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🌱 來自: systemic sclerosis and scleroderma disorders

Diagnostic studies and monitoring of systemic sclerosis and scleroderma disorders

• Autoantibodies: >95% Pts w/ auto-Ab; generally mutually exclusive
  • ⊕ anti-Scl-70 (anti-topoisomerase 1): a/w diffuse SSc; ↑ risk pulm fibrosis
  • ⊕ anticentromere: a/w limited SSc; ↑ risk of severe digit ischemia and PHT
  • ⊕ anti-RNA-Pol III: a/w diffuse SSc; ↑ risk renal crisis; a/w cancer
  • ⊕ ANA (>90%), ⊕ RF (30%), ⊕ anti-U1-RNP a/w overlap syndrome
  • Other: anti-Th/To (a/w limited SSc), U3-RNP (a/w ILD), PmScl (polymyositis-SSc overlap)
• CXCL4 levels reported to correlate w/ degree of fibrosis (NEJM 2014;370:433)
• at ↣ baseline: ✓ BUN/Cr & UA for proteinuria, PFTs (spirometry, lung volumes, DLCO), high- res chest CT (if diffuse disease), TTE (RVSP for PHT), RHC if ↑ RVSP or suspect PHT
• Annual PFTs; TTE q1-2y
• Skin bx not routine, but helpful to assess other possible causes for skin thickening
• ↑ risk of malignancy (esp. lung cancer) compared to general population
• Frequent (eg, daily) BP ✓ to monitor for HTN suggestive of scleroderma renal crisis