坏死性肉芽肿性血管炎肺损害的临床分析

目的 回顾性分析坏死性肉芽肿性血管炎（ NGV ） 肺损害的临床及影像资料, 提高对本病的认识。方法 对2009 年3 月至2013 年3 月南京大学医学院附属鼓楼医院确诊有肺损害的20 例NGV 患者的临床及影像学资料进行回顾性分析。结果 男11 例, 女9 例; 发病年龄18 ～76 岁,平均（ 44. 7 ±15） 岁。可累及多个系统或器官, 以肺脏受累最常见, 占100% , 症状主要为咳嗽、咳痰、咯血、呼吸困难等。其次鼻部受累85% , 肾脏受累85% , 关节肌肉受累35% , 耳受累40%, 眼受累20% 。肺部影像学表现多样, 以多发结节/ 肿块影伴或不伴空洞病灶为主（ 65% ） , 亦可呈实变影及斑片影, 易被误诊。最常见的病理表现为坏死性肉芽肿和小血管炎。胞浆型抗中性粒细胞胞浆抗体（ c-ANCA） 阳性率为70% , ANCA 阴性率为5% 。结论 NGV 可累及多系统、多器官, 肺是最易侵犯的脏器之一, 但肺部临床症状及胸部影像均无特异性表现, 容易误诊。组织活检及ANCA 检查是明确诊断的重要方法。

Clinical Analysis of Necrotizing Granulomatous Vasculitis with Pulmonary Involvement

Objective To improve the awareness of necrotizing granulomatous vasculitis （ NGV）with pulmonary involvement by analyzing the clinical data and chest radiographic features.？Methods Theclinical data and chest radiographic features of 20 NGV cases with pulmonary involvement, who werediagnosed in Nanjing Drum Tower Hospital between March 2009 and March 2013, were retrospectivelyanalyzed.？Results There were 11 males and 9 females. The onset age ranged from 18 to 76 years withmean age of （ 44. 7 ±15） years. The patients frequently presented with multi-system involvement, with theincidence of pulmonary involvement of 100% , which presented with coughing, expectoration, hemoptysis, anddyspnea, etc. The followed involved organs were nose （ 85% ） , kidney（ 85% ） , joints and muscle （ 35% ） , ear（ 40% ） and ocular （ 20% ） . The radiographic manifestations varied among the cases. Images mostly showedmultiple nodules /mass with/without cavity in lung, and patchy infiltration was also observed, which often ledto misdiagnosis of NGV. The most common pathologic features were necrotizing granulomatosis andmicrovasculitis. cANCA was positive in 70% of patients, while ANCA was negative in 5% of patients.Conclusions NGV often has multi-organ involvement, and the lung is one of the most commonly involvedorgans. The clinical symptoms and radiographic manifestations of NGV have no specificity, and the diseasecan be easily misdiagnosed. Biopsy and ANCA assay can be important means for NGV diagnosis.