利妥昔单抗治疗特发性多发性castleman病合并严重神经病变、干燥综合征和膜肾病1例：病例报告和文献综述
Idiopathic multiple castleman disease case combined with severe neuropathy, Sjogren's syndrome and membrane nephropathy treated by rituximab: a case report and literature review.
摘要(中文译文)
特发性多中心型Castleman病（iMCD）是一种以系统性免疫失调为特征的淋巴增殖性疾病。已发现多种细胞因子参与该病的发病机制。因此，iMCD累及多系统使诊断和疗效评估变得复杂。尽管指南推荐抗白细胞介素-6（IL-6）药物作为主要治疗，但二线治疗的选择仍不明确。一名65岁女性在接种COVID-19疫苗后十天出现进行性多神经病和肾病范围蛋白尿。评估显示多中心淋巴结肿大、IL-6升高及浆细胞型CD组织病理学（HHV-8阴性）。同时确诊干燥综合征和抗PLA2R阴性膜性肾病。排除POEMS综合征后，诊断为中等严重程度的iMCD-NOS。初始利妥昔单抗-环磷酰胺-地塞米松治疗导致神经系统矛盾性恶化，尽管VEGF水平下降。由于经济限制无法获得抗IL-6治疗。开始单药利妥昔单抗治疗，并在24个月内持续九个周期，至2024年1月实现临床缓解，炎症标志物接近正常化、蛋白尿消退及神经系统恢复。本病例表明，在无法获得抗IL-6治疗时，利妥昔单抗单药治疗可使伴有并发自身免疫表现的iMCD-NOS实现临床缓解。延迟应答模式——生物标志物改善先于临床恢复——凸显了系列VEGF监测以及在判定治疗失败前坚持B细胞靶向治疗的重要性。
Abstract
Idiopathic multicentric Castleman disease (iMCD) is a lymphoproliferative disorder characterized by dysregulated systemic immunity. Multiple cytokines had been found involved in the disease pathogenesis. Hence, involvement of multiple systems in iMCD complicates diagnosis and efficacy assessments. Although guidelines recommend anti-interleukin-6 (IL-6) agents as the primary treatment, options for second-line therapy remain indeterminate. A 65-year-old woman presented with progressive polyneuropathy and nephrotic-range proteinuria ten days after COVID-19 vaccination. Evaluation revealed multicentric lymphadenopathy, elevated IL-6, and plasmacytic-variant CD histopathology (HHV-8 negative). Concurrent Sjögren's syndrome and anti-PLA2R-negative membranous nephropathy were confirmed. After exclusion of POEMS syndrome, iMCD-NOS with intermediate severity was diagnosed. Initial rituximab-cyclophosphamide-dexamethasone therapy resulted in paradoxical neurological worsening despite declining VEGF levels. Anti-IL-6 therapy was inaccessible due to economic constraints. Single-agent rituximab was initiated and continued for nine cycles over 24 months, achieving clinical remission by January 2024 with near-normalization of inflammatory markers, resolution of proteinuria, and neurological recovery. This case demonstrates that rituximab monotherapy can achieve clinical remission in iMCD-NOS with concurrent autoimmune manifestations when anti-IL-6 therapy is unavailable. The delayed response pattern-with biomarker improvement preceding clinical recovery-highlights the importance of serial VEGF monitoring and persistence with B-cell-directed therapy before concluding treatment failure.
如何引用
AMA
Shu Wang, Tong Chen, Shaojun Liu, Zunguo Du, Yanyan Kong, Yan Yuan, et al. Idiopathic multiple castleman disease case combined with severe neuropathy, Sjogren's syndrome and membrane nephropathy treated by rituximab: a case report and literature review.. Frontiers in immunology. 2026; doi:10.3389/fimmu.2026.1804654.
APA
Shu Wang, Tong Chen, Shaojun Liu, Zunguo Du, Yanyan Kong, Yan Yuan, et al (2026). Idiopathic multiple castleman disease case combined with severe neuropathy, Sjogren's syndrome and membrane nephropathy treated by rituximab: a case report and literature review.. Frontiers in immunology. https://doi.org/10.3389/fimmu.2026.1804654
GB/T 7714
Shu Wang, Tong Chen, Shaojun Liu, Zunguo Du, Yanyan Kong, Yan Yuan, et al. Idiopathic multiple castleman disease case combined with severe neuropathy, Sjogren's syndrome and membrane nephropathy treated by rituximab: a case report and literature review.[J]. Frontiers in immunology, 2026 doi:10.3389/fimmu.2026.1804654.
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