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25 Cards in this Set
- Front
- Back
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Collagen Vascular Disease: synonyms
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Systemic inflammatory rheumatic diseases
Systemic inflammatory diseases Connective tissue diseases |
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Collagen Vascular Disease: types
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SLE
Scleroderma Polymyositis/dermatomyositis |
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Collagen Vascular Disease: common features
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Multisystem involvement (skin, joints, muscle, lung, kidneys, blood)
Demographics (Women>men, young and middle aged) Constitutional symptoms (weight loss, fever, fatigue, arthralgia, myalgia) Lab findings (anemia, elevated ESR/CRP, organ specific markers, serologic markers) |
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Collagen Vascular Disease: common pathologies
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Immune activation/abnormalities > Inflammation > Vasculopathy > Fibrosis
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Collagen Vascular Disease: Immune activation/abnormatlities
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ANA antibodies
Other specific autoantibodies Low serum complements Antibody complexes |
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Collagen Vascular Disease: Vasculopathy
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Fibrotic intimal hyperplasia
Adventitial fibrosis Arterial lumen severely compromised |
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Collagen Vascular Disease: Fibrosis
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Scar tissue in organs due to untreated inflammations
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SLE: Pathogenic characterisitics (in order of importance)
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Immune activation/abnormalities, inflammation
Vasculopathy Fibrosis |
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SLE: pathogenesis
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1. Excessive auto-antibody formation (ANA,anti-dsDNA etc.)
2. Cytotoxic antibodies and immune complexes cause inflammation and destroy tissue 3. Immune complexes cause: low serum C3/C4, glomerlulonephritis 4. Cytotoxic antibodies cause hemolytic anemia |
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SLE: Classification criteria for SLE (need 4/11 for diagnosis)
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malar rash, discoid rash, photosensitivity, oral ulcers, arthrtitis, serositis, renal diease, seizures/psychosis, hematologic abnormalities, ANA, Immunologic
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SLE: Signs and symptoms
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Rash (butterfly, scalp), alopecia, oral ulcers, raynaud's
Kidney: proteinuria, renal insufficiency, glomerulonephritis Lungs: Pleuritis, pleural effusion, pneumonitis Cardiac: pericarditis, myocarditis, endocarditis, premature atherosclerosis Hematologic: cytopenias in any cell line |
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Scleroderma: Pathogenic characteristics (in order of importance)
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Fibrosis=Vasculopathy >Immune activation/abnormalitiy >Inflammation
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Scleroderma: classification
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Localized: skin only
Generalized: Limited (CREST syndrome), Diffuse (progressive systemic sclerosis) |
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Scleroderma: CREST syndrome manifestations
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Calcinosis (fingers)
Raynaud's Esophageal dysmotility Sclerodactyly Telangiectasia |
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Scleroderma: Progressive Systemic Sclerosis Manifestations
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Skin: widespread invovement
Lung: interstitial pulmonary fibrosis Colon: wide-mouthed diverticula |
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Dermatomyositis/polymyositis: Pathogenic characterisitcs
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Inflammation > Immune activation/abnormalities > vasculopathy > fibrosis
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Dermatomyositis/polymyositis: Chief complaint
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Muscle weakness
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Polymyositis: pathogenesis
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Interstitial mononuclear cell infiltrate in muscle fibres
Myocyte necrosis |
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Dermatomyositis: Clinical features
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Myositis: similar to polymyositis
Skin rash: Heliotropic, shawl sign, Gottron's papules Vasculopathy: nailfold capillaries, Raynaud's |
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Myositis: diagnosis
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Hx and Px: muscle weakness
Blood: elevated CK, anti-Jo1 Confirm by: EMG, MRI, Muscle biopsy (gold standard) |
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Collagen Vascular Disease: Treatment of inflammation
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Corticosteroids: oral, IV
Azathioprine Methotrexate Mycophenolate Mofetil Cyclophosphamide Hydroxychloroquine (SLE) |
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Collagen Vascular Disease: Treatment of vasculopathy
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Vasodilators: CCB's, etc.
Endothelial active agents |
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Collagen Vascular Disease: Treatment of fibrosis
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none
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Collagen Vascular Disease: Treatment of immune abnormalities
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No need, monitor and treat organs that are affected
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Corticosteroids: adverse effects
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Acute: infection, steroid psychosis, steroid myopathy
Chronic: Truncal obesity, moon face, acne, hirsutism, hypertension, hyperglycemia, skin atrophy, cataracts, fluid retention, osteoporosis, avascular necrosis |
