1/3 Systemic sclerosis Management 1/3 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.
