MPO-AAV患者临床特点及其肺部受累预后危险因素分析

目的 分析髓过氧化物酶阳性的抗中性粒细胞胞浆抗体相关性血管炎 (myeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis,MPO-AAV)患者临床特点并探讨肺部受累患者呼吸衰竭、肺部感染和死亡的危险因素。方法 收集复旦大学附属中山医院2005年2月至2013年12月初诊入院的MPO-AAV患者基线时临床资料,截至2014年8月或死亡,应用Logistic多因素回归分析肺部受累患者不同预后的独立危险因素。结果 共纳入98例确诊的MPO-AAV患者,早期系统型13例,全身型50例,重型28例,难治型7例。呼吸系统以咳嗽咳痰、胸闷气促为主要表现;胸部X线和高分辨CT(high resolution computed tomography,HRCT)示以渗出 (36例)、肺间质改变 (35例)常见;肺活检示8例肺组织病理学见肺泡间隔破坏、慢性炎性细胞浸润。糖皮质激素治疗92例,免疫抑制剂治疗79例,两种球蛋白治疗19例,免疫吸附5例。治疗后改善者71例,复发者17例。发生急性呼吸衰竭者16例,死亡9例,肺部感染者35例;分别以急性呼吸衰竭、死亡和肺部感染为终点,Logistic回归分析肺部受累患者临床资料发现:肺部感染、肺间质病变、Scr＞500 μmol/L以及ALB<30 g/L是急性呼吸衰竭的危险因素;发病年龄≥65岁、急性呼吸衰竭是患者死亡的危险因素;急性呼吸衰竭是患者肺部感染的危险因素。结论 MPO-AAV患者多有肺部受累,同时伴有全身及血液、肾脏等其他系统受累的异常表现。肺部感染是MPO-AAV肺部受累患者发生急性呼吸衰竭的主要诱发因素,且二者相互促进增加患者死亡风险;发病年龄>65岁是呼吸衰竭患者死亡的危险因素。
Objective To analyze the clinical characteristics of myeloperoxidase anti-neutrophil cytoplasmic antibody-associated vasculitis (MPO-AAV) patients and explore risk factors for respiratory failure,lung infection and death respectively in patients with lung involvement.Methods Patients admitted in Zhongshan hospital,Fudan University from Feb.,2005 to Dec.,2013 were enrolled in this study and followed up to 31st,Aug of 2014 or death.Clinical materials were collected in their first time and during their follow-up period.A multiple Logistic regression analysis was used to analyze risk factors for different outcomes.Results Ninety-eight patients were recruited in this study,who was classified as early systemic (n=27),generalized (n=50),severe (n=28) and refractory disease (n=7).In patients with lung involvement,initial manifestations were cough and chest distress with inflammatory exudation (n=36) and interstitial changes (n=35) as mainly features in high resolution computed tomography (HRCT).Destroyed alveolar septum and chronic inflammatory cells infiltration can be seen in local lung tissues by lung biopsy in 8 patients.Patients were treated with glucocorticoids (n=92),immunosuppressive agents (n=79),IVIG (n=19) and immunoadsorption therapy (n=5).Seventy-one patients were improved,while 17 patients relapsed after treatment.Respiratory failure occurred in 16 patients,lung infection 35 occured in patients, and 9 patients were death during follow-up period.The multiple Logistic regression showed that risk factors for acute respiratory failure included pulmonary infection,interstitial lung disease,Scr>500 μmol/L and ALB<30 g/L.Age at onset ≥65 years and acute respiratory failure were risk factors for death in patients with lung involvement,while acute respiratory failure was a