Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Key Laboratory of Ministry of Education, Beijing, China.
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Key Laboratory of Ministry of Education, Beijing, China.
Clin Exp Rheumatol. 2020 Jul-Aug;38(4):626-632. Epub 2020 Feb 14.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease that affects various organs of the body. The aim of this study is to elucidate the clinical characteristics of IgG4-RD with central nervous system (CNS) involvement.
Among 589 patients with IgG4-RD in a prospective single-centre cohort study in Peking Union Medical College Hospital, 15 patients had CNS involvement. The clinical data including demographic features, symptoms, involved organs, laboratory findings, radiological results, pathology, treatment and outcome were analysed.
Seventeen patients, including nine men and six women, had IgG4-related neuropathy, with an average age of 49.8±12.3 years. IgG4 related hypophysitis was the most common manifestation, accounting for 40% (6/15) of cases, followed by hypertrophic cranial pachymeningitis (n=4), hypertrophic spinal pachymeningitis (n=2), intracranial mass (n=2), cavernous sinus and orbital disease (n=1). Most patients had multi-organ involvement, with the most common extra-CNS manifestations were Mikulicz disease (MD) and lymphadenitis in 5 (33.3%) cases. Serum IgG4 levels were elevated in 12/15(80%) patients and the median value was 438.5 (104, 2250)mg/dL. Fourteen cases underwent biopsy, of which tissue was taken directly from CNS lesions in 4 cases. All patients received treatment with glucocorticoids (GCs) combined with immunosuppressants, including cyclophosphamide, tacrolimus, mycophenolate mofetil, and tripterygium glycosides. Complete remission was achieved in 3/15 (20.0%) patients, and 11/15 cases (73.3%) achieved partial remission.
IgG4-related CNS involvement is a rare and distinct entity of IgG4-RD. Treatment with corticosteroids combined with immunosuppressive agents yielded favourable responses.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种影响全身多个器官的系统性疾病。本研究旨在阐明伴有中枢神经系统（CNS）受累的 IgG4-RD 的临床特征。
在一项北京协和医院前瞻性单中心队列研究的 589 例 IgG4-RD 患者中，有 15 例患者存在 CNS 受累。分析了包括人口统计学特征、症状、受累器官、实验室检查结果、影像学结果、病理学、治疗和预后在内的临床资料。
17 例患者，其中 9 例为男性，6 例为女性，患有 IgG4 相关性神经病，平均年龄为 49.8±12.3 岁。最常见的表现是 IgG4 相关垂体炎，占 40%（6/15），其次是肥厚性颅脊硬膜炎（n=4）、肥厚性脊髓硬膜炎（n=2）、颅内肿块（n=2）、海绵窦和眼眶疾病（n=1）。大多数患者存在多器官受累，最常见的 CNS 外表现为 Mikulicz 病（MD）和淋巴结炎，分别有 5 例（33.3%）。15 例患者中有 12 例（80%）血清 IgG4 水平升高，中位数为 438.5（104，2250）mg/dL。14 例行活检，其中 4 例直接取自 CNS 病变组织。所有患者均接受糖皮质激素（GCs）联合免疫抑制剂治疗，包括环磷酰胺、他克莫司、霉酚酸酯和雷公藤多苷。15 例患者中，3 例（20.0%）达到完全缓解，11 例（73.3%）达到部分缓解。
IgG4 相关 CNS 受累是 IgG4-RD 的一种罕见且独特的疾病实体。GCs 联合免疫抑制剂治疗可获得良好的反应。
