Progressive chronic kidney disease secondary to tubulointerstitial nephritis in primary biliary cirrhosis
نویسندگان
چکیده
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.
منابع مشابه
Tubulointerstitial nephritis associated with primary biliary cirrhosis
Primary biliary cirrhosis (PBC) is an immunemediated chronic cholestatic liver disease. The etiology is unknown, although it is presumed to be autoimmune in natural (1). PBC is most frequently a disease of women and occurs between the fourth and sixth decades of life. The symptoms may strongly affect patients’ quality of life and may induce incapacitation (2). The major pathology of this diseas...
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