Scleroderma, limited and diffuse
Description
- Multisystem autoimmune disease: vasculopathy + autoimmunity + fibrosis of skin and internal organs
- The only rheumatic disease in which fibrosis, not inflammation, is the dominant end-organ process
Subtypes - defined by the EXTENT of skin involvement
| Limited cutaneous (lcSSc / CREST) | Diffuse cutaneous (dcSSc) | |
|---|---|---|
| Proportion | ~55% | ~35% |
| Skin | Distal to elbows and knees + face; gradual onset | Proximal to elbows/knees, trunk; rapid onset |
| Raynaud | Precedes skin change by years | Onset within ~1 year of skin change |
| Antibody | Anti-centromere | Anti-Scl-70 (topoisomerase I); nucleolar ANA pattern |
| ILD | <20%, later onset | ~40%, early and can be severe (~15%) |
| Renal crisis | Rare | ~10% |
| PAH | More common - the late killer of limited disease | Less common |
- CREST: Calcinosis, Raynaud, o(E)sophageal dysmotility, Sclerodactyly, Telangiectasia
- *Sclerodactyly above the elbows or across the chest = diffuse. That single examination finding assigns the subtype, the antibody, and the prognosis*
- "Scleroderma sine scleroderma" - visceral disease (pulmonary fibrosis, renal crisis, cardiac failure, GI pseudo-obstruction) without skin thickening; the diagnosis rests on autoantibodies (ANA, anti-centromere, anti-Scl-70)
Cutaneous features
- Raynaud phenomenon (>95%), telangiectasia including on the lips, subcutaneous calcinosis with fingertip ulceration, skin tightening (sclerodactyly)
- General inspection: smooth, shiny, tight facial skin; facial telangiectasia with hyper- or hypo-pigmentation ("salt and pepper"); pinched beaked nose; microstomia with radial perioral furrowing
- Skin evolves through oedematous -> indurative -> atrophic phases
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