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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Detalles Bibliográficos
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
Descripción
Sumario: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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