PDF(Pubmed)
Abstract：
IgG4-related disease is an immune-mediated fibroinflammatory condition. Isolated manifestation in the spine as hypertrophic pachymeningitis is very rare and the mass-like lesion on MRI often mimic tumour or infection. Patients would present with symptoms that result from mass effect or neurovascular compression. Studies showed that serum and CSF IgG4 levels are rarely informative, and therefore, tissue biopsy is crucial for accurate diagnosis. Apart from supporting the diagnosis, MRI is helpful in delineating the extent of disease and follow-up after treatment. A 18F-FDG PET/CT scan is useful in detecting systemic manifestations of IgG4-related disease. Although IgG4-related disease generally responds well to corticosteroid at inflammatory state, relapse is not uncommon. Current treatment strategies for IgG4-related hypertrophic pachymeningitis are high dose corticosteroid therapy and early decompressive surgery to avoid chronic neurological complications. We described a case of a 27-year-old gentleman complaining of lower limb weakness and numbness. MRI showed a mass-like epidural lesion at the thoracic spine causing cord compression. Open biopsy of the epidural mass demonstrated histopathological characteristics of IgG4-related disease. Patient responded well to early surgical decompression of the spinal cord and corticosteroid as evidenced by symptom improvement and resolving mass on subsequent MRI study. However, a follow-up MRI revealed disease recurrence years later.
摘要：
IgG4相关疾病是一种免疫介导的纤维炎性疾病。在脊柱中孤立表现为肥厚性硬脑膜炎非常罕见，MRI上的肿块样病变通常模仿肿瘤或感染。患者会出现由质量效应或神经血管压迫引起的症状。研究表明，血清和CSFIgG4水平很少提供信息，因此,组织活检对于准确诊断至关重要。除了支持诊断，MRI有助于描绘疾病的程度和治疗后的随访。18F-FDGPET/CT扫描可用于检测IgG4相关疾病的全身表现。尽管IgG4相关疾病通常在炎症状态下对皮质类固醇反应良好，复发并不少见。IgG4相关性肥厚性硬脑膜炎的当前治疗策略是大剂量皮质类固醇治疗和早期减压手术以避免慢性神经系统并发症。我们描述了一个27岁的绅士抱怨下肢无力和麻木的案例。MRI显示胸椎有肿块样硬膜外病变，导致脊髓受压。硬膜外肿块的开放活检显示IgG4相关疾病的组织病理学特征。患者对脊髓和皮质类固醇的早期手术减压反应良好，随后的MRI研究中症状改善和肿块消退证明了这一点。然而,随访MRI显示疾病在数年后复发.
