CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1687704
This article is part of the Research TopicCase Reports in Autoimmune and Autoinflammatory Disorders: Volume IIView all 31 articles
Anti-Gamma Aminobutyric Acid B Receptor Antibody-Associated Limbic Encephalitis in Relapsing Polychondritis: a rare case report and literature review
Provisionally accepted
- 1Gansu Provincial Hospital, Lanzhou, China
- 2Hangzhou Normal University, Hangzhou, China
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Relapsing polychondritis (RP) is an immune-mediated disorder that primarily involves the targeting of cartilaginous tissues for inflammation and destruction. Limbic encephalitis (LE) is a rare central nervous system (CNS) manifestation of RP. We report the case of a 39-year-old man who was diagnosed with RP complicated by anti-gamma-aminobutyric acid B receptor (GABABR) antibody‑associated LE and presented with recurrent headache, fever, bilateral auricular swelling, scleral injection, and cognitive impairment. Laboratory tests revealed positive anti-GABABR IgG antibodies in both the serum (titer 1:100) and cerebrospinal fluid (CSF) (titer 1:1), and CSF lymphocytic pleocytosis. Brain MRI revealed bilateral frontal and parietal subcortical and periventricular T2-FLAIR hyperintensities. Immunosuppression therapy with high-dose methylprednisolone and cyclophosphamide induced rapid symptom resolution and no relapse occurred during a follow-up period of one year. This case expands the spectrum of RP-associated LE, emphasizes the necessity of neuronal autoantibody screening in RP patients with neurological symptoms, and suggests potential pathogenic links involving antigenic cross-reactivity between cartilage and neural tissues and GABAergic metabolism dysregulation.
Keywords: Relapsing polychondritis, Anti-gamma-aminobutyric acid B receptor, LimbicEncephalitis, Autoimmune mechanism, Central Nervous System
Received: 18 Aug 2025; Accepted: 15 Oct 2025.
Copyright: © 2025 Jinling, Baiyu, Lijuan, Tianjiao, Yishu, Minghua and Taowen. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Ren Taowen, rtw0931@163.com
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