摘要
目的 探讨呼吸性细支气管炎伴间质性肺病 (RBILD)的临床病理特点以及与脱屑性间质性肺炎 (DIP)的关系。方法 回顾性分析 1例经电视胸腔镜肺活检诊断为RBILD患者的临床表现、影像学和组织病理学特点、糖皮质激素治疗的效果和随访结果 ,并与 1例病理学确诊的DIP患者进行比较。结果 2例患者均为 5 7岁男性 ,吸烟史分别为 2 4、30年。临床表现为咳嗽、咯痰、气促 ,双下肺闻及veclro音 ;肺功能检查显示RBILD患者为混合型通气功能障碍 ,DIP患者为限制性轻度通气功能障碍。胸部X线片显示 2例患者双肺均有散在斑点状、斑片状密度不均阴影 ;高分辨率CT表现为两肺散在分布的间质增厚影 ,部分呈网格状改变 ,以外周和下肺为主 ,DIP患者有磨玻璃影。病理特征 :RBILD表现为在呼吸性细支气管及周围气腔内有大量均一的含色素的巨噬细胞聚集 ,肺间质有轻度的纤维组织增生和慢性炎症 ;DIP的上述病变更明显和弥漫。 2例患者均对糖皮质激素治疗反应良好 ,经随访 3年余 ,患者病情稳定无复发。结论 RBILD和DIP在临床表现上不易区分 ,而开胸肺活检组织病理学检查可区分和明确诊断 ,两者有相似之处 ,可能为同一疾病实体。
Objective To investigate the clinicopathologic characteristics of respiratory bronchiolitis-associated interstitial lung disease (RBILD) and its relationship to desquamative interstitial pneumonia(DIP). Methods The clinical and pathological data of one patient with RBILD confirmed by video-assisted thoracoscopic lung biopsy were reviewed retrospectively, and compared with one patient with DIP. Results Both patients were 57 year old male, and they had a history of cigarette smoking for 24 and 30 years respectively. The clinical manifestations were cough and sputum production, breathlessness with exertion, and crackles on chest examination. Lung function test showed a mild abnormality with mixed obstructive-restrictive and restrictive pattern respectively. The chest radiograph showed scattered small nodules and patchy densities. High resolution computer tomography showed scattered interstitial infiltrates and reticular changes in the middle and lower lung fields, but patchy ground-glass attenuation was found only in DIP. The pathological examination showed the presence of clusters of pigmented macrophages within the lumens of respiratory bronchioles, alveolar ducts and peribronchiolar alveolar spaces, with patchy submucosal and peribronchiolar infiltration of lymphocytes and histiocytes. Mild peribronchiolar fibrosis was found in RBILD. Compared with RBILD,the lesions of DIP were more severe and widespread. Both the patients responded favorably to glucocorticoids. They were followed for more than three years. Conclusion RBILD could be confused with DIP in clinical manifestations, but can be separated by open lung biopsy. Considering their similarities, these two lesions may be regarded as a same disease entity.
出处
《中华结核和呼吸杂志》
CAS
CSCD
北大核心
2004年第6期373-377,共5页
Chinese Journal of Tuberculosis and Respiratory Diseases
基金
上海市科学技术委员会科研基金资助项目(0 3 4119868)
上海市卫生局科学技术发展基金资助项目 (9940 5 )
关键词
呼吸性细支气管炎
间质性肺病
脱屑性间质性肺炎
比较分析
Bronchiolitis, obliterans
Pathology,clinical
Desquamative interstitial pneumonia
Idiopathic interstitial pneumonia
