ArticleClinical kidney journal2020
A patient with chronic kidney disease, primary biliary cirrhosis and metabolic acidosis.
Article in Clinical kidney journal, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 4 citations in OpenAlex.
- Prevalence and incidence of chronic kidney disease in patients with primary biliary cholangitis.Journal of gastroenterology · 2026Article
- Liver-kidney crosstalk in primary biliary cholangitis: insights into the mechanisms of renal involvement.Frontiers in immunology · 2026Review
- Autoimmune Tubulopathies.Journal of the American Society of Nephrology : JASN · 2025Review
- A case report of hypokalemic periodic muscular weakness secondary to Sjögren's syndrome with distal renal tubular acidosis.Clinical case reports · 2023Article
- The pathophysiology of distal renal tubular acidosis.Nature reviews. Nephrology · 2023Review
- Distal Renal Tubular Acidosis in Patients with Autoimmune Diseases-An Update on Pathogenesis, Clinical Presentation and Therapeutic Strategies.Biomedicines · 2022Review
Corrections and comments
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Authors and funding
6 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
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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Registered trials
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