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Central nervous system involvement in patients with antineutrophil cytoplasmic antibody–associated vasculitis: a study of 29 cases in a single Chinese center

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Abstract

Background

In antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), central nervous system (CNS) involvement is relatively uncommon. The current study retrospectively investigated the clinical features and outcomes of AAV patients with CNS involvement.

Methods

A total of 497 AAV patients were retrospectively recruited in our center, twenty-nine of which had CNS involvement. Clinical and radiological manifestations and the outcomes of these patients were analyzed.

Results

The predominant symptom was sensorimotor impairment. According to the MRI findings, twenty-four patients had cerebral ischemic lesions, four patients had hemorrhagic lesions, and one patient had pituitary mass. With a median follow-up of 25 (range 9–45) months, 23 of 24 patients with cerebral ischemic lesions responded to induction therapy, and symptoms were ameliorated. The remaining one died from acute myocardial infarction 2 months after the diagnosis of cerebral ischemic lesions. Compared with patients without CNS involvement, patients with CNS involvement had significantly higher level of Birmingham Vasculitis Activity Score (23.5 ± 5.3 versus 18.8 ± 6.5, P < 0.01) and significantly higher proportion of peripheral nervous system involvement (58.6% versus 14.6%, P < 0.01). However, we did not found significant difference of patients’ survival between those with and without CNS involvement.

Conclusion

CNS involvement in Chinese patients with AAV was mainly manifested as cerebral ischemic lesions. Compared with patients without CNS involvement, patients with CNS involvement had a significantly more active disease of AAV, and significantly higher proportion of peripheral nervous system involvement.

Key Points

• CNS involvement in Chinese patients with AAV was mainly manifested as cerebral ischemic lesions.

• Patients with CNS involvement had a significantly more active disease of AAV.

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Acknowledgments

We thank all the colleagues in our department for their kind cooperation in this article.

Funding

This study was supported by two grants from the National Natural Science Fund (No. 81870477, 81870478), and two grants from Scientific Research Seed Fund of Peking University First Hospital (No. 4804001 and 2018SF004).

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Authors and Affiliations

  1. Renal Division, Department of Medicine, Peking University First Hospital, Beijing, 100034, China

    Tian-Tian Ma, Zhi-Ying Li, Yu-Shan Geng, Min Chen & Ming-Hui Zhao

  2. Peking University Institute of Nephrology, Beijing, 100034, China

    Tian-Tian Ma, Zhi-Ying Li, Yu-Shan Geng, Min Chen & Ming-Hui Zhao

  3. Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, China

    Tian-Tian Ma, Zhi-Ying Li, Yu-Shan Geng, Min Chen & Ming-Hui Zhao

  4. Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing, 100034, China

    Tian-Tian Ma, Zhi-Ying Li, Yu-Shan Geng, Min Chen & Ming-Hui Zhao

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  1. Tian-Tian Ma
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  2. Zhi-Ying Li
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  3. Yu-Shan Geng
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  4. Min Chen
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Correspondence to Min Chen.

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Approval and consent statement

The research was in compliance with the Declaration of Helsinki and approved by the Ethics Committee of Peking University First Hospital. All participating subjects gave their informed consent in writing prior to participation in the study.

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Ma, TT., Li, ZY., Geng, YS. et al. Central nervous system involvement in patients with antineutrophil cytoplasmic antibody–associated vasculitis: a study of 29 cases in a single Chinese center. Clin Rheumatol 39, 2185–2193 (2020). https://doi.org/10.1007/s10067-020-04975-y

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  • DOI: https://doi.org/10.1007/s10067-020-04975-y

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