ImportanceImmunoglobulin G4 (IgG4)–related disease is an increasingly recognized fibroinflammatory condition that can involve multiple organs, including the pachymeninges. The understanding of IgG4-related pachymeningitis (IgG4-RP) remains limited because of its rarity and the predominance of knowledge derived from case reports and case series.ObjectiveTo systematically review and synthesize the clinical presentation, investigation findings, and prognosis of IgG4-RP to better understand its diagnosis and management.Evidence ReviewA comprehensive systematic review was conducted following guidelines from the Preferred Reporting Items for Systematic Reviews and Meta-analyses. PubMed/MEDLINE, Embase, and Scopus were searched from their inception until May 30, 2023, using terms related to IgG4-related disease and pachymeningitis without language or publication restrictions. Case reports and series that met the 2020 Revised Comprehensive Diagnostic Criteria or the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria were included. Data on clinical presentations, investigation findings, and treatment outcomes were extracted and summarized.FindingsA total of 148 case reports contributed data from 208 patients. Their median (IQR) age was 52 (39-62) years; 132 patients were male (63.5%) and 76 female (36.5%). Headache and cranial nerve dysfunctions were the most common neurological manifestations. Systemic involvement was identified in nearly half of the patients. Diagnostic imaging often showed preferential involvement of cavernous sinus and middle fossa. Laboratory results highlighted elevated serum IgG4 levels in 97 of 147 patients (65%) of patients and cerebrospinal fluid pleocytosis in 43 of 82 patients (52%). Storiform fibrosis or obliterating phlebitis were uncommon pathological findings. Mortality was below 1% (1/134; 0.7%), but only a third of patients presented complete clinical improvement, and the recurrence rate was 60 patients (40%) in a median (IQR) follow-up time of 9 (1-20) months. Glucocorticoids were the most commonly prescribed treatment, in 143 of 169 patients (85%); rituximab was prescribed as maintenance therapy in 53 of 169 patients (31%).Conclusions and RelevanceIgG4-RP commonly presents with headaches and cranial nerve dysfunction, posing diagnostic challenges due to the significant absence of systemic manifestations, low IgG4 serum levels, and atypical pathological findings. Current treatment outcomes are limited by incomplete recovery and frequent relapses underscoring the necessity for new treatment strategies.

中文翻译：

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IgG4 相关硬脑膜炎的临床表现、检查结果和结局


重要性免疫球蛋白 G4 （IgG4） 相关疾病是一种越来越公认的纤维炎症性疾病，可累及多个器官，包括硬脑膜。由于 IgG4 相关硬脑膜炎 （IgG4-RP） 罕见且从病例报告和病例系列中获得的知识占主导地位，因此对 IgG4 相关硬脑膜炎 （IgG4-RP） 的理解仍然有限。目的系统回顾和综合IgG4-RP的临床表现、检查结果和预后，以更好地理解其诊断和治疗。证据综述按照系统综述和荟萃分析首选报告项目的指南进行了全面的系统综述。使用与 IgG4 相关疾病和硬脑膜炎相关的检索词 PubMed/MEDLINE、Embase 和 Scopus，检索时间从 2023 年 5 月 30 日到 2023 年 5 月 30 日，没有语言或出版限制。纳入符合 2020 年修订的综合诊断标准或 2019 年美国风湿病学会/欧洲抗风湿病联盟分类标准的病例报告和系列。提取并总结有关临床表现、调查结果和治疗结局的数据。结果共有 148 份病例报告提供了来自 208 名患者的数据。他们的中位 （IQR） 年龄为 52 （39-62） 岁;132 例患者为男性 （63.5%），女性 76 例 （36.5%）。头痛和颅神经功能障碍是最常见的神经系统表现。近一半的患者被发现全身受累。诊断性影像学检查常显示海绵窦和中颅窝优先受累。实验室结果显示，147 例患者中有 97 例 （65%） 血清 IgG4 水平升高，82 例患者中有 43 例 （52%） 脑脊液细胞增多。 Storiform 纤维化或闭塞性静脉炎是罕见的病理表现。死亡率低于 1% （1/134;0.7%），但只有 1/3 的患者表现出完全的临床改善，复发率为 60 例患者 （40%），中位 （IQR） 随访时间为 9 （1-20） 个月。糖皮质激素是最常用的处方治疗，169 名患者中有 143 名 （85%）;169 例患者中有 53 例 （31%） 被开具利妥昔单抗作为维持治疗。结论和相关性IgG4-RP 通常表现为头痛和颅神经功能障碍，由于明显缺乏全身表现、低 IgG4 血清水平和非典型病理表现，给诊断带来挑战。目前的治疗结果受到不完全恢复和频繁复发的限制，这凸显了新治疗策略的必要性。