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