Wang Jianchun, Xu Feifan, Duan Hongzhou, Zhao Juan, Zhang Haoze, Ren Jingru, Niu Zhenyu, Gao Feng, Chen Siwei, Liu Ran
Department of Neurology, Peking University First Hospital, Beijing, China.
Department of Neurosurgery, Peking University First Hospital, Beijing, China.
Front Immunol. 2025 Aug 29;16:1619756. doi: 10.3389/fimmu.2025.1619756. eCollection 2025.
Hypertrophic spinal pachymeningitis (HSP) and retroperitoneal fibrosis (RPF) are rare inflammatory disorders, often associated with immunoglobulin G4-related disease (IgG4-RD). This case underscores the diagnostic intricacies and therapeutic challenges in a patient with overlapping neurological and systemic manifestations.
A 34-year-old female presented with concurrent RPF and HSP leading to urinary tract obstruction and progressive spinal cord compression. The patient initially presented with lower limb weakness and urinary dysfunction, followed by acute paraparesis after a fall. Despite normal serum IgG4 levels, a pathological examination of the dural biopsy confirmed the diagnosis of IgG4-RD. Following a multidisciplinary treatment approach that included surgical decompression, glucocorticoids, immunomodulators and antifibrotic therapy, the patient achieved a favorable clinical outcome.
A systematic review of 22 cases involving patients with HSP revealed that key manifestations included localized pain, motor deficits, sensory abnormalities and autonomic dysfunction. The majority of patients (82%, 18/22) exhibited isolated HSP, with a predominance of thoracic spine involvement. Pathological examination demonstrated lymphoplasmacytic infiltration in all cases (100%, 22/22), with 95% (19/20) meeting the criteria for IgG4-positive plasma cells; storiform fibrosis and obliterative phlebitis were observed in 56% (5/9) of cases. Treatment strategies primarily involved surgical decompression (95%, 21/22) and glucocorticoids (95%, 21/22), with 52% (11/21) receiving additional immunosuppressive agents. Clinical outcomes showed complete neurological recovery in 19% (4/21), partial recovery in 71% (15/21). Recurrence was documented in 17% (3/18) of patients with available follow-up data.
This rare case underscores the importance of integrating clinical, radiological, and histopathological findings to diagnose HSP and RPF, particularly in the context of IgG4-RD. Early multidisciplinary management is critical to improving outcomes.
肥厚性硬脊膜脊髓炎（HSP）和腹膜后纤维化（RPF）是罕见的炎症性疾病，常与免疫球蛋白G4相关性疾病（IgG4-RD）相关。本病例强调了一名具有重叠神经和全身表现患者的诊断复杂性和治疗挑战。
一名34岁女性同时患有RPF和HSP，导致尿路梗阻和进行性脊髓压迫。患者最初表现为下肢无力和排尿功能障碍，跌倒后出现急性截瘫。尽管血清IgG4水平正常，但硬脊膜活检的病理检查确诊为IgG4-RD。采用包括手术减压、糖皮质激素、免疫调节剂和抗纤维化治疗在内的多学科治疗方法后，患者取得了良好的临床结局。
对22例HSP患者的系统评价显示，主要表现包括局部疼痛、运动功能障碍、感觉异常和自主神经功能障碍。大多数患者（82%，18/22）表现为孤立性HSP，以胸椎受累为主。病理检查显示所有病例（100%，22/22）均有淋巴细胞浆细胞浸润，95%（19/20）符合IgG4阳性浆细胞标准；56%（5/9）的病例观察到席纹状纤维化和闭塞性静脉炎。治疗策略主要包括手术减压（95%，21/22）和糖皮质激素（95%，21/22），52%（11/21）的患者接受了额外的免疫抑制剂治疗。临床结局显示19%（4/21）的患者神经功能完全恢复，71%（15/21）的患者部分恢复。有随访数据的患者中，17%（3/18）记录有复发。
这个罕见病例强调了整合临床、影像学和组织病理学检查结果以诊断HSP和RPF的重要性，特别是在IgG4-RD的背景下。早期多学科管理对于改善结局至关重要。
