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Scleroderma

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

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Scleroderma

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

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

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

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

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

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