Systemic Sclerosis (Scleroderma) - Diagnosis and Management ...

Systemic Sclerosis (Scleroderma) - Diagnosis and Management Summary

 • Clinical Presentation: 

    - Localized: (affects skin only): linear (e.g., en coup de sabre), morphea

    - Systemic: may be limited cutaneous [lcSSc] (67% pts; skin thickening in hands/face only, commonly with CREST sx) or diffuse cutaneous [dcSSc] (33% pts; diffuse skin thickening, more significant multi-organ dz, less commonly with CREST sx)

    - CREST sx: Calcific nodules, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasias 

    - Other systemic sx: renal crisis, ILD (>70%), PAH (10-40%)

    - NB: systemic sclerosis sine scleroderma → pts with scleroderma but w/o skin findings

 • Workup: +anti-centromere (a/w lcSSc, only seen in 5% pts with dcSSc), +anti-Scl-70 (a/w dcSSc), +RNA-pol-III (a/w dcSSc and scleroderma renal crisis), +fibrillarin (a/w severe dcSSc,

esp. in African-Americans

 • Treatment: 

    - Skin: MMF, MTX

    - GI: PPIs, motility agents

    - Lung: CCBs, endothelin-1 antag., PDE inhibitors, prostacyclin agonists

    - MSK: Low dose pred, hydroxychloroquine, MTX

    - Raynaud’s: CCBs

 • Complications: Increased risk of multiple cancers

    - Scleroderma renal crisis (up to 20%): AKI, abrupt HTN; a/w antiRNA-pol III; treat with ACEi (captopril) + avoid steroids

#Systemic #Sclerosis #Scleroderma #Diagnosis #Management #treatment #rheumatology
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