CAJ | 학술논문

目的：分析儿童核周型抗中性粒细胞胞浆抗体相关性系统性血管炎（p-ANCA）的肺部影像特点。方法回顾性分析我院15例p-ANCA相关性肺炎患儿的临床及影像资料，其中女13例、男2例；年龄1～17岁，中位年龄10岁。15例患儿均行胸部CT平扫检查，其中12例为原发p-ANCA相关的显微镜下多血管炎（MPA）、2例为继发MPA、1例为变应性肉芽肿性血管炎(CSS)。由2位中级以上影像科医师共同进行图像分析。结果12例原发性MPA中，1例见双肺大片实变影，以双肺下叶背侧为主，实变影周围可见少许磨玻璃影，伴双侧胸腔积液；1例左下肺见限局性实变影，周围伴有少许小叶间隔增厚及同侧胸腔积液；1例患儿肺内仅见右肺中叶限局性间质增厚；9例双肺可见散在或弥漫的磨玻璃影，以胸膜下分布为主，病灶内可见肺内间质及（或）胸膜增厚影。2例继发性MPA中1例咳血患儿CT上可见双肺少许磨玻璃影，未见小叶间隔增厚及小结节影，未见胸膜渗出改变；1例无呼吸道症状，仅有肾功能异常，CT检查示右肺下叶叶间胸膜下小结节影，伴晕征。1例CSS患儿的CT表现为两肺弥漫分布的小片状磨玻璃影，双肺舌叶可见实变影，多发小叶中央结节及小叶周边结节，小叶间隔增厚，肺底部及肺周围肺野病变明显。病变肺野内部分支气管管壁增厚，管腔略明显。双肺胸膜及叶间胸膜增厚。结论 p-ANCA相关肺炎的胸部CT表现为非特异性肺小血管出血征象。
목적：분석인동핵주형항중성립세포포장항체상관성계통성혈관염（p-ANCA）적폐부영상특점。방법회고성분석아원15례p-ANCA상관성폐염환인적림상급영상자료，기중녀13례、남2례；년령1～17세，중위년령10세。15례환인균행흉부CT평소검사，기중12례위원발p-ANCA상관적현미경하다혈관염（MPA）、2례위계발MPA、1례위변응성육아종성혈관염(CSS)。유2위중급이상영상과의사공동진행도상분석。결과12례원발성MPA중，1례견쌍폐대편실변영，이쌍폐하협배측위주，실변영주위가견소허마파리영，반쌍측흉강적액；1례좌하폐견한국성실변영，주위반유소허소협간격증후급동측흉강적액；1례환인폐내부견우폐중협한국성간질증후；9례쌍폐가견산재혹미만적마파리영，이흉막하분포위주，병조내가견폐내간질급（혹）흉막증후영。2례계발성MPA중1례해혈환인CT상가견쌍폐소허마파리영，미견소협간격증후급소결절영，미견흉막삼출개변；1례무호흡도증상，부유신공능이상，CT검사시우폐하협협간흉막하소결절영，반훈정。1례CSS환인적CT표현위량폐미만분포적소편상마파리영，쌍폐설협가견실변영，다발소협중앙결절급소협주변결절，소협간격증후，폐저부급폐주위폐야병변명현。병변폐야내부분지기관관벽증후，관강략명현。쌍폐흉막급협간흉막증후。결론 p-ANCA상관폐염적흉부CT표현위비특이성폐소혈관출혈정상。
Objective To investigate the chest HRCT characteristics of pediatric perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) associated systemic vasculitis. Methods Retrospectively analysis of the clinical and HRCT data of 15 pediatric cases diagnosed as p-ANCA related pneumonia according to the classification and diagnostic criteria of Chapel Hill meeting of 1994 in our hospital were retrospectively analyzed. They were 13 girls and 2 boys. The age range was 1—17 years and the median was about 10 years old, In the 15 patients, 12 were diagnosed as primary MPA, 2 were secondary MPA, 1 was CSS. Two radiologists with 10 years of radiological experience read the CT imaging together. Results 1 of 12 primary MPA were found large opacities and ground glass shades in both sides of lung,especially the posterior parts on CT images with bilateral pleural effusion; 1 case had focal opacities in left lower lobe, around with thickened interlobular septa and pleural effusion; Another 1 case only had focal thickened interstitial in right middle lobe; The other 9 cases showed scattered or diffused ground glass opacity, with thickened pulmonary interstitial and (or) pleura. 1 of the 2 secondary MPA who had hemoptysis showed ground glass opacity on CT images with no thickened septal nodules or pleural effusion. The other one who had renal anomalies and no respiratory symptoms showed subpleural nodules with halo sign in right low lobe. The CSS showed diffused patchy ground glass opacity, consolidation of both lingular lobes, nodules of centri-and peri-lobular, thickened septa near the pleura, obviously thickened bronchial wall, slightly widened bronchial lumen and thickened pleura. Conclusion The classic imaging features of p-ANCA pneumonia on HRCT is the non-specific small pulmonary vessel hemorrhage.