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Renal manifestations in adult-onset Still’s disease: a systematic review

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Abstract

Objective

We aimed to review the literature on the clinical presentation, renal pathology, treatment, and outcome of renal manifestations in adult-onset Still’s disease (AOSD).

Methods

We used PRISMA guidelines for our systematic review and included all English-language original articles from inception till September 15, 2023, on AOSD and kidney involvement in any form. Data on patient demographics, diagnostic criteria, clinical presentation, renal pathology, treatment employed including dialysis, outcome, cause of death were collected and analyzed.

Results

The median age at the diagnosis of renal issues was 37, with a higher prevalence among females (58.1%). Among the cases, 28 experienced renal problems after being diagnosed with AOSD, 12 had simultaneous diagnoses of renal issues and AOSD, and in 4 cases, renal problems appeared before AOSD diagnosis. Out of the 44 cases, 36 underwent renal biopsy, revealing various pathology findings including AA amyloidosis (25%), collapsing glomerulopathy (11.4%), thrombotic microangiopathy (TMA) (11.4%), IgA nephropathy (9.1%), minimal change disease (6.8%), and others. Some cases were clinically diagnosed with TMA, proximal tubular dysfunction, or macrophage activation syndrome-related acute kidney injury. Treatment approaches varied, but glucocorticoids were commonly used. Renal involvement was associated with increased mortality and morbidity, with 6 out of 44 patients passing away, 4 progressing to end-stage renal disease (ESRD), and data on 2 cases’ outcomes not available.

Conclusion

Renal manifestations in AOSD are diverse but rarely studied owing to the rarity of the disease. Studies with larger data would be essential to study further on the pathogenesis and implications.

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Data availability

All datasets for this study are included in the manuscript and the supplementary files.

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Acknowledgements

We acknowledge the contributions of all medical professionals involved in researches whose publications helped us come up with this review. We also acknowledge the kindness of the patients and their families who are helping to disseminating medical knowledge. We are grateful for our institutions who supported this work through resources and facilities.

Funding

This research was not funded by any specific grant from any public, commercial, or non-profit sectors.

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Authors and Affiliations

  1. Yale New Haven Health/Bridgeport Hospital, 267 Grant St, Bridgeport, CT, 06610, USA

    P. V. Akhila Arya

  2. Department of Internal Medicine, Yale New Haven Health, Bridgeport Hospital, Bridgeport, USA

    Erica Marnet

  3. Department of Internal Medicine, Texas Tech University, El Paso, USA

    Madhumita Rondla

  4. Department of Nephrology, Stanford School of Medicine, Stanford, USA

    Jia Wei Tan

  5. Department of Internal Medicine, Yale New Haven Health, Stamford, USA

    Dileep Unnikrishnan

  6. Internal Medicine/Nephrology, Yale New Haven Health, Bridgeport Hospital, Bridgeport, USA

    Gregory Buller

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Conception or design of the work: AA, JWT, GB. Acquisition, analysis, or interpretation of data for the work: AA, EM, DU. Drafting the work or revising it critically for important intellectual content: AA, EM, MR, GB. Final approval of the version to be published: AA, EM, MR, JWT, DU, GB. All authors take full responsibility for the integrity and accuracy of all aspects of the work.

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Arya, P.V.A., Marnet, E., Rondla, M. et al. Renal manifestations in adult-onset Still’s disease: a systematic review. Rheumatol Int (2024). https://doi.org/10.1007/s00296-024-05578-5

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