Systemic sclerosis with MPO-ANCA associated vasculitis with renal and skininvolvement

Scleroderma renal crisis is the usual form of presentation of renal disease in systemic sclerosis. We report a woman who at age 63 was given a diagnos is of scleroderma with Raynaud's phenomenon and cutaneous, oesophageal and lung involvement but no evidence of renal disease and no treatment with D-p enicillamine. Two years later she developed progressive renal failure, neph rotic range proteinuria, haematuria and the presence of serum MPO-ANCA; she was normotensive. Renal biopsy revealed extracapillary and necrotizing glo merulonephritis and skin biopsy showed leucocytoclastic vasculitis. This cl inical picture was compatible with necrotizing vasculitis of the microscopi c polyarterits type. After treatment with pulse steroids followed by oral s teroids and monthly intravenous cyclophosphamide her renal function stabili sed and the serum MPO-ANCA disappeared.