A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis

Abstract Autoimmune disorders such as rheumatoid arthritis or Sjögren’s syndrome can be associated with impaired renal acid excretion. Only few cases of patients with primary biliary cirrhosis (PBC) and distal renal tubular acidosis (dRTA) have been described. Here, we present the case of a 60-year-...

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Published inClinical kidney journal Vol. 13; no. 3; pp. 463 - 467
Main Authors Elitok, Saban, Sidler, Marius, Bieringer, Markus, Mohebbi, Nilufar, Schneider, Wolfgang, Wagner, Carsten A
Format Journal Article
LanguageEnglish
Published Oxford University Press 01.06.2020
Subjects
Chronic kidney failure
Exceptional Cases
Physiological aspects
Germany
dRTA
intercalated cells
autoantibodies
acidosis
primary biliary cirrhosis
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Summary:Abstract Autoimmune disorders such as rheumatoid arthritis or Sjögren’s syndrome can be associated with impaired renal acid excretion. Only few cases of patients with primary biliary cirrhosis (PBC) and distal renal tubular acidosis (dRTA) have been described. Here, we present the case of a 60-year-old woman with PBC and dRTA. Her kidney biopsy showed an absence of markers of acid-secretory Type A intercalated cells (A-ICs) and expression of aquaporin-2, a marker of principal cells, in all cells lining the collecting duct. Moreover, the serum of the patient contained antibodies directed against a subset of cells of the collecting duct. Thus, PBC-related autoantibodies may target acid-secretory A-ICs and thereby impair urinary acidification.
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ISSN:2048-8505
2048-8513
DOI:10.1093/ckj/sfz059