Anti Topoisomerase I (Scl-70), anti-RNA polymerase III positivity is classically seen with:
Diffuse systemic sclerosis Skin involvement- usually Rapid onset. Diffuse and with rapid progression Raynauds phenomenon- Onset coincident with skin involvement-mild Musculoskeletal-Severe ahralgia, carpal tunnel syndrome Pulmonary fibrosis-Frequent, early and severe Pulmonary hypeension-May occur, often in association with pulmonary fibrosis Scleroderma renal crisis-Occurs in 15%; early Calcinosis cutis-May occur, mild Characteristic autoantibodies-Anti Topoisomerase I (Scl-70), anti-RNA polymerase III CREST syndrome-A subset of patients with limited systemic sclerosis a) calcinosis cutis, b) Raynaud's phenomenon, c) esophageal dysmotility, d) sclerodactyly e) telangiectasia Raynaud's phenomenon and other typical features of systemic sclerosis occur in the absence of detectable skin thickening in some patients. This syndrome is been termed SSc sine scleroderma. Ref: Harrison,E-18,P- 2758.
Limited cutaneous sclerosis
Diffuse cutaneous sclerosis
CREST syndrome
SSc sine scleroderma