Table 2 Classification of systemic scleroderma. (From: LeRoy et al. 1988)
From: Scleroderma
Limited type | Diffuse type |
|---|---|
Limited cutaneous systemic scleroderma | Diffuse cutaneous systemic scleroderma |
Long standing Raynaud’s syndrome before onset of skin fibrosis | Short interval (<1–2 years) of Raynaud’s syndrome before onset of skin fibrosis |
Limited peripheral skin involvement: acroscleroderma | Extension of fibrosis beyond the elbows to the trunk, tendon friction rubs |
Calcinosis, telangiectasia, pulmonary arterial hypertension | Pulmonary fibrosis, gastrointestinal disease, myocardial involvement, renal involvement |
Capillary ectasias in nail fold (mega-capillaries) | Capillary “drop-out” in the nail fold |
Anticentromere antibodies | Topo-isomerase (Scl 70) autoantibodies |