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


Elitok, Saban; Sidler, Marius; Bieringer, Markus; Mohebbi, Nilufar; Schneider, Wolfgang; Wagner, Carsten A (2020). A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis. Clinical Kidney Journal, 13(3):463-467.

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.

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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Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Nephrology
04 Faculty of Medicine > Institute of Physiology
07 Faculty of Science > Institute of Physiology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Uncontrolled Keywords:Nephrology, Transplantation
Language:English
Date:21 June 2020
Deposited On:18 Feb 2020 14:47
Last Modified:01 Feb 2021 15:28
Publisher:Oxford University Press
ISSN:2048-8505
OA Status:Gold
Free access at:PubMed ID. An embargo period may apply.
Publisher DOI:https://doi.org/10.1093/ckj/sfz059
PubMed ID:32699627

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