【作者】 樊娜；

【导师】 马忠森；

【作者基本信息】 吉林大学 ， 临床医学， 2012， 硕士

【摘要】 弥漫性间质性肺疾病(diffuse interstitial lung disease，DILD)是以肺泡壁为主并包括肺泡周围组织及其相邻支撑结构病变的一组疾病；是以弥漫性肺实质、肺泡炎和间质纤维化为基本病理改变，以活动后呼吸困难，胸部X线可见双肺弥漫性阴影，限制性通气障碍，弥散功能降低和低氧血症为临床特征的异质性疾病群构成的临床-病理实体的总称；是多种原因引起的急慢性肺部疾病的共同结局；是危害人类健康的重要疾病，其发病率及病死率逐年增高［1］。目前，已知的DILD病因近200种，但确切发病机制尚未完全阐明。DILD对现有治疗方法有较为理想效果的相当有限，大多数DILD治疗相当困难。DILD的发病机制及有效的治疗方案已成为国内外探索研究的热点。本文主要就2011年发表的百余篇中英文文献进行复习、整理，以利于临床工作的参考以及为新的研究开拓思路。更多还原

【Abstract】 Patients with diffuse interstitial lung diseases (DILDs) come tomedical attention mainly because of the onset of progressive exertionaldyspnea or a persistent, nonproductive cough. Hemoptysis, wheezing, andchest pain may be present. Often, the identification of interstitial opacitieson chest x-ray focuses the diagnostic approach toward one of theDILDs.DILDs represent a large number of conditions that involve theparenchyma of the lung—the alveoli, the alveolar epithelium, thecapillary endothelium, and the spaces between these structures, as well asthe perivascular and lymphatic tissues. This heterogeneous group ofdisorders is classified together because of similar clinical,roentgenographic, physiologic, or pathologic manifestations. Thesedisorders are often associated with considerable morbidity and mortality,and there is little consensus regarding the best management of most ofthem.DILDs have been difficult to classify because>200knownindividual diseases are characterized by diffuse parenchymal lunginvolvement, either as the primary condition or as a significant part of amultiorgan process, as may occur in the connective tissue diseases(CTDs). One useful approach to classification is to separate the ILDs intotwo groups based on the major underlying histopathology:(1) thoseassociated with predominant inflammation and fibrosis, and (2) thosewith a predominantly granulomatous reaction in interstitial or vascular areas. Each of these groups can be further subdivided according towhether the cause is known or unknown. For each DILD there may be anacute phase, and there is usually a chronic one as well. Rarely, some arerecurrent, with intervals of subclinical disease.The DILDs arenonmalignant disorders and are not caused by identified infectious agents.The precise pathway(s) leading from injury to fibrosis is not known.Although there are multiple initiating agent(s) of injury, theimmunopathogenic responses of lung tissue are limited, and themechanisms of repair have common features.As mentioned above, thetwo major histopathologic patterns are a granulomatous pattern and apattern in which inflammation and fibrosis predominate.Although thecourse of DILD is variable, progression is common and often insidious.All treatable possibilities should be carefully considered. Since therapydoes not reverse fibrosis, the major goals of treatment are permanentremoