Atypical Fabry's disease presenting with cholesterol crystal embolization

We describe a 65-year-old man who presented with pulmonary hemorrhage and p rogressive renal insufficiency three months after resection surgery for an abdominal aortic aneurysm. Intensive treatment with corticosteroids and hem odialysis were not effective, and the patient died. Postmortem examination of the kidneys revealed widespread cholesterol clefts within the renal arte rioles and a number of lamellar inclusion bodies were observed by electron microscopy, The diagnosis of Fabry's disease was made by the absence of pla sma a-galactosidase A activity. This was a very rare case of subclinical Fa bry's disease coexistent with cholesterol crystal embolization, mimicking p ulmonary-renal syndrome.