以肺部病变为主要表现的ANCA相关性血管炎10例临床分析

摘要

目的 从呼吸系统疾病的角度分析和认识ANCA相关性血管炎(ANCA-associated Vasculitis,AAV).方法 回顾性分析我院确诊的10例以呼吸系统病变为主要表现的AAV患者的临床资料,对患者的临床表现、实验室检查、影像学表现以及其他特殊检查结果进行汇总分析,采用五因子评分系统评估预后.结果 本组患者显微镜下多血管炎(microscopic polyangiitis,MPA)7例,肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA)2例,嗜酸性肉芽肿性血管炎(eosinophilic granulomatosis with polyangiitis,EGPA)1例,男性3例,女性7例.咳嗽咳痰咯血及呼吸困难是AAV最常见的呼吸系统症状,部分患者合并其他系统特别是肾脏累及.实验室检查MPA均有pANCA和MPO-ANCA阳性,GPA和EGPA患者有cANCA和PR3-ANCA阳性,7例患者有CRP升高(82.18±48.67mg/L),10例患者均有血沉升高(62.9±13.5mm/h);仅有4例患者出现肌酐轻度升高(139±29.3umol/L),3例患者淋巴细胞总数降低,提示继发感染风险大,预后欠佳.影像学表现多表现为双肺磨玻璃斑片影、间质改变以及蜂窝肺表现,对诊断具有提示作用.肺功能检查表现为限制性通气功能障碍,部分患者出现肺动脉高压.结论 肺部是AAV的常见累及器官,部分患者以肺部病变为主要表现,呼吸科医生应加强对AAV的认识,提高诊断率,减少误诊和漏诊.%Objective To investigate how to diagnose and treat AAV patients with pulmonary involvement as primary manifestation.Methods The clinical data of 10 AAV patients whose primary manifestations were respiratory involvement were retrospectively analyzed, including clinical manifestation, laboratory examination, imaging features and other results of special checks.Their prognosis was evaluated by 5-factore scoring system.Results Among the 10 AAV patients, 7 cases were microscopic polyangiitis (MPA), 2 cases were granulomatosis with polyangiitis (GPA), and 1 case was eosinophilic granulomatosis with polyangiitis (EGPA).3 were male and 7 female.Cough, sputum, hemoptysis and dyspnea were the most common symptoms.Some patients were complicated with renal and other system involvement.pANCA and MPO-ANCA were positive in every MPA patient, and cANCA and PR3-ANCA were positive in GPA and EGPA patients.Laboratory tests showed elevated C-reactive protein in 7 patients (82.18±48.67mg/L), and erythrocyte sedimentation rate (ESR) in all patients (62.9±13.5mm/h).Only 4 patients had mildly elevated serum creatinine (139±29.3umol/L).6 patients ran lymphocyte count tests, and 3 of them lymphocyte count decreased which was a sign of high risk of secondary infection and bad prognosis.CT scans showed ground glass opacity, reticular shadows and honeycomb lesions, which could be helpful for diagnosis.Pulmonary function test showed restrictive pattern of ventilation.Echocardiography revealed that some patients had mild pulmonary artery hypertension (3 in 5 patients).Conclusion Most AAV patients have respiratory involvement, and lung diseases are the primary or only manifestations in some of them.Pulmonary physicians should improve their understanding about the disease, to avoid misdiagnosis.