摘要
目的:总结ANCA相关性血管炎(AAV)重叠IgG4相关性疾病(IgG4-RD)病例特点和治疗转归。方法:分析2018年8月至2019年7月复旦大学附属中山医院4例AAV重叠IgG4-RD患者的临床资料,并进行相关文献复习和分析汇总。结果:我中心4例患者中,2例为确诊IgG4-RD重叠肉芽肿性多血管炎(GPA),1例可能IgG4-RD重叠显微镜下多血管炎(MPA),1例可能IgG4-RD重叠GPA。4例患者均为女性,年龄26~56岁,平均(42±12)岁;病程4~13个月,平均(7±4)个月;表现为眼球炎性假瘤、鼻窦炎、中耳炎、乳突炎、涎腺炎,严重者出现硬脑膜炎、肺和肾受累。所有患者用激素与免疫抑制剂(包括环磷酰胺、甲氨蝶呤、硫唑嘌呤、来氟米特)治疗后,2例诱导缓解未成功,2例治疗8~15个月后复发。其中1例激素冲击联合利妥昔单抗,1例激素加量联合甲氨蝶呤和利妥昔单抗治疗,病情得到缓解。结论:AAV重叠IgG4-RD可能是一种新型重叠综合征,文献报道中肥厚性脑膜炎、眼眶肿物、慢性主动脉周围炎和间质性肾炎常见。本报道中眼眶、鼻咽部、腮腺和肺部病变常见,激素和免疫抑制剂治疗效果不佳,可能是一种伴IgG4升高的难治性AAV。该组病例诱导缓解率低,易复发,激素冲击和利妥昔单抗治疗有效。
Objective To summarize the characteristics and treatment outcomes of immunoglobulin G4-related disease(IgG4-RD)overlapped with anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV).Methods The clinical data of four patients with AAV overlaped with IgG4-RD from Zhongshan Hospital of Fudan University from August 2018 to July 2019 were collected and the related literature were reviewed.Results Four patients were included,in which two were diagnosed with IgG-RD and granulomatosis with polyangiitis(GPA),one was probable IgG4-RD and microscopic polyangiitis(MPA),and one was probable IgG4-RD and GPA.All patients were female,with an average age of(42±12)(26-56)years,and disease duration was(7±4)(4-13)months.The manifestations were ocular inflammatory pseudotumor,sinusitis,otitis media,mastoiditis,parotitis,meningitis,lung and kidney involvement.After treatment with glucocorticoid and immunosuppressants(including cyclophosphamide,methotrexate,azathioprine,leflunomide),2 patients failed to achieve remission,and 2 patients relapsed 8-15 months after treatment.One patient was treated withglucocorticoid pulse therapy combined with rituximab and one was treated with glucocorticoid combined with methotrexate and rituximab,and the patient was relieved.Conclusion AAV and IgG4-RD may be a new overlap syndrome.Hypertrophic meningitis,orbital mass,chronic periaortic inflammation and interstitial glomerulonephritis are reported in the literature.The pathological changes of orbit,nasopharynx,parotid gland and lung are common.Glucocorticoids and immunosuppressive agents have poor treatment response,which indicates that AAV is refractory when combined with elevated IgG4.The induced remission rate is low,and easy to relapse.Glucocorticoid pulse therapy and rituximab treatment are effective.
作者
张丽娟
纪宗斐
姜林娣
Zhang Lijuan;Ji Zongfei;Jiang Lindi(Department of Rheumatology and Immunology,Xiamen Hospital,Zhongshan Hospital,Fudan University,Xiamen 361000,China;Department of Rheumatology and Immunology,Zhongshan Hospital,Fudan University,Shanghai,200032,China)
出处
《中华风湿病学杂志》
CAS
CSCD
北大核心
2020年第4期268-272,I0003,共6页
Chinese Journal of Rheumatology
基金
国家自然科学基金面上项目(81774114)
国家中医药管理局区域中医(专科)诊疗中心建设项目-风湿病科
上海市中西医临宋协作试点项目[ZY(2018-2020)-FWTX-1010]
上海市中医专科联盟建设项目[ZY(2018-2020)-FWTX-4017]
上海市长宁区卫生系统医学特色专科建设项目(20162003)
浦江风湿青年培育计划(SPROG201805)。
