El Hasbani Georges, El Ouweini Hala, Dabdoub Fatema, Hourani Roula, Jawad Ali Sm, Uthman Imad
Division of Rheumatology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Faculty of Medicine, American University of Beirut, Beirut, Lebanon.
Clin Med Insights Case Rep. 2022 Mar 9;15:11795476221083114. doi: 10.1177/11795476221083114. eCollection 2022.
Cerebral vasculitis is a very rare extra-articular complication of rheumatoid arthritis (RA) that is often challenging to diagnose. Elevated titers of rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP), and antinuclear antibodies (ANA) have been linked with severe complications. The absence of highly elevated titers of RF, anti-CCP, and ANA can complicate the diagnosis of RA-associated cerebral vasculitis. We report the case of a 59-year-old woman with long-standing arthritis maintained on rituximab and leflunomide who developed sudden headaches and altered level of consciousness. Laboratory work-up revealed normal lymphocyte count and mildly elevated total serum protein and anti-CCP with negative RF and ANA and no evidence for viral or bacterial infections. Cerebrospinal fluid analysis (CSF) showed slightly elevated anti-CCP with normal levels of CXCL-13 and interleukin 6 (IL-6). Brain magnetic resonance imaging (MRI) showed ill-defined lesion of high T2 signal. Using MR angiogram, MR perfusion, and MR spectroscopy, the diagnosis of rheumatoid cerebral vasculitis was confirmed. The patient was treated with intravenous methyl-prednisolone with fast complete improvement. We conclude that adequate immunosuppression in RA might not be able to prevent rare extra-articular manifestations such as rheumatoid cerebral vasculitis.
脑动脉炎是类风湿关节炎（RA）一种非常罕见的关节外并发症，其诊断往往具有挑战性。类风湿因子（RF）、抗环瓜氨酸肽抗体（抗CCP）和抗核抗体（ANA）滴度升高与严重并发症有关。RF、抗CCP和ANA滴度未显著升高会使RA相关脑动脉炎的诊断复杂化。我们报告了一例59岁长期患有关节炎的女性病例，该患者接受利妥昔单抗和来氟米特治疗，出现突发头痛和意识水平改变。实验室检查显示淋巴细胞计数正常，血清总蛋白轻度升高，抗CCP升高，RF和ANA阴性，且无病毒或细菌感染证据。脑脊液分析（CSF）显示抗CCP略有升高，CXCL - 13和白细胞介素6（IL - 6）水平正常。脑磁共振成像（MRI）显示T2高信号的边界不清病变。通过磁共振血管造影、磁共振灌注和磁共振波谱分析，确诊为类风湿性脑动脉炎。患者接受静脉注射甲泼尼龙治疗后迅速完全康复。我们得出结论，RA中充分的免疫抑制可能无法预防类风湿性脑动脉炎等罕见的关节外表现。
