Can distinguish primary from secondary via ____________
all pts with scleroderma
scleroderma Clinical Manifestations (Raynauds) occurs in
CCB Dress warmly, minimize cold exposure. Limit stressful situations.
scleroderma Clinical Manifestations (Raynauds): treatment
ARBs can also be used (losartan )
scleroderma Clinical Manifestations (Raynauds): treatment: if CCBs are not tolerated
commonly
scleroderma Clinical Manifestations (System: GI ): _______ affected
bilateral and symmetric skin thickening ● Start distal and moves proximal, hyperpigmented ● Obliteration of sweat and sebaceous glands. ● Face becomes thin with retraction of lips, can be mask-like ● Telengectasias of face, hands, lips, and oral mucosa ● Pailful ulcerations of IP joints, bony promiences, and fingertips. ● Calcinosis cutis→ calcifications in the skin
scleroderma Clinical Manifestations: (systems: skin) hallmark
Early skin involvement is inflammatory and can be controlled with systemic antihistamines or short-term low-dose corticosteroids. Skin dryness→ manage with hydrophilic ointments and bath oils. Fingertip ulcerations: protected with occlusive bandages to promote healing and prevent infections. Infected skin ulcers; topical or oral Abx.
scleroderma Clinical Manifestations: (systems: skin) treatment
thickening and hardening of skin
scleroderma hallmark
chronic and progressive disease with no cure
scleroderma is a ______________, although current treatments can effectively control symptoms, slow disease progression, and improve quality of life.
1. Autoimmunity and inflammation 2. Fibrosis and Matrix Deposition 3. Vascular injury and obliteration.
scleroderma pathology (triad)