Gautier Félicien, Neumann Lisa, Adle-Biassete Homa, Rubenstein Emma, Bernat Anne-Laure, Chimon Alice, Mouly Stéphane, Sène Damien, Comarmond Cloé
Department of Internal Medicine and Clinical Immunology, Lariboisière Hospital, Université Paris Cité, 75010 Paris, France.
Department of Neurology, Lariboisière Hospital, Université Paris Cité, 75010 Paris, France.
Autoimmun Rev. 2023 Apr;22(4):103285. doi: 10.1016/j.autrev.2023.103285. Epub 2023 Feb 2.
Hypertrophic pachymeningitis is a rare clinical disorder involving localized or diffuse thickening of the dura mater. Considering pachymeningitis is both in the clinical spectrum of IgG4-RD and ANCA vasculitis (specifically granulomatosis with polyangiitis), an overlap syndrome is discussed.
We report a case of hypertrophic pachymeningitis revealed by headache and cranial nerve dysfunction, and coexistence of biopsy-proven IgG4-RD pachymeningitis and MPO-ANCA positivity. Furthermore, all cases previously reported in the literature of pachymeningitis with IgG4-RD and presence of ANCA were analyzed.
Thirteen patients with pachymeningitis, IgG4-RD and ANCA were analyzed. Patients with HP-related IgG4 and ANCA are mainly male (8, 62%). Median age at diagnosis was 64 years. Main clinical manifestations at diagnosis were localized to the head and neck with headaches (10, 77%), cranial nerve dysfunction (7, 54%), hearing impairment (6, 46%) and vertigo (4, 31%). Except 1 patient with diffuse aortitis, no other systemic manifestation was observed at diagnosis and during follow-up. Serum IgG4 was often elevated (11, 85%) and ANCA was mainly with myeloperoxidase specificity (11, 85%). Seven patients had cerebrospinal fluid analyse with lymphocytic pleocytosis in 5 cases (71%), elevated proteins in 4 cases (57%), positive oligoclonal bands in 3 cases (42%) and decreased glucose in one case (14%). On the MRI, the thickening of the dura mater concerned most often the posterior fossa, in 7 cases (54%). Among 10 cases with histological findings, all showed increased IgG4-positivity of plasma cells, 50% lymphocytic infiltrate but none presented the three major histological criteria of IgG4-related disease. Three (30%) showed histological signs of vasculitis with vascular wall damage and/or giant cells. Among the 12 patients treated with steroid therapy, a clinical improvement was noted in 11 cases (92%). Relapse occurred during tapering in 4 patients (33%). An immunosuppressive drug was added in 2nd line for 7 cases (54%), with a clinical improvement in all.
Pachymeningitis with IgG4 and ANCA seems a localized disease to the head and neck. Leptomeningeal biopsy commonly found IgG4 criteria and no vasculitis. All patients responded well to steroid therapy and immunosuppressive drugs, especially rituximab, with clinical and radiological improvement but relapse and/or sequelae are not uncommon.
肥厚性硬脑膜炎是一种罕见的临床疾病，表现为硬脑膜局限性或弥漫性增厚。鉴于硬脑膜炎既属于IgG4相关性疾病的临床范畴，也属于抗中性粒细胞胞浆抗体（ANCA）血管炎（特别是肉芽肿性多血管炎）的范畴，故对重叠综合征进行探讨。
我们报告一例因头痛和颅神经功能障碍而发现的肥厚性硬脑膜炎病例，该病例经活检证实为IgG4相关性硬脑膜炎且MPO-ANCA呈阳性。此外，对既往文献报道的所有合并IgG4相关性疾病和ANCA阳性的硬脑膜炎病例进行了分析。
对13例合并硬脑膜炎、IgG4相关性疾病和ANCA的患者进行了分析。与肥厚性硬脑膜炎相关的IgG4和ANCA患者以男性为主（8例，占62%）。诊断时的中位年龄为64岁。诊断时的主要临床表现局限于头颈部，包括头痛（10例，占77%）、颅神经功能障碍（7例，占54%）、听力障碍（6例，占46%）和眩晕（4例，占31%）。除1例合并弥漫性主动脉炎外，诊断时及随访期间未观察到其他全身表现。血清IgG4常升高（11例，占85%），ANCA主要具有髓过氧化物酶特异性（11例，占85%）。7例患者进行了脑脊液分析，5例（71%）出现淋巴细胞增多，4例（57%）蛋白升高，3例（42%）寡克隆带阳性，1例（14%）葡萄糖降低。在磁共振成像（MRI）上，硬脑膜增厚最常累及后颅窝，共7例（占54%）。在10例有组织学检查结果的病例中，所有病例均显示浆细胞IgG4阳性增加，50%有淋巴细胞浸润，但均未呈现IgG4相关性疾病的三大主要组织学标准。3例（30%）显示血管炎的组织学征象，伴有血管壁损伤和/或巨细胞。在12例接受类固醇治疗的患者中，11例（92%）临床症状改善。4例（33%）在减药过程中复发。7例（54%）二线加用免疫抑制药物，所有患者临床症状均改善。
合并IgG4和ANCA的硬脑膜炎似乎是一种局限于头颈部的疾病。软脑膜活检常见IgG4标准且无血管炎。所有患者对类固醇治疗和免疫抑制药物反应良好，尤其是利妥昔单抗，临床和影像学均有改善，但复发和/或后遗症并不少见。
