Systematic Review of Kidney Involvement in Antisynthetase Syndrome.

Authors

Qi-Shun Wu, Ling Yang, Xin Lin, Zhang-Li Wu, Zhi-Liang Yu, Ya-Yun Zeng

Published in

Kidney international reports. Volume 11. Issue 9. Pages 106669. Epub Jun 16, 2026.

Abstract

Antisynthetase syndrome (ASyS) is a rare autoimmune disorder characterized by autoantibodies targeting aminoacyl-tRNA synthetases. Kidney involvement has been historically regarded as uncommon and remains poorly characterized.
We conducted a systematic review using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines (PROSPERO: CRD420261282317). MEDLINE, Scopus, Web of Science, and Google Scholar were searched from inception to January 2026. We included adults with biopsy-proven or clinically defined kidney involvement in ASyS. Risk of bias was assessed using Joanna Briggs Institute tools; certainty of evidence was evaluated using the Grading of Recommendations Assessment, Development and Evaluation framework.
Thirty-eight articles comprising 52 patients were included. Kidney disease presented with or following interstitial lung disease (ILD) in 81% of cases. Among 35 biopsied patients, membranous nephropathy (29%), pauci-immune crescentic glomerulonephritis (23%), acute tubular necrosis (17%), and tubulointerstitial nephritis (11%) were identified. Non-Jo1 antibodies predominated in vasculitic phenotypes and were associated with higher peak creatinine than anti-Jo1 (4.7 vs. 2.3 mg/dl; P = 0.01). Corticosteroid monotherapy achieved complete recovery in 48% of mild disease, whereas rituximab was effective in 6 of 8 refractory cases. At a median of 18 months follow-up, 13% progressed to chronic kidney disease (CKD) stages 4 and 5.
Kidney involvement in ASyS carries substantial clinical significance. Non-Jo1 serotype may predict worse kidney outcomes, though this association requires confirmation in prospective cohorts with multivariable adjustment. Routine kidney surveillance and early antibody-guided immunosuppression may improve outcomes, pending validation.

PMID:
42438724
Bibliographic data and abstract were imported from PubMed on 13 Jul 2026.

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