Coexistence of Fabry's disease and necrotizing and crescentic glomerulonephritis

The coexistence of Fabry's disease, an X-linked hereditary disease, and oth er renal diseases, has rarely been described in the same patient. Combined Fabry's disease and pauci-immune necrotizing and crescentic glomerulonephri tis (NCGN) is hitherto unreported. We present the clinical and pathologic d ata of two patients with combined Fabry's disease and NCGN. Both patients p resented with fevers of unknown origin and progressive renal insufficiency, however, lacked any other pathognomic signs of Fabry's disease such as acr oparesthesias, dyshidrosis, and cutaneous angiokeratomas. The possible path ogenic mechanisms and causal relationship between the two disease processes are discussed.