IgA nephropathy, antineutrophil cytoplasmic antibodies and crescentic glomerulonephritis in a patient with the Hermansky-Pudlak syndrome

Hermansky-Pudlak syndrome is an uncommon cause of renal dysfunction. Becaus e of the risk of bleeding in this condition, few patients have undergone a renal biopsy. Renal dysfunction has been attributed to the deposition of ce roid pigment in the tubules and interstitial fibrosis. We report a case wit h renal biopsy findings of ceroid deposition and interstitial fibrosis, but also of mesangial IgA deposition, crescentic glomerulonephritis, and an in terstitial lymphocytic infiltrate. Furthermore, perinuclear antineutrophil cytoplasmic antibodies of the IgG subclass were detected in a blood sample. It is well known that ceroid pigment in this syndrome accumulates in monoc ytes, macrophages and T lymphocytes and it has been suggested that th is ma y affect their function, We suggest that this novel combination of renal ch anges might be explained on the basis of alterations in immune mechanisms i n the Hermansky-Pudlak syndrome. Copyright (C) 2001 S. Karger AG,Basel.