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未知领域:白塞病中的视网膜血管炎和冷球蛋白血症——一例报告

Uncharted territory: retinal vasculitis and cryoglobulinemia in Behçet's disease - a case report.

作者信息

Zou Yadan, Yang Kun, Zhang Xiaoying, Li Sheng-Guang

机构信息

Department of Rheumatology and Immunology, Peking University International Hospital, Peking University, Beijing, China.

Second Department of Clinical Medicine, Shanxi Medical University, Taiyuan, China.

出版信息

Front Immunol. 2025 Jul 4;16:1533595. doi: 10.3389/fimmu.2025.1533595. eCollection 2025.

DOI:10.3389/fimmu.2025.1533595
PMID:40688074
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12271173/
Abstract

Behçet's disease (BD) is a systemic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and ocular involvement, often presenting with retinal vasculitis as a severe complication. Although mixed cryoglobulinemia, typically associated with hepatitis C virus (HCV) infection, is well-documented in other autoimmune diseases, its coexistence with BD is exceedingly rare. This report details the case of a 56-year-old male presenting with BD complicated by HCV-related mixed cryoglobulinemia, manifesting as retinal vasculitis, purpuric skin lesions, and systemic vasculitis. Despite initial corticosteroid treatment, the patient required escalated therapy, including immunosuppressants and antiviral agents, to achieve disease stability. This case underscores the need for a multidisciplinary approach in managing BD with secondary cryoglobulinemia and highlights the complex interplay between autoimmune and viral-induced vasculitis. Our findings contribute to the literature by documenting a rare presentation of BD and providing insights into comprehensive treatment strategies for similar cases.

摘要

白塞病(BD)是一种全身性炎症性疾病,其特征为复发性口腔和生殖器溃疡、皮肤病变以及眼部受累,常伴有视网膜血管炎这一严重并发症。虽然混合性冷球蛋白血症通常与丙型肝炎病毒(HCV)感染相关,在其他自身免疫性疾病中有充分记载,但其与白塞病共存极为罕见。本报告详细介绍了一名56岁男性病例,该患者患有白塞病并伴有HCV相关的混合性冷球蛋白血症,表现为视网膜血管炎、紫癜性皮肤病变和系统性血管炎。尽管最初采用了皮质类固醇治疗,但患者仍需要升级治疗,包括使用免疫抑制剂和抗病毒药物,以实现病情稳定。该病例强调了在管理继发性冷球蛋白血症的白塞病时采用多学科方法的必要性,并突出了自身免疫性和病毒诱导性血管炎之间复杂的相互作用。我们的研究结果通过记录白塞病的罕见表现并为类似病例的综合治疗策略提供见解,为文献做出了贡献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/424bb8ffcc2f/fimmu-16-1533595-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/2ec09add4415/fimmu-16-1533595-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/5794db4eb552/fimmu-16-1533595-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/37d45569e1fc/fimmu-16-1533595-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/424bb8ffcc2f/fimmu-16-1533595-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/2ec09add4415/fimmu-16-1533595-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/5794db4eb552/fimmu-16-1533595-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/37d45569e1fc/fimmu-16-1533595-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7ac5/12271173/424bb8ffcc2f/fimmu-16-1533595-g004.jpg

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本文引用的文献

1
Frosted branch angiitis in Behcet disease.白塞病中的霜样树枝状视网膜血管炎。
QJM. 2024 Jul 1;117(7):527-528. doi: 10.1093/qjmed/hcae044.
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Behçet's Syndrome.白塞病
N Engl J Med. 2024 Feb 15;390(7):640-651. doi: 10.1056/NEJMra2305712.
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Br J Dermatol. 2021 Dec;185(6):1209-1220. doi: 10.1111/bjd.20643. Epub 2021 Sep 20.
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Cryoglobulinaemia.冷球蛋白血症。
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How I treat cryoglobulinemia.我如何治疗冷球蛋白血症。
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Occlusive retinal vasculitis secondary to Behçet's disease.白塞病继发的闭塞性视网膜血管炎。
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Gamma Delta (γδ) T Cells and Their Involvement in Behçet's Disease.γδ T 细胞及其在贝赫切特病中的作用。
J Immunol Res. 2015;2015:705831. doi: 10.1155/2015/705831. Epub 2015 Oct 11.
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Cryoglobulinemia Vasculitis.冷球蛋白血症性血管炎。
Am J Med. 2015 Sep;128(9):950-5. doi: 10.1016/j.amjmed.2015.02.017. Epub 2015 Mar 30.