Monday 22 November 2010

MRCP revision battle 57.3: Systemic sclerosis

Systemic sclerosis is a connective tissue disease characterised by thickening and fibrosis of skin (scleroderma) and involvement of internal organs.

Female: male 4:1

Commonest in 5th/6th decades

Limited systemic sclerosis = skin involvement is limited to face, hands and feet.
Associated with anti-centromere antibodies in 70-80%

Diffuse systemic sclerosis = more skin involvement
Anti Scl 70 in 40%

Features of sclerosis:
  • Raynaulds - 1st presentation in 70% of cases
  • arthralgia
  • renal failure
  • scleroderma
  • GI problems
  • classical skin presentation:
    • telangiectasia
    • peri-oral puckering
    • smooth shiny skin
    • livedo reticularis
  • pulmonary fibrosis/hypertension

ESR/CRP are raised

CREST is a type of limited systemic sclerosis:
- calcinosis, raynaulds, eosophageal dysmotility, sclerodalyl, telangectasia
- renal crsis is rare but pulmonary hypertension is common

Linear scleroderma = coup de sabre

  • supportive
  • d-penicillamine
  • steroids if lung disease
  • ACE-i to help kidneys