A case of Wegener's granulomatosis showing a dramatic response to corticosteroid and cyclophosphamide therapy--evaluation of anticytoplasmic antibodies (ACPA) in serum samples.

A case of Wegener's granulomatosis with renal dysfunction is described. Granulomatous lesions of the lung, left eyelid and left leg, and chronic sinusitis were observed at the time of admission. Renal and skin biopsy specimens revealed typical features of Wegener's granulomatosis on light microscopy and immunofluorescence microscopy. A dramatic response occurred following corticosteroid and cyclophosphamide therapy, characterized by improvement of proteinuria and renal function, and disappearance of the granulomas and 67gallium citrate accumulations in both the eyes and nose. Furthermore, the levels of anticytoplasmic antibodies (ACPA) in the serum samples were significantly decreased after such therapy. It appears that combined therapy with corticosteroid and cyclophosphamide can be effective in improving proteinuria and renal dysfunction, and in reducing of ACPA in patients with Wegener's granulomatosis.