Systemic sclerosis

Systemic sclerosis is a connective tissue disease characterised by fibrosis of the skin and visceral organs. It is relatively rare, affecting around 20 per 100.00. Females are more affected than males (6:1 in UK) and it is commonest in the 5th decade.

There are 2 main subdivision:
  • Limited systemic sclerosis
    • skin involvement is limited to face, hands and feet
    • organs are involved later
    • associated with anti-centromere antibodies in 50%
    • CREST (calcinosis, Raynauds, oesophageal dysmotilty, sclerodatyl and telangectasia) is a subtype of limited systemic sclerosis
  • Diffuse systemic sclerosis
    • More skin involvement
    • Earlier visceral involvement
    • Anti Scl 70- found in 40%
Both types are associated with ANA in 85-98% of cases.
Anti-Ro is found in 9% of cases and is associated with a very severe and rapidly progressive disease.

Features of systemic sclerosis include:
  • Raynauds – occurs in 98% of cases and is 1st symptom in 70% of cases
  • GI tract involvement – strictures
  • Skin involvement
    • Telangiectasia
    • Microstomia/peri-oral puckering
    • Smooth shiny skin
  • Renal failure
  • Pulmonary hypertension/fibrosis
  • Arthralgia

Treatment is
  • Supportive
  • Nifedipine/lorsartan for Raynaulds
  • ACE I to protect kidneys
  • d-penicillamine
  • possibly steroids

Small print gem: side effects of penicillamine include proteinuria, leucopenia, thrombocytopenia, mucosal ulceration, cholestatis.

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