Clinical analysis of 15 cases with myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis
10.3760/cma.j.cn112138-20210701-00455
- VernacularTitle:髓过氧化物酶抗中性粒细胞胞质抗体相关肥厚性硬膜炎15例临床分析
- Author:
Wenjuan GUAN
1
;
Yanxia DING
;
Lijun LIU
;
Wei LI
;
Lijun JING
;
Xin ZHANG
;
Lijuan ZHANG
;
Hui LI
;
Shuhua CHENG
;
Shengyun LIU
Author Information
1. 郑州大学第一附属医院风湿免疫科，郑州450052
- Keywords:
Hypertrophic pachymeningitis;
Myeloperoxidase;
Antineutrophil cytoplasmic antibody;
Vasculitis
- From:
Chinese Journal of Internal Medicine
2022;61(5):565-569
- CountryChina
- Language:Chinese
-
Abstract:
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 4 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 mmH 2O (1 mmH 2O=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 4 tests for AAV or IgG 4-related disease.
