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Published on: June 23, 2015
Progressive chronic kidney disease secondary to tubulointerstitial nephritis in primary biliary cirrhosis
Tarun Bansal1, Anna Takou2, Arif Khwaja1
1Sheffield Kidney Institute, Northern General Hospital , Sheffield , UK.
Abstract:
Primary biliary cirrhosis (PBC) is an autoimmune disease characterized by the presence of anti-mitochondrial antibodies (AMA). Whilst asymptomatic distal tubular acidosis (DTA) is the commonest renal lesion reported in PBC, tubulointerstitial nephritis (TIN) has also been reported as a rare association. Although PBC could be a familial disorder, there have been no previous reports of familial chronic TIN in association with PBC. We report a case of progressive chronic kidney disease (CKD) due to TIN in a mother and daughter known to suffer from PBC and review the previously reported literature. Both showed good response to steroids.
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