Scleroderma Fibrosis and vascular changes Uncommon Peak onset 20-50 years Female: Male 3:1 Aetiological factors include Genetic Environmental: drugs, solvents, silica

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

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

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

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