title: Progressive chronic kidney disease secondary to tubulointerstitial nephritis in primary biliary cirrhosis.
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.
authors.affiliation: Sheffield Kidney Institute, Northern General Hospital , Sheffield , UK.
authors.forename: Tarun
authors.initials: T
authors.lastname: Bansal
authors.affiliation: Department of Histopathology , Northern General Hospital , Sheffield , UK.
authors.forename: Anna
authors.initials: A
authors.lastname: Takou
authors.affiliation: Sheffield Kidney Institute, Northern General Hospital , Sheffield , UK.
authors.forename: Arif
authors.initials: A
authors.lastname: Khwaja
keywords: chronic kidney disease; familial; primary biliary cirrhosis (PBC); tubulointerstitial nephritis