[A case of limited form of Wegener's granulomatosis associated with persistent high titer of serum antineutrophil cytoplasmic antibodies].

A thirty-six year-old male was admitted to our hospital because of a two year history of orbital mass. He had experienced inflammatory tumor of right lung six years before admission. Both the lung tumor and orbital mass were revealed as chronic panarteritis with diffuse necrotizing lesions. He was treated with low dose prednisolone, but the orbital mass gradually enlarged. We detected fluorescent cytoplasmic pattern of antineutrophil cytoplasma antibodies (ANCA) by the indirect immunofluorescence method (IF). We ascertained this case to be a limited form of Wegener's granulomatosis (WG) by histopathological finding and positive ANCA. The value of ANCA, estimated by enzyme linked immunosorbent assay (ELISA) was rather higher than that of generalized form of WG in other reports. There was no reduction of the size of orbital mass and the value of ANCA despite strong immunosuppressive therapy. In this case, the value of ANCA didn't represented disease activity. The presence of histopathological findings such as those observed in the present patient, i.e., abnormally high ANCA levels not associated with disease severity or progression, which are not in accordance with those conventionally reported, was considered very interesting in terms of the prevailing beliefs concerning involvement of ANCA in the mechanism of onset of WG.