Idiopathic multicentric osteolysis with nephropathy

Idiopathic multicentric osteolysis is a rare syndrome that manifests with p rogressive loss of carpal and tarsal bones in childhood. Affected children have arthritic-like episodes, followed by progressive deformities, radiogra phic osteolytic changes, and variable degrees of disability. A rare form of this disease (type III, sporadic) is associated with serious nephropathy. We present the first reported case of type III idiopathic multicentric oste olysis in a Chinese woman. The patient, a 34-year-old woman with normal men tal development and no family history of bone or kidney disease, presented with a 4-day history of nausea and vomiting. She had shortening and swellin g of the hands, which had occurred in childhood and persisted ar the time o f admission. X-ray studies showed disappearance of the carpel bones, and mu ltiple osseous erosions of the tarsal bones. Hypertension, severe azotemia, and metabolic acidosis were also noted. Advanced renal disease was documen ted after a series of investigations, including renal biopsy. She is now di alysis-dependent. This case illustrates the importance of early diagnosis a nd management of idiopathic multicentric osteolysis with nephropathy.