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 symptoms 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 significant 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 disease , with the incidence of group A ( 7/13 ) being significantly higher that group B ( 1/11 ) ( P < 0. 05 ). ( 5 ) Eight patients underwent 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 being reticular opacities, honey combing, ground - glass opacity ( GGO ), and interlobular septa thickening. The incidence of GGO was more often in group B ( 5/21 vs. 9/12 ) ( P <0. 05 ). Conclusion ( 1 ) AASV - DILD patients mostly present with multi - organic lesions. And as the non - pulmonary symptoms predominate, a missed diagnosis easily occurs for pulmonary disorder. ( 2 ) Apart from the lung, the commonest impaired organ is the kidney in AASV - DILD, the incidence of ARF is high, and CRF usually occurs in patients who present with renal lesions as the major symptoms. ( 3 ) Pulmonary heart disease commonly occur in patients presenting mainly with pulmonary symptoms. ( 4 ) BALF of AASV - DILD patients mainly shows a high percentage of neutrophils. The transhronehial lung biopsy has a certain value in diagnosing AASV. ( 5 ) No special feature of imaging exists in AASV - DILD, and antineutrophil cytoplasmic antibody test should be preformed routinely to avoid misdiagnosis.
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