MPO-ANCA相关性肥厚性硬脑膜炎合并肉芽肿性血管炎临床分析Clinical analysis of myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic cranial pachymeningitis with granulomatosis with polyangiitis
陈怡玮;彭涛;刘希;胡文涛;石珂;王梦涵;李俊敏;卢宏;
摘要(Abstract):
目的探讨髓过氧化物酶抗中性粒细胞胞质抗体(MPO-ANCA)相关性肥厚性硬脑膜炎(HCP)合并肉芽肿性血管炎(GPA)的临床特点、治疗及预后。方法回顾性分析2016-06—2020-06郑州大学第一附属医院神经内科住院治疗的MPO-ANCA相关性HCP合并GPA患者的临床资料。结果 5例患者均为亚急性或慢性起病,病程1个月~3 a,首发症状主要为头痛5例,脑神经受损2例。首次入院时所有患者核周型-抗中性粒细胞胞浆抗体(p-ANCA)阳性,MPO-ANCA浓度及非特异性炎症指标升高,胞质型抗中性粒细胞胞质抗体(c-ANCA)和蛋白酶3抗中性粒细胞胞质抗体(PR3-ANCA)正常,2例尿常规隐血阳性,24 h尿蛋白总量升高,颅脑MRI增强扫描均可见不同部位的硬脑膜强化及鼻窦炎,3例伴乳突炎。5例患者糖皮质激素治疗均有效,4例病情反复,3例联用免疫抑制剂治疗后症状缓解。结论 MPO-ANCA相关性HCP合并GPA病例多伴鼻窦炎、乳突炎、尿隐血、尿蛋白等,应早期给予糖皮质激素联合免疫抑制剂治疗,避免疾病向全身型发展。
关键词(KeyWords): 髓过氧化物酶;抗中性粒细胞胞质抗体;肥厚性硬脑膜炎;肉芽肿性血管炎
基金项目(Foundation): 国家自然科学基金(编号:81971175)
作者(Authors): 陈怡玮;彭涛;刘希;胡文涛;石珂;王梦涵;李俊敏;卢宏;
参考文献(References):
- [1]KUPERSMITH M J,MARTIN V,HELLER G,et al.Idiopathic hypertrophic pachymeningitis[J].Neurology,2004,62(5):686-694.DOI:10.1212/01.wnl.0000113748.53023.b7.
- [2]YONEKAWA T,MURAI H,UTSUKI S,et al.A nationwide survey of hypertrophic pachymeningitis in Japan[J].J Neurol Neurosurg Psychiatry,2014,85(7):732-739.DOI:10.1136/jnnp-2013-306410.
- [3]JENNETTE J C,FALK R J,BACON P A,et al.2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides[J].Arthritis Rheum,2013,65(1):1-11.DOI:10.1002/art.37715.
- [4]WATTS R,LANE S,HANSLIK T,et al.Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies[J].Ann Rheum Dis,2007,66(2):222-227.DOI:10.1136/ard.2006.054593.
- [5]LEAVITT R Y,FAUCI A S,BLOCH D A,et al.The American College of Rheumatology 1990 criteria for the classification of wegener's granulomatosis[J].Arthritis Rheum,1990,33(8):1101-1107.DOI:10.1002/art.1780330807.
- [6]LANHAM J G,ELKON K B,PUSEY C D,et al.Systemic Vasculitis with Asthma and Eosinophilia:A Clinical Approach to the Churg-Strauss Syndrome[J].Medicine(Baltimore),1984,63(2):65-81.
- [7]JENNETTE J C,FALK R J,ANDRASSY K,et al.Nomenclature of Systemic Vasculitides[J].Arthritis Rheum,1994,37(2):187-192.DOI:10.1002/art.1780370206.
- [8]IMAFUKU A,SAWA N,KAWADA M,et al.Incidence and risk factors of new-onset hypertrophic pachymeningitis in patients with anti-neutrophil antibody-associated vasculitis:using logistic regression and classification tree analysis[J].Clin Rheumatol,2019,38(4):1039-1046.DOI:10.1007/s10067-018-4372-z.
- [9]YOKOSEKI A,SAJI E,ARAKAWA M,et al.Hypertrophic pachymeningitis:significance of myeloperoxidase anti-neutrophil cytoplasmic antibody[J].Brain,2014,137(Pt 2):520-536.DOI:10.1093/brain/awt314.
- [10]SAKAIRI T,SAKURAI N,NAKASATOMI M,et al.Hypertrophic pachymeningitis associated with antineutrophil cytoplasmic antibody-associated vasculitis:a case series of 15 patients[J].Scand J Rheumatol,2019,48(3):218-224.DOI:10.1080/03009742.2018.1498916.
- [11]SHIMOJIMA Y,KISHIDA D,HINENO A,et al.Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis:A retrospective study of anti-neutrophil cytoplasmic antibody-associated vasculitis[J].Int J Rheum Dis,2017,20(4):489-496.DOI:10.1111/1756-185x.13046.
- [12]CHOI H A,LEE M J,CHUNG C S.Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis[J].J Neurol,2017,264(4):724-732.DOI:10.1007/s00415-017-8416-0.
