Systemic sclerosis / CREST
Incidental findings Raynaud’s
+ve signs Tight, shiny skin fingers, sclerodactyly, calcinosis, telangiectasia
Differential diagnosis Systemic sclerosis / CREST
Function Hand function
Tests FBC, CXR
Anticentromere, Anti Scl-70
Systemic sclerosis questions

  • Do you have any difficulty with swallowing
  • Do your fingers change colour in the cold
  • Dry eyes/mouth (associated sjogren’s)
Raynauds questions

  • Do your fingers change colour in the cold
  • What colour do they go – is there a particular sequence (white/blue/red)
  • How long have you had this problem
  • What is your job; does it involve vibrating tools
Notes

  • CREST: Calcinosis, raynauds, esophageal dysmotility, sclerodactyly and telangiectasia. More favourable prognosis than systemic sclerosis; anti-centromere antibody.
  • Limited cutaneous scleroderma = only extremities (10y survival 71%), diffuse cutaneous scleroderma = trunk and extremities (10y survival 21%).
  • Rx: symptomatic / immunosupression (cyclophosphamide, methotrexate). No role for steroids.