Scleroderma

Scleroderma is a disease that keeps rheumatologists busy.

General

Clinical:

• Anti-centromere antibody (ACA) +ve.
  • Common in limited systemic scleroderma, AKA CREST syndrome.
• Scl-70 (topoisomerase I) +ve.
  • Common in diffuse scleroderma.

CREST syndrome:

• Calcinosis.
• Raynaud's phenomenon.
• Esophageal dysmotility.
• Sclerodactyly.
• Telangiectasia.

Associated pathology

• Scleroderma panniculitis.
• Scleroderma renal crisis - see thrombotic microangiopathy.
• Digit ischemia - may require finger amputation.

See also

• Basics.
• Systemic lupus erythematosus.