- [13]李建章.中枢神经系统血管炎的共性及诊断标准商讨[J].中国实用神经疾病杂志,2017,20(5):1-2.DOI:10.3969/j.issn.1673-5110.2017.05.001.
- [14]SHIMOJIMA Y,KISHIDA D,SEKIJIMA Y.Increased BAFF and APRIL levels in the cerebrospinal fluid of patients with anti-neutrophil cytoplasmic antibody-related hypertrophic pachymeningitis[J].Cytokine,2017,99:305-309.DOI:10.1016/j.cyto.2017.08.013.
- [15]SZANTO A,NAGY G,MOLNAR C S,et al.Description of patients with IgG4-related disease from a Hungarian centre[J].Scand J Rheumatol,2014,43(4):334-337.DOI:10.3109/03009742.2013.862567.
- [16]POPKIROV S,KOWALSKI T,SCHLEGEL U,et al.Immunoglobulin-G4-related hypertrophic pachymeningitis with antineutrophil cytoplasmatic antibodies effectively treated with rituximab[J].J Clin Neurosci,2015,22(6):1038-1040.DOI:10.1016/j.jocn.2014.12.020.
- [17]XU W L,LING Y C,WANG Z K,et al.Diagnostic performance of serum IgG4 level for IgG4-related disease:a meta-analysis[J].Sci Rep,2016,6:32035.DOI:10.1038/srep32035.
- [18]LUTALO P M,D'CRUZ D P.Diagnosis and classification of granulomatosis with polyangiitis(aka Wegener's granulomatosis)[J].J Autoimmun,2014,48-49:94-98.DOI:10.1016/j.jaut.2014.01.028.
- [19]SEROR R,MAHR A,RAMANOELINA J,et al.Central nervous system involvement in Wegener granulomatosis[J].Medicine(Baltimore),2006,85(1):54-65.DOI:10.1097/01.md.0000200166.90373.41.
- [20]JAYNE D.The diagnosis of vasculitis[J].Best Pract Res Clin Rheumatol,2009,23(3):445-453.DOI:10.1016/j.berh.2009.03.001.
- [21]CHEN H,ZHANG W,JING J,et al.The clinical and imaging features of hypertrophic pachymeningitis:a clinical analysis on 22 patients[J].Neurol Sci,2019,40(2):269-274.DOI:10.1007/s10072-018-3619-4.
- [22]WATANABE K,TANI Y,KIMURA H,et al.Hypertrophic cranial pachymeningitis in MPO-ANCA-related vasculitis:a case report and literature review[J].Fukushima J Med Sci,2013,59(1):56-62.DOI:10.5387/fms.59.56.
- [23]HASEGAWA H,KOHSAKA H,TAKADA K,et al.Renal involvement in antimyeloperoxidase antineutrophil cytoplasmic antibody-positive granulomatosis with polyangiitis with chronic hypertrophic pachymeningitis[J].JRheumatol,2012,39(10):2053-2055.DOI:10.3899/jrheum.120473.
- [24]BARBIERI F R,NOVEGNO F,IAQUINANDI A,et al.Hypertrophic Pachymeningitis and Hydrocephalus-The Role of Neuroendoscopy:Case Report and Review of the Literature[J].World Neurosurg,2018,119:183-188.DOI:10.1016/j.wneu.2018.07.194.
- [25]DI STEFANO V,DONO F,DE ANGELIS M V,et al.Hypertrophic pachymeningitis and cerebral venous thrombosis in myeloperoxidase-ANCA associated vasculitis[J].BMJ Case Rep,2019,12(1).DOI:10.1136/bcr-2018-226780.
- [26]MUKHTYAR C,GUILLEVIN L,CID M C,et al.EU-LAR recommendations for the management of primary small and medium vessel vasculitis[J].Ann Rheum Dis,2009,68(3):310-317.DOI:10.1136/ard.2008.088096.
- [27]STONE J H,MERKEL P A,SPIERA R,et al.Rituximab versus cyclophosphamide for ANCA-associated vasculitis[J].N Engl J Med,2010,363(3):221-232.DOI:10.1056/NEJMoa0909905.
- [28]HAHN L D,FULBRIGHT R,BAEHRING J M.Hypertrophic pachymeningitis[J].J Neurol Sci,2016,367:278-283.DOI:10.1016/j.jns.2016.06.024.
- [29]KAIEDA S,YOSHIDA N,MINEZAKI M,et al.The Successful Treatment of Myeloperoxidase Antineutrophil Cytoplasmic Antibody-positive Hypertrophic Pachymeningitis in Patients with the Limited Form of Granulomatosis with Polyangiitis Using Methotrexate:Two Case Reports[J].Intern Med,2017,56(8):959-965.DOI:10.2169/internalmedicine.56.7742.
- [30]SHARMA A,KUMAR S,WANCHU A,et al.Successful treatment of hypertrophic pachymeningitis in refractory Wegener's granulomatosis with rituximab[J].Clin Rheumatol,2010,29(1):107-110.DOI:10.1007/s10067-009-1291-z.