原发性抗中性粒细胞胞浆抗体相关性血管炎伴弥漫性间质性肺疾病临床特点分析  

Clinical Characteristis of Primary Antineutrophil Cytoplasmic Antibody-associated Vasculitis with Diffuse Interstitial Lung Diseases

作　　者：孙越[1] 方秋红[1] 马迎民[1] 原庆[1] 黄爱本[1] 

机构地区：[1]首都医科大学附属北京世纪坛医院呼吸内科,北京市100038

出　　处：《中国全科医学》2013年第16期1868-1871,共4页Chinese General Practice

基　　金：2008年北京市优秀人才培养资助个人项目(20081D0303100103)

摘　　要：目的探讨原发性抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AASV)伴弥漫性间质性肺疾病(DILD)的临床特点。方法回顾性分析42例AASV-DILD患者的临床资料,将上述患者分为以呼吸道症状及非呼吸道症状为主要临床表现的A组和B组,对两组资料进行比较。结果 (1)A组24例,B组18例,临床症状两组相比咳嗽(17/24比1/18)、气短(12/24比0)、蛋白尿血尿并水肿(3/24比14/18)、关节肌肉疼痛(1/24比6/18)发生率差异均有统计学意义(P<0.05)。(2)A、B两组年龄、病程、血气分析、肺功能检查、支气管镜检查、抗核抗体滴度、ANCA阳性率及类别差异均无统计学意义(P>0.05)。(3)30例患者发生肾功能衰竭(RF),急性肾功能衰竭(ARF)发生率为14/42;慢性肾功能衰竭(CRF)发生率B组为11/18,高于A组发生率5/24,差异有统计学意义(P<0.05)。(4)8例患者超声心动图提示肺心病(PHD)表现,A组发生率(7/13)高于B组(1/11),差异有统计学意义(P<0.05)。(5)8例患者行经支气管镜肺活检(TBLB),2例提示小血管炎病理改变;11例支气管肺泡灌洗液(BALF)提示中性粒细胞百分比增高。(6)33例行高分辨CT(HRCT)检查,主要影像学表现为网格影、蜂窝肺、磨玻璃影、小叶间隔增厚,磨玻璃影两组发生率(5/21比9/12)差异有统计学意义(P<0.05)。结论 (1)AASV-DILD患者表现为多脏器受累,当以非呼吸系统症状为主要表现时,呼吸系统病变易漏诊。(2)AASV-DILD除肺脏外,肾脏是最易受累的脏器,有较高的ARF发生率,CRF多见于以肾脏受累为主要临床症状患者。(3)PHD多发生于以呼吸系统症状为主的患者。(4)AASV-DILD患者BALF以中性粒细胞增高为主,TBLB在诊断AASV中有一定价值。(5)AASV-DILD影像学无特征性,应常规进行ANCA检测,避免漏诊。Objective To explore the features of primary antineutrophil cytoplasmic antibody - associated vasculitis with diffuse interstitial lung diseases （AASV -DILD） . Methods The clinical data of 42 patients with AASV - DILD were retrospectively analyzed, and the data were compared between patients whose predominant clinical presentations were respiratory symptoms （ group A） and patients whose were non - respiratory symptoms （ group B） . Results （ 1 ） The predominant symp- toms of 24 cases in group A and 18 case in group B were cough （17/24 vs. 1/18）, shortness of breath （12/24 vs. 0）, pro- teinuria and hematuria with edema （3/24 vs. 14/18）, articular and muscular pain （1/24 vs. 6/18 ）, with statistically signifi- cant differences （ P 〈 0. 05 ）. （2） No significant differences was found between groups A and B in age, time of disease attack ; and results of blood gas, pulmonary function test, bronchoscopy examination, bronchoalveolar lavage fluid （ BALF）, antinucle- ar antibody titer, antineutrophil cytoplasmic antibody titer （P 〉 0. 05 ）. （3） Totally 30 patients got renal failure （RF）, and the incidence of acute case （ARF） was 14/42. The incidence of chronic case （CRF） was 11/18 in group B, being significantly higher than 5/24 in group A （P 〈 0. 05）. （4） There were 8 patients whose ultrasound cardiogram revealed pulmonary heart dis- ease, with the incidence of group A （7/13） being significantly higher that group B （1/11） （P 〈 O. 05）. （5） Eight patients un- derwent transbronchial lung biopsy, which revealed small vasculitis in 2. And there were 11 patients whose BALF showed high percentage of neutrophils. （6） Thirty - three patients received high resolution CT test, with the main imaging manifestations be- ing reticular opacities, honey combing, ground - glass opacity （ GGO）, and interlobular septa thickening. The incidence ofGGO was more often in group B （5/21 vs. 9/12） （P 〈 0. 05 ）. Conclusion （1） AASV -

关 键 词：抗体 抗中性白细胞胞质 肺疾病 间质性 血管炎 疾病特征 

分 类 号：R54[医药卫生—心血管疾病] R563.9[医药卫生—内科学]