We report a 42-year-old man who showed alveolar hemorrhage and glomeru
lonephritis as well as episcleritis and skin rash. He had an extremely
high titer of cytoplasm-staining antineutrophil cytoplasmic antibody
(C-ANCA) and was diagnosed as having systemic vasculitis based on hist
ological findings of kidney and skin biopsies. After immunosuppressive
therapy clinical manifestations resolved within several weeks and C-A
NCA titers commensurably declined. C-ANCA titers, however, increased a
gain and remained high despite clinical remission. In general, there i
s a close relationship between ANCA titers and clinical activities in
ANCA-associated diseases, but they displayed a large discrepancy in th
is patient. Indeed, the serum of the patient in remission contained th
e antibody against 29-kD neutrophil extracts which was detected by imm
unoblot analysis. These findings suggest that C-ANCA may not necessari
ly be, by itself, pathogenetic for the development of the vasculitis.