目的:提高对IgG4相关性疾病伴有中枢神经系统病变的认识,以早期诊断、早期治疗。方法:回顾性分析10例IgG4相关性疾病伴有中枢神经系统病变患者的临床资料,总结其临床表现、实验室检查、影像学检查、组织病理学及治疗方面的特点。结果:10例IgG4相关性疾病合并中枢神经系统受累的患者中,垂体受累者6例,硬脑膜受累者4例;单纯神经系统受累者仅2例,其余8例患者均合并其他脏器受累;一半患者以神经系统受累为首发表现,神经系统症状多表现为多饮、多尿、头痛、视力下降等。实验室检查中,9例(90.0%)血清IgG4水平升高,血清总IgE升高者7例(87.5%),部分患者血清IgG、血红细胞沉降率、C反应蛋白升高,补体C3、C4下降。8例患者接受了不同部位的组织病理活检,病理检查均可见大量淋巴细胞、浆细胞浸润,部分可见纤维组织增生。所有患者均接受了糖皮质激素治疗,8例(80%)联合了免疫抑制剂治疗,3例因病情反复给予利妥昔单抗治疗。所有患者中,2例(20%)达到完全缓解,8例(80%)部分缓解,中位随访时间13.5个月,复发4例。结论:IgG4相关性疾病的中枢神经系统病变中,垂体、硬脑膜为常见受累部位,大多合并其他脏器受累,但半数患者以神经系统症状为首发表现,因症状不典型,应结合实验室、影像学检查,必要时行组织病理活检明确诊断。

Objective: To improve the understanding of the clinical characteristics of immunoglobulin G4-related disease (IgG4-RD) with central nervous system (CNS) involvement, so as to contribute to the early diagnosis and treatment. Methods: In this study, 10 patients with IgG4-RD with CNS involvement were selected. Clinical data including demographic features, clinical manifestations, organ involvement, laboratory findings, radiological results, pathology, treatments and outcomes were collected retrospectively. Results: Among the 10 IgG4-RD patients complicated with CNS involvement, 6 cases manifested as pituitary involvement and 4 cases manifested as dural involvement. Only 2 patients had nervous system involvement alone, while the left patients were complicated with other organ involvement besides nervous system. Half of the patients presented nervous system involvement as the initial manifestation, and the main symptoms of nervous system included polydipsia, polyuria, headache, decreased vision and so on. In the laboratory examination, the serum IgG4 level was increased in 9 cases (90.0%), and the total serum IgE was increased in 7 cases (87.5%). At the same time, their IgG, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were increased, while complement (C) 3 and C4 were decreased in some patients. Eight patients underwent tissue biopsies in different parts, the pathological examination showed a large number of lymphocyte and plasma cell infiltration, accompanied by fibrous tissue proliferation in part. All the patients were treated with glucocorticoids, and 8 cases (80%) were given immunosuppressants at the same time, and 3 cases were treated with rituximab when they expe-rienced disease relapse. As a result, 2 cases (20%) achieved complete remission (CR), and 8 cases (80%) achieved partial remission (PR). The median follow-up time was 13.5 months, and 4 cases relapsed. Conclusion: Pituitary and dura are common affected organs of IgG4-RD with CNS involvement. Most IgG4-RD patients with CNS involvement are combined with other organ involvement, but half of them presented nervous system involvement as the initial manifestation, and the symptoms are not typical. In order to make an early diagnosis, laboratory, imaging examination and tissue biopsy should be taken into consideration in combination if necessary.