A case of variant form of Wegener's granulomatosis manifested scleritis at the onset

A 35 years old female had been treated with steroids for bilateral scleritis. High fever appeared 9 months later since the onset of scleritis. Proteinuria, hematuria and elevation of serum creatinine were pointed out and she was sent to our hospital because of rapid deterioration of renal function. Renal biopsy revealed crescentic glomerulonephritis. There was no finding of necrotizing vasculitis nor granuloma. Anti-human neutrophil cytoplasmic antibody (ANCA) was strongly positive in cytoplasmic pattern on admission, and then she was diagnosed as Wegener's granulomatosis (WG). Methylprednisolone pulse therapy combined with anti-coagulant and anti-platelet agents was effective in improving of renal function and clinical symptoms. ANCA became negative one month later together with clinical improvements. Eye involvements as initial symptoms of WG have been reported in only 4 cases. She manifested rapidly progressive glomerulonephritis following scleritis 9 month later without typical respiratory tract involvement. This clinical course was considered as a rare case for WG. ANCA was considered to be a very useful tool for diagnosis and treatment of WG.