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30 January 2022: Articles

Distal Renal Tubular Acidosis Associated with Autoimmune Diseases: Reports of 3 Cases and Review of Mechanisms

Unusual clinical course, Challenging differential diagnosis, Diagnostic / therapeutic accidents, Unusual setting of medical care, Educational Purpose (only if useful for a systematic review or synthesis), Rare coexistence of disease or pathology

Marcelo Augusto Duarte Silveira A* , Antônio Carlos Seguro D , Samirah Abreu Gomes D , Maria Helena Vaisbich D , Lúcia Andrade A

DOI: 10.12659/AJCR.933957

Am J Case Rep 2022; 23:e933957

Table 1. Possible pathophysiological mechanisms involved in the occurrence of distal renal tubular acidosis in autoimmune disease.

Immune-mediated diseasePathophysiological mechanism of dRTA
Reduced AE2 expression (congenital or caused by proteolysis) could be related to changes intracellular pH. This could lead to protein mistargeting in liver cells and in kidney tubules, antigenic changes and autoimmunity [].Presence of IgG autoantibody against the band 3 isoform (Cl/HCO) in hepatocytes and alpha-type intercalated cells [].Cross-reactive cytotoxicity between hepatocyte surface antigens (membrane proteins) and tubular Tamm-Horsfall glycoprotein [].12
Tubulointerstitial nephritis with mononuclear lymphocytic infiltrate [,].Absence of proton-ATPase in alpha-type intercalated cells [].Autoantibody against carbonic anhydrase II (RTA type 3) [].19
Tubulointerstitial nephritis (association with Sjögren syndrome) [].25
Proton-ATPase defect in alpha-type intercalated cells [].Tubulointerstitial nephritis [].26
Autoantibody against alpha-type intercalated cells [].29
dRTA – distal renal tubular acidosis; ATPase – adenosine triphosphatase; RTA type 3 – the rare combination of type 1 (distal) and type 2 (proximal) RTA; IgG – immunoglobulin G; Cl/HCO – chloride/bicarbonate exchanger.

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923