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Scleroderma (Systemic Sclerosis)
Scleroderma (aka Systemic Sclerosis) means hardening of skin. Systemic sclerosis is an autoimmune inflammatory and fibrotic connective tissue disease, and can present in 2 ways:
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis
Clinical features - CREST:
Calcinosis
Raynaud’s phenomenon
Oesophageal dysmotility (Dysphagia, GORD)
Sclerodactyly
Telangiectasia
Diagnostic markers - Anti-centromere
Diffuse cutaneous systemic sclerosis
Clinical features - Those of CREST plus:
CVS problems – HTN, Coronary artery disease
Lung problems – Pulmonary HTN, Fibrosis
Kidney problems – Glomerulonephritis, Scleroderma renal crisis
Diagnostic markers - Anti-Scl-70
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