LSUHSC Renal Pathology Consultative Services - Case Study #  1   -  All case studies

Case Study #: 1

Clinical History:

A 28-year male guest of the State of Louisiana (prisoner) presented with asthma and was found to have an elevated creatinine. He denied illicit drug use. Physical examination: Normal. Laboratory data:  Creatinine: 4.0mg/dl Urinalysis: 3+ protein, no rbcs, no cellular casts 24-hour urine: 2.5 gram protein Serologies: Hep B/C, ANA, ANCA all negative C3: normal A renal biopsy was performed.

Figure 1:   Cortex showing diffuse tubular atrophy and interstitial fibrosis with a bland infiltrate.     Figure 2:   The cortical fibrosis is dense and replaces all native structures.     Question: What is the diagnostic finding? What diagnosis does it strongly suggest? Answer:   Diagnosis: Chronic sclerosing interstitial nephritis, suggestive of aristolochic (Chinese herb) or other toxic nephropathy. Discussion: The patient did not admit to the ingestion of drugs or herbal remedies, thus the diagnosis cannot be established with certainty. However, the dense replacement fibrosis that was the dominant finding in this biopsy is very unusual and has only been reported with herbal remedy associated disease. In these remedies aristolochic acid has been implicated. When the entire kidney is available for examination the replacement fibrosis is most severe in the peripheral cortex. Collecting ducts often show cytologic atypia and from 40-46% of patients have been reported to develop urothelial carcinoma. For this reason, bilateral nephrectomies have been performed in some cases. References: Nephrol Dial Transplant 18:440-442, 2003. Am J Kid Dis 35:313-318, 2000. Am J Kid Dis 24:172-180.

Stephen M. Bonsib, MD  (Dr Bonsib's Bio)

Chairman Pathology

Director, Renal Pathology Consultative Services

Albert G. and Harriet G. Smith Professor of Pathology

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