老年ANCA相关性小血管炎肺损害11例临床分析

目的探讨老年抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎肺脏损害的临床特征,提高对其肺损害的认识。方法回顾性分析北京大学第一医院老年科60岁以上的ANCA相关性小血管炎肺损害患者的临床病理资料。结果11例ANCA相关性小血管炎肺脏损害患者平均年龄75.9(60～89)岁,均有慢性基础病。发病至确诊的中位间隔时间为4个月(2周～1年)。小血管炎临床症状多为咳嗽、咯痰(90.9%);喘憋气短或活动耐力下降(63.6%);咯血/痰中带血(36.4%)和发热(45.5%)。胸部影像学9例(91.9%)表现为肺间质病变,其中8例呈显著肺纤维化表现;2例(18.2%)呈两肺大片渗出的弥漫肺泡出血表现(其中1例在肺纤维化基础上发生);1例呈结节性病变(9.1%)。8例(72.7%)患者初诊时无肾损害。确诊主要依靠血清ANCA的检测和组织活检病理诊断。经免疫抑制治疗后,多数患者症状好转,初诊及随访期间5例(45.5%)因呼吸衰竭或严重感染死亡。结论原发性ANCA相关性小血管炎患者常有肺部受累并可以为首发表现。其症状和胸部影像表现多样,肺间质病变突出。正确及时的诊断治疗能改善预后。

Clinical analysis of ANCA associated vasculitis with pulmonary damage in 11 cases

Objective To retrospectively analyze the clinical features of anti-neutrophil cytoplasmic antibody （ANCA） associated vasculitis with pulmonary damage in elderly patients in an attempt to raise the awareness of this disease. Methods Clinical data of ANCA associated vasculitis with pulmonary damage in elderly patients （〉~ 60yr） were retrospectively analyzed. Results The average age of the 11elderly patients was 75.9 （60-89） years, and all had chronic underlying diseases. The median interval between onset and the diagnosis was 4 months （2 weeks- 1 year）. The common symptoms of vasculitis were cough and expectoration （90.9%）, dyspnea or exertion intolerance （63.6%）, hemoptysis （36.4%）, and fever （45.5%）. The most common imaging manifestations were interstitial pulmonary lesions （91.9%）, which were found to be evident interstitial pulmonary fibrosis in 8 cases. Two cases displayed multiple large patches of exudative shadow, indicating diffuse alveolar hemorrhage. One case had nodule lesions. Renal damage was not detected at presentation in 8 cases （72.7%）. Diagnosis was confirmed by serum ANCA assay and tissue biopsy. Although the majority achieved clinical remission after immunosuppressive therapy, 5 cases （45.5%） died of respiratory failure or severe infections within the induction therapy period or during follow-up. Conclusion Pulmonary involvement in patients with ANCA associated vasculitis was common and may be the first presentation. The symptoms and radiological manifestations of pulmonary involvement were variable, and interstitial pulmonary lesions were notable. Early diagnosis and appropriate treatment may improve the prognosis.