30 January 2022
: Case report 
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 Silveira12ABDEF*, Antônio Carlos Seguro23DEF, Samirah Abreu Gomes34DEF, Maria Helena Vaisbich5DEF, Lúcia Andrade23ADEFDOI: 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 disease | Pathophysiological 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. | |