Chen Yuxue, Liu Lu, Xie Cuihong
Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, People's Republic of China.
Department of Pharmacy, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, People's Republic of China.
J Inflamm Res. 2025 May 24;18:6673-6680. doi: 10.2147/JIR.S521138. eCollection 2025.
Hypertrophic pachymeningitis (HP) is a rare and chronic clinical disease characterized by thickening of the dura mater, leading to persistent headache, cranial neuropathy, seizures, and other neurological symptoms. Immune-mediated causes, particularly antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and IgG4-related disease (IgG4-RD), are among the most common etiologies. We report a case of a 54-year-old female with recurrent headache, blepharoptosis, hearing loss, and markedly elevated inflammatory markers. Blood tests, and serum levels of IgG4 were within normal ranges. Contrast enhanced cranial MRI revealed thickening and enhancement of bilateral cerebral hemispheres and tentorial dural maters. Additional findings included mild left lacrimal gland enlargement, bilateral middle ear mastoiditis, and tympanic tegmen destruction. Abdominal high-resolution computed tomography (CT) showed enlarged retroperitoneal lymph nodes. Histopathology demonstrated dense lymphoplasmacytic and neutrophilic infiltration with 80 IgG4-positive plasma cells per high-power field and an IgG4/IgG cell ratio of 20%. An initial diagnosis of possible IgG4-RD was made. However, the patient's symptoms responded poorly to prednisolone (20 mg/day), and fever ensued. P, and were found in the cerebrospinal fluid measured by NGS. Subsequent laboratory testing showed positive p-ANCA and anti-myeloperoxidase antibodies (anti-MPO), with a negative anti-nuclear antibodies panel, leading to a revised diagnosis of MPO-ANCA-associated HP. Treatment was escalated to intravenous methylprednisolone (40 mg/day), cyclophosphamide, and anti-infectious agents, leading to improved symptoms and decreased inflammatory markers. However, there was a recurrence during the taper of prednisolone. The addition of rituximab achieved complete remission. MPO-ANCA-associated HP is a rare inflammatory disorder that brings diagnostic challenges and requires comprehensive differential diagnosis. In relapsed or refractory cases, rituximab may be a valuable therapeutical option.
肥厚性硬脑膜炎（HP）是一种罕见的慢性临床疾病，其特征为硬脑膜增厚，可导致持续性头痛、颅神经病变、癫痫发作及其他神经症状。免疫介导的病因，尤其是抗中性粒细胞胞浆抗体（ANCA）相关血管炎和IgG4相关性疾病（IgG4-RD），是最常见的病因。我们报告一例54岁女性，有反复头痛、上睑下垂、听力丧失及炎症标志物显著升高。血液检查及血清IgG4水平均在正常范围内。增强头颅磁共振成像（MRI）显示双侧大脑半球及小脑幕硬脑膜增厚并强化。其他发现包括左侧泪腺轻度肿大、双侧中耳乳突炎及鼓室盖破坏。腹部高分辨率计算机断层扫描（CT）显示腹膜后淋巴结肿大。组织病理学显示密集的淋巴细胞、浆细胞及中性粒细胞浸润，每高倍视野有80个IgG4阳性浆细胞，IgG4/IgG细胞比率为20%。初步诊断为可能的IgG4-RD。然而，患者症状对泼尼松龙（20mg/天）反应不佳，继而出现发热。通过二代测序（NGS）在脑脊液中检测到P和 。随后的实验室检查显示核周型抗中性粒细胞胞浆抗体（p-ANCA）和抗髓过氧化物酶抗体（抗MPO）阳性，抗核抗体谱阴性，从而将诊断修订为MPO-ANCA相关的HP。治疗升级为静脉注射甲泼尼龙（40mg/天）、环磷酰胺及抗感染药物，症状改善，炎症标志物下降。然而，在泼尼松龙减量过程中病情复发。加用利妥昔单抗后实现完全缓解。MPO-ANCA相关的HP是一种罕见的炎症性疾病，带来诊断挑战，需要全面的鉴别诊断。在复发或难治性病例中，利妥昔单抗可能是一种有价值的治疗选择。
