闭塞性细支气管炎伴机化性肺炎的临床和影像学研究

Bronchiolitis obliterans organizing pneumonia:the clinical and radiological features of seven cases and a review of the literature

目的 提高对闭塞性细支气管炎伴机化性肺炎 (BOOP)的临床、影像学特征、纤维支气管镜肺活检病理表现的认识。方法 7例BOOP患者均有完整的临床、实验室检查、胸片和CT检查资料 ,经纤维支气管镜肺活检病理证实 ,对其临床、影像学和病理学表现进行回顾性分析。结果BOOP常见的临床表现为咳嗽、进行性呼吸困难和吸气末肺部爆裂音。肺功能示限制性通气功能障碍 ,弥散功能明显减低。经纤维支气管镜肺活检病理检查肺泡管、肺泡腔内见单核细胞不同程度的浸润 ,腔内被成纤维细胞和疏松肉芽组织阻塞。X线表现为两肺多发性气腔实变阴影 ,分布无特点 ;CT表现为气腔实变阴影 ,主要分布在胸膜下和 /或支气管血管束周围 ,伴或不伴毛玻璃影 ;3例患者CT主要表现为线状或不规则条索状阴影 ,具有一定的特征性。 7例患者对肾上腺皮质激素治疗有显著疗效。结论 临床表现结合影像学特点可提示BOOP临床诊断 ,但需要进一步病理证实 ,经纤维支气管镜肺活检是诊断BOOP有效的检查方法之一 ,但以经胸腔镜或开胸肺活检价值最高。

Objective To highlight the clinical features,radiographic manifestations and pathology of bronchiolitis obliterans organizing pneumonia.Methods The diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP) was made based on clinical and radiological features and comfirmed by transbronchial lung biopsy (TBLB) and pathological examination.All data were analyzed and relevant literatures were reviewed. ResultsThe most frequent presentations of BOOP were cough,progressive dyspnea and fine end-inspiratory crackles.Pulmonary function tests showed a restrictive pattern and a decreased diffusing capacity.The pathologic findings achieved by TBLB disclosed infiltration with mononuclear cells in bronchioles,alveolar ducts and alveoli.The lumina were obliterated with fibroblasts and loose granulation tissues.The majority of chest radiographs showed bilateral multifocal consolidation with no zonal preponderance.CT scans revealed patchy air-space consolidation in all cases,often by a predominantly subpleural and/or peribronchovascular distribution with or without ground-glass opacities.In 3 of the 6 patients the typical CT features of BOOP predominantly consisted of linear or band-like opacities or both.Corticosteroid treatment resulted in significant improvement in seven cases.ConclusionsThe diagnosis of BOOP is usually suggested by clinical and radiological findings and confirmed by histopathological examination,preferably through surgical open or video-assisted thoracoscopic biopsy.Transbronchial lung biopsy should be regarded as a useful diagnostic tool for BOOP.