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Systemic sclerosis
Management
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Skin and connective tissue disease:
Skin thickening: cyclophosphamide, methotrexate, or mycophenolate mofetil. Emollients or antihistamines for itch.
Raynaud's: 1st line calcium channel blockers (e.g. amlodipine), 2nd line add/switch to PDE-5 inhibitors (e.g. sildenafil, tadalafil).
Calcinosis: consider surgical resection if severe.
Internal organ disease:
Oesophageal dysmotility: PPIs, prokinetic agent (e.g. metoclopromide, erythromycin).
Scleroderma renal crisis: ACEi, renal replacement therapy if severe.
ILD: cyclophosphamide, transplantation if severe.
Pulmonary HTN: PDE-5 inhibitor, endothelin receptor antagonist (bosentan, ambrisentan, macitentan).
Ongoing inflammation (synovitis, myositis, arthritis): low dose steroids.
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