Vol 15, No 4 (2022)
Case report
Published online: 2023-02-20

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Slowly progressive chronic kidney disease caused by tubulointerstitial nephritis in a patient with primary biliary cholangitis

Wojciech Wołyniec12, Agnieszka Ptasińska-Perkowska3, Tomasz Liberek4
DOI: 10.5603/RDTF.2023.0005
Renal Disease and Transplantation Forum 2022;15(4):180-186.


Tubulointerstitial nephritis is a rare complication of primary biliary cholangitis. The most typical presentation is progressive renal disease, and a substantial number of patients have renal tubular acidosis and mild proteinuria. Treatment with steroids is effective, but there are no precise recommendations concerning doses and the duration of therapy. This article presents a case of a 41-year-old woman with primary biliary cholangitis and slowly progressive chronic kidney disease. Renal tubular acidosis and very high urinary β2 microglobulin excretion but no albuminuria were observed. A kidney biopsy revealed a diffuse interstitial inflammatory infiltrate in both cortex and medulla, dominated by T lymphocytes and macrophages, less numerous B lymphocytes, neutrophils, and eosinophils. After initiation of steroids, a rapid 10-fold decrease in β2 microglobulin urine excretion and a mild decrease in serum creatinine were observed. This case shows how mildly symptomatic tubulointerstitial nephritis is in a patient with primary biliary cholangitis. The authors emphasize the importance and crucial role of kidney biopsy.

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