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目的抗中性粒细胞胞质抗体相关性血管炎(ASV)是西方国家常见的结缔组织疾病,我国人群中ASV并不少见,本文总结了我院的有关病例特点及治疗。方法总结分析我院确诊ASV患者的临床表现、实验室检查、诊治和预后。结果16例ASV患者,确诊MPA11例,WG5例,从发病到确诊平均(8±5)个月,88%的患者肾脏受累,50%肺脏受累,还有其他鼻、耳、眼、声带、关节等各器官受累的表现,常被误诊。治疗以皮质激素和环磷酰胺为主,14例ASV经治疗总缓解率达79%。结论ASV可累及各年龄层,病程进展迅速,多脏器系统性病变发生率高,易误诊,早期诊治是提高该病生存率的关键。
Objective Anti-neutrophil cytoplasmic antibody-associated vasculitis (ASV) is a common connective tissue disease in western countries. It is not uncommon for ASV in our population. This article summarizes the characteristics and treatment of related cases in our hospital. Methods The clinical manifestations, laboratory tests, diagnosis and treatment and prognosis of ASV patients diagnosed in our hospital were summarized. Results In 16 patients with ASV, 11 cases were diagnosed as MPA and 5 cases were treated with WG. The average time from onset to diagnosis was (8 ± 5) months. 88% of the patients had kidney involvement, 50% of lung involvement, and other nasal, Other organs involved in the performance, often misdiagnosed. Treatment with corticosteroids and cyclophosphamide, 14 cases of ASV treatment response rate of 79%. Conclusions ASV can affect all age groups, with rapid progression, multiple organ diseases, high misdiagnosis and early diagnosis and treatment, which are the keys to improve the survival rate of ASD.