隐源性机化性肺炎18例的临床病理特征

Clinicopathologic features of 18 cases of cryptogenic organizing pneumonia

目的回顾分析经病理确诊的隐源性机化性肺炎临床、病理和影像学特征,以提高临床诊断水平。方法1993年4月至2005年10月在北京协和医院住院、经病理确诊的机化性肺炎23例,对其进行临床、影像学和病理学综合分析。结果23例机化性肺炎中5例为药物引起或合并结缔组织病,18例为隐源性机化性肺炎(cryptogen ic organ izing pneumon itis,COP)。其中10例经皮肺活检或经支气管肺活检,8例开胸肺活检或经胸腔镜肺活检,男8例,女10例,年龄(53.5±11.0)岁。气短、咳嗽及肺部爆裂音及湿性啰音是主要的症状和体征。肺功能显示限制性通气功能障碍(11例)及弥散障碍(18例)。胸部CT表现为磨玻璃样变(4例),肺实变(13例)伴支气管充气征(3例),胸腔积液(4例)。支气管肺泡灌洗液中淋巴细胞占细胞总数的0.40±0.16,中性粒细胞为0.15±0.08,嗜酸粒细胞为0.05±0.03;CD4/CD8为0.43±0.21。所有患者均给予糖皮质激素治疗。16例患者经随诊(8.67±6.21)个月仍存活,临床缓解或病情稳定。1例患者对糖皮质激素反应差,死于进行性加重的呼吸衰竭。另1例患者糖皮质激素治疗后出现肺部感染,死于感染性休克。结论临床表现及影像学特征对COP的诊断有一定的提示作用,但确诊需依靠病理检查。经皮肺活检或经支气管镜肺活检结合支气管肺泡灌洗液检查,对临床诊断隐源性机化性肺炎有较高的价值。

Objective To analyze the characteristics of pathologically proved cases of cryptagenic organizing pneumonia （COP） including manifestation of CT scan, bronchoalveolar lavage （BAL） fluid cytology, pulmonary histopatholagical appearance. Methods Twenty-three consecutive patients from April 1994 to October 2005 in Peking Union Medical College Hospital with pathologic features of organizing pneumonia were enrolled in the study. Results Eighteen patients were diagnosed as COP. Pereutaneous lung biopsy/transbronchial lung biopsy was performed in 10 patients, while open lung biopsy/video-assistant thorascopic biopsy was performed in 8 patients. There were 8 males and 10 females, aged （53.5 ± 11.0） yr. Dyspnea, cough, inspiratory crackles were the most common symptoms and signs. Lung function tests showed a restrictive ventilatory defect （11/18）, and impairment of carbon monoxide transfer factor （TLCO, 18/18）. Chest computed tomography scan in 18 cases mainly showed ground glass opacities （4/18）, patchy areas of alveolar consolidation（ 13/18）with air bronchograms （3/18）, and pleural effussion （4/18）. BAL fluid cytological analysis showed the percentage of lymphocyte was 0. 40 ± 0. 16, neutrophil 0. 15 ± 0. 08, eosinophil 0. 05 ±0. 03; the CD4/CD8 ratio was 0. 43 ±0. 21. All patients were treated with corticosteroids. Sixteen were alive and clinically improved or stabilized at the last follow up （ 8.7 ± 6. 2 months after diagnosis）. Two patients died, one of respiratory failure, and another of pulmonary infection. Conclusions The diagnosis of COP is usually suggested by clinical-radiological findings, but needs to be confirmed histopathologically. The combination of BAL fluid cytological findings and lung histological appearance obtained during a bronchoscopic procedure appears to be an effective method for diagnosing this disease.