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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

“CREST syndrome (calcinosis and sclerodactyly)” © James Heilman, MD CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0/)

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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