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An overlap of IgG4-related tubulointerstitial nephritis and microscopic polyangiitis-associated glomerulonephritis: a case-based review

  • CASE BASED REVIEW
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Abstract

Because of some similarities in organ involvement, clinical manifestations, and histopathological features, IgG4-related disease (IgG4-RD) may occur concurrently with some clinicopathologic variants of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). An overlap syndrome of IgG4-RD and AAV has recently been proposed in clinical and/or histopathological studies, indicating that there may be some potential pathophysiological associations between the two disease entities; however, the mechanisms underlying these are incompletely understood. Here, we describe a rare case of a 63-year-old man with IgG4-related tubulointerstitial nephritis (IgG4-TIN) and microscopic polyangiitis-associated glomerulonephritis (MPA-GN) overlap syndrome. The clinical diagnosis of MPA was based on the 2022 American College of Rheumatology (ACR)/European League Against Rheumatology (EULAR) classification criteria. Remission induction therapy with intravenous methylprednisolone was initiated, followed by oral prednisone maintenance therapy with gradual tapering. The patient remained asymptomatic and his renal function was essentially normalized within 3.5 months of follow-up. The serum IgG4 levels decreased to 5 g/L. We also conducted a literature review to identify clinical findings, treatment options, and outcomes of patients with concurrent IgG4-RD and MPA and briefly discussed the potential pathophysiological association between IgG4-RD and MPA. Our findings enrich the database of this rare overlap syndrome and provide a basis for the diagnosis and early intervention in both diseases. These results provide some insights for clinicians to recognize and treat this overlap syndrome.

Key Points

• We report a unique case that enriches the database of this rare overlap syndrome and provides a basis for the outcome and early intervention in both diseases.

•Several studies have suggested that IgG4-RD and MPA have a potential association, and we describe the pathophysiological mechanism of the overlap syndrome for the first time.

• There have been no standardized diagnostic criteria for MPA for over 30 years; the 2022 ACR/EULAR classification criteria are expected to solve this problem.

• We provide some clues for the diagnosis and treatment of this overlap syndrome based on the literature review.

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Data availability

The datasets generated and/or analyzed during the current study are available upon request from the corresponding author.

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Acknowledgements

We appreciate the patient for agreeing to share his story in the current manuscript.

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Authors and Affiliations

  1. Department of Gastroenterology, The Second Hospital of Jilin University, Changchun, Jilin, 130041, China

    Ronghua He

  2. Department of Nephrology and Rheumatology, The Second Hospital of Jilin University, Changchun, Jilin, 130041, China

    Mingqi Ma, Ping Luo & Qiaoyan Guo

Authors
  1. Ronghua He
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  2. Mingqi Ma
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  3. Ping Luo
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  4. Qiaoyan Guo
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Contributions

Conceptualization: Ronghua He. Writing—original draft preparation: Ronghua He. Data collection: Mingqi Ma and Qiaoyan Guo. Methodology: Qiaoyan Guo and Mingqi Ma. Writing, review, and editing: Qiaoyan Guo and Ping Luo.

Corresponding author

Correspondence to Qiaoyan Guo.

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Ethics approval and consent to participate

This study was approved by the Ethics Committee of the Second Hospital of Jilin University (No. 2018198) and adhered to the Helsinki Declaration guidelines.

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Written informed consent was obtained from the patient for publication of the case details and accompanying figures. A copy of the written consent is available for review by the editor of this journal.

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He, R., Ma, M., Luo, P. et al. An overlap of IgG4-related tubulointerstitial nephritis and microscopic polyangiitis-associated glomerulonephritis: a case-based review. Clin Rheumatol 42, 1459–1467 (2023). https://doi.org/10.1007/s10067-022-06493-5

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  • DOI: https://doi.org/10.1007/s10067-022-06493-5

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