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Scleroderma = systemic sclerosis vs. localized scleroderma (morphea)
Systemic sclerosis
Limited cutaneous systemic sclerosis (lcSSc): skin involvement restricted to face and distal extremities
- CREST: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) --> subset of limited cutaneous SS
- High risk of pulmonary HTN in absence of ILD
Diffuse cutaneous systemic sclerosis (dcSSc): skin involvement proximal to distal forearms and knees
- Higher risk of ILD, serositis, renal crisis
Localized scleroderma (morphea)
Typically circumscribed, generalized, linear skin lesions without internal organ involvement
Posted 06/22/16 10:10:23 AM by Matthew Cummings
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Palpable tendon friction rubs: pathogenesis unclear, likely due to fibrin deposition in tendon’s synovial sheath
- Found in 20-40% of patients with dcSSc, associated with disease activity and predictor of continued diffuse skin involvement
Posted 06/22/16 10:17:16 AM by Matthew Cummings
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Nailbed capillaroscopy: presence of megacapillaries and decreased capillary density present in 60-80% of patients with lcSSc and dcSSc
Posted 06/22/16 10:42:29 AM by Matthew Cummings
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Extractable nuclear antigen (ENA) panel
- Anti-SSA (Ro), Anti-SSB (La): Sjögren's (and associated with congenital heart block in neonatal lupus)
- Anti-Smith: SLE
- Anti-U1-RNP: Mixed connective tissue disease
- Anti-Jo-1 (synthetase): Polymyositis, anti-synthetase syndrome (ILD, symmetric small joint arthritis, myositis, mechanic's hands)
- Anti-Scl-70 (anti-topoisomerase I): diffuse systemic sclerosis (higher risk for ILD)
Anti-centromere: limited cutaneous systemic sclerosis
Anti-RNA polymerase 3: associated with rapidly progressive skin disease in diffuse cutaneous systemic sclerosis, increased risk for renal crisis, GAVE, and malignancy
Posted 06/22/16 10:53:49 AM by Matthew Cummings
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Scleroderma renal crisis
Risk factors: +anti-RNA polymerase, diffuse cutaneous or rapidly progressive SSc, recent treatment w/ high dose corticosteroids
Physiology: narrowing of arterial vessels --> renal hypoperfusion --> hyperplasia of JGM apparatus --> increased renin release
Treatment: ACEI
Posted 06/22/16 11:07:57 AM by Matthew Cummings
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Raynoud's phenomenon treatment: dihydropyridine CCBs (nifedipine XL, amlodipine), ASA
Posted 06/22/16 11:16:56 AM by Matthew Cummings
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For further reading...
Posted 06/22/16 11:18:55 AM by Matthew Cummings