Guan W J, Ding Y X, Liu L J, Li W, Jing L J, Zhang X, Zhang L J, Li H, Cheng S H, Liu S Y
Department of Rheumatology and Immunology, First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Department of Neurology, First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Zhonghua Nei Ke Za Zhi. 2022 May 1;61(5):565-569. doi: 10.3760/cma.j.cn112138-20210701-00455.
To study the clinical features of myeloperoxidase(MPO) antineutrophil cytoplasmic antibody (ANCA) associated hypertrophic pachymeningitis (HP). Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed. Nine cases were males and the other 6 were females, with an average age of (58±8) years. All cases presented with chronic headache. Contrast-enhanced magnetic resonance imaging (MRI) scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal. Nine cases developed multiple cranial nerve paralysis, with trigeminal nerve and auditory nerve involved most commonly. The main clinical manifestations were facial pain, hearing loss and tinnitus. Two cases were complicated with hypertrophic spinal pachymeningitis (HSP) and 4 cases were complicated with pulmonary diseases. Positive serum perinuclear pattern ANCA (pANCA) and MPO could be found in all cases, positive serum IgG was seen in two patients. erythrocyte sedimentation rate(ESR;25-116 mm/1h) and C-reactive protein (CRP;29.02-146.00 mg/L) were both elevated in 14 cases. Nine cases had elevated intracranial pressure[180-235 mmHO (1 mmHO=0.009 8 kPa)] and abnormal protein level (457.6-3710.0 mg/L) in cerebrospinal fluid. Six cases were treated with glucocorticoids (prednisone 20-60 mg/d) and 9 cased with glucocorticoids and immunosuppressants (methotrexate 15 mg/week or cyclophosphamide 100 mg/d po). All patients achieved remission. MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis (AAV). It rarely involves the lung or kidney. Steroids and immunosuppressive agents are effective. In HP with unknown underlying diseases, it is suggested to screen ANCA and IgG tests for AAV or IgG-related disease.
研究髓过氧化物酶（MPO）抗中性粒细胞胞浆抗体（ANCA）相关肥厚性硬脑膜炎（HP）的临床特征。回顾性分析15例诊断为MPO-ANCA血管炎并发HP的患者的临床资料。9例为男性，6例为女性，平均年龄（58±8）岁。所有病例均有慢性头痛。对比增强磁共振成像（MRI）扫描显示脑和/或脊髓硬脑膜局部或弥漫性增厚，而脑实质正常。9例出现多发性颅神经麻痹，最常累及三叉神经和听神经。主要临床表现为面部疼痛、听力丧失和耳鸣。2例并发肥厚性脊髓硬脑膜炎（HSP），4例并发肺部疾病。所有病例血清核周型ANCA（pANCA）和MPO均为阳性，2例患者血清IgG阳性。14例患者红细胞沉降率（ESR；25-116mm/1h）和C反应蛋白（CRP；29.02-146.00mg/L）均升高。9例患者颅内压升高[180-235mmH₂O（1mmH₂O = 0.009 8kPa）]，脑脊液蛋白水平异常（457.6-3710.0mg/L）。6例患者接受糖皮质激素治疗（泼尼松20-60mg/d），9例患者接受糖皮质激素和免疫抑制剂治疗（甲氨蝶呤15mg/周或环磷酰胺100mg/d口服）。所有患者均缓解。MPO-ANCA相关HP是ANCA相关血管炎（AAV）中一种特殊类型的中枢神经系统受累情况。它很少累及肺或肾。类固醇和免疫抑制剂治疗有效。对于潜在疾病不明的HP患者，建议筛查ANCA和IgG以排查AAV或IgG相关疾病。
