Service de Médecine Interne (M.L., N.M.), Hôpital l'Archet 1, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur; Service de Cytologie Pathologique (F.B.-V.), Hôpital l'Archet 1, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur; and Service de Neurologie (M.C., S.B., C.G., V.B., C.L.-F.), Service de Radiologie (L.M.), Service de Radiologie Interventionnelle (J.S.), and Service de Neurochirurgie (D.F.), Hôpital Pasteur 2, Centre Hospitalier Universitaire de Nice, Université Côte d'Azur, France.
Neurol Neuroimmunol Neuroinflamm. 2019 May 7;6(4):e568. doi: 10.1212/NXI.0000000000000568. eCollection 2019 Jul.
Meningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).
Two IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.
Forty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.
DISCUSSION/CONCLUSION: IgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.
脑膜受累在 IgG4 相关疾病中较为罕见，仅在病例报告和病例系列中有所描述。由于对该病的综述较为缺乏，我们报告了 2 例病例，并对 IgG4 相关肥厚性硬脑膜炎（IgG4-HP）进行了文献复习。
报告了 2 例 IgG4-HP 病例，其中 1 例累及脊髓，对手术治疗有反应，另 1 例累及大脑，对利妥昔单抗治疗有反应。然后，我们对发表在 PubMed-NCBI 数据库中的经组织学证实的 IgG4-HP 的临床病例和病例系列进行了回顾。
共研究了 42 份病例报告和 5 份病例系列（60 例患者，20 例女性）。中位年龄为 53 岁。18 例患者有系统性受累，24 例患者有单一器官 IgG4-HP。55%的患者血清 IgG4 升高。53 例中有 20 例接受了手术治疗。类固醇治疗和免疫抑制剂在 85%和 90%以上的病例中有效。类固醇治疗停止后疾病复发率为 42.1%。
讨论/结论：IgG4-HP 的特征为缺乏神经外器官受累和全身表现。由于血清标志物很少有提示性，因此应进行组织病理学研究，这对诊断至关重要。18F-FDG 正电子发射断层扫描有助于对系统性疾病进行特征描述。IgG4-HP 没有特定的 CSF 标志物，需要用更大的样本量来研究 CSF IgG4 水平的诊断价值。我们为 IgG4-HP 提供了一种治疗方案。这些治疗策略依赖于早期手术、类固醇和早期免疫抑制治疗，以预防神经并发症。
