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

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...

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Autores principales: Elitok, Saban, Sidler, Marius, Bieringer, Markus, Mohebbi, Nilufar, Schneider, Wolfgang, Wagner, Carsten A
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Exceptional Cases
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7367120/
https://www.ncbi.nlm.nih.gov/pubmed/32699627
http://dx.doi.org/10.1093/ckj/sfz059
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author Elitok, Saban
Sidler, Marius
Bieringer, Markus
Mohebbi, Nilufar
Schneider, Wolfgang
Wagner, Carsten A
author_facet Elitok, Saban
Sidler, Marius
Bieringer, Markus
Mohebbi, Nilufar
Schneider, Wolfgang
Wagner, Carsten A
author_sort Elitok, Saban
collection PubMed
description 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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spelling pubmed-73671202020-07-21 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 Clin Kidney J Exceptional Cases 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. Oxford University Press 2019-06-21 /pmc/articles/PMC7367120/ /pubmed/32699627 http://dx.doi.org/10.1093/ckj/sfz059 Text en © The Author(s) 2019. Published by Oxford University Press on behalf of ERA-EDTA. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Exceptional Cases
Elitok, Saban
Sidler, Marius
Bieringer, Markus
Mohebbi, Nilufar
Schneider, Wolfgang
Wagner, Carsten A
A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
title A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
title_full A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
title_fullStr A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
title_full_unstemmed A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
title_short A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
title_sort patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis
topic Exceptional Cases
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7367120/
https://www.ncbi.nlm.nih.gov/pubmed/32699627
http://dx.doi.org/10.1093/ckj/sfz059
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