2. Scleroderma Fibrosis and vascular changes Uncommon
Peak onset years
Female: Male 3:1
Aetiological factors include
Genetic
Environmental: drugs, solvents, silica

3. Subtypes Limited Diffuse CREST Skin changes proximal to MCPJ
Visceral involvement: lung, kidney, oesoph

4. Clinical features Raynauds 95%
Skin: oedema, hardening, salt- and –pepper rash,ulcerations
Joints: arthritis, contractures
GIT: oesophagus, bowel
Lung: fibrosis, pulmonary hpt
Renal crisis

17. Diagnosis Cutaneous Vascular Visceral Serology supports ANF 40-90%
CREST: anti- centromere Ab
Diffuse: anti-topoisomerase1
RNA Polymerase

18. Management Raynaud’s Skin Analgesics Oesophageal symptoms Specific
Penicillamine
Steroids + cyclophosphamide
ACE inhibitors