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Acute interstitial nephritis caused by ANCA-associated vasculitis: a case based review

  • Case Based Review
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Abstract

Antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis (AAV) typically manifests as rapidly progressive glomerulonephritis with crescent formation. In this report, we present a local case of myeloperoxidase (MPO)-AAV–associated acute interstitial nephritis (AIN), showing slight pauci-immune glomerulonephritis and positive MPO-ANCA. This case is characterized by foot process effacement of podocytes in the glomerulus, a favorable prognosis, and an absence of crescentic formation. To further understand this condition, we conducted a comprehensive literature search on Google Scholar and PubMed, employing both free text words and MeSH terms related to “AAV and AIN.” This search yielded 24 cases, which we analyzed for their clinical features, laboratory findings, renal pathological characteristics, and therapeutic outcomes. AAV-associated interstitial nephritis predominantly affects elderly patients and is often associated with anemia, proteinuria, hematuria, and nonspecific manifestations, including fever, anorexia, fatigue, edema, and weight loss. Most of the cases in our review were MPO-ANCA-positive and exhibited isolated interstitial inflammation. These patients typically presented with relatively lower levels of serum creatinine, 24-h urine protein levels, and MPO-ANCA titers. All patients in our study received immunosuppressive therapy, including glucocorticoids, immunosuppressants, and rituximab, with the majority achieving clinical remission. Isolated AIN in the context of AAV is a rare occurrence, but it displays distinct clinical, laboratory, and pathological features. Patients with this presentation show a positive response to immunosuppressive treatment. Nevertheless, the establishment of definitive therapy guidelines for AAV-associated AIN remains uncertain and necessitates further investigation to develop comprehensive treatment guidelines. AIN, particularly when lacking typical glomerulus lesions, may represent a novel subgroup within MPO-AAV warranting additional research and clinical attention.

Key Points

• This study contributes valuable scientific insights by highlighting that MPO-AAV-associated interstitial nephritis, even without crescentic formation, can exhibit podocyte foot process effacement and respond well to treatment.

• The presence of AIN, independent of crescentic glomerulonephritis, suggests the potential emergence of a new subclass within MPA-AAV.

• Notably, some cases of MPO-AAV-associated AIN may present with normal levels of Scr (Table 5, cases 5, 6, and 17).

• This observation highlights the importance of considering renal biopsy, diagnosis, and therapy in a timely manner to prevent the development of chronic kidney disease (CKD).

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Funding

This work was funded by the National Key Research and Development Program of China (2020YFC2005000 to XX), and the Natural Science Foundation of Hunan Province (2022JJ30070 to RT).

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Authors and Affiliations

  1. Department of Nephrology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, 410008, Hunan, China

    Amir Muhammad, Zhou Xiao, Ting Meng, Jianping Ning, Hui Xu, Rong Tang & Xiangcheng Xiao

  2. Department of Pathology, Xiangya Hospital, Central South University, Changsha, 410008, China

    Wei Lin

  3. Department of Nephrology, Third Hospital of Changsha, Changsha, 410015, China

    Yingli Zhang

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  1. Amir Muhammad
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Contributions

Amir Muhammad processed data collection, analysis, and manuscript drafting. Zhou Xiao contributed to data collection and analysis. Wei Lin processed renal pathological analysis. Jianping Ning and Hui Xu supervised the study concept and design. Rong Tang and Cheng Xiao Xiang supervised the study concept, design, and manuscript revision. Yingli Zhang and Ting Meng revised the manuscript. All authors approved the final version of this manuscript.

Corresponding authors

Correspondence to Rong Tang or Xiangcheng Xiao.

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The research was approved by the Ethics Committee of Xiangya Hospital of Central South University. All procedures in this study were conducted according to the regulations of the Declaration of Helsinki. Patient signed the informed consent.

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Muhammad, A., Xiao, Z., Lin, W. et al. Acute interstitial nephritis caused by ANCA-associated vasculitis: a case based review. Clin Rheumatol 43, 1227–1244 (2024). https://doi.org/10.1007/s10067-023-06798-z

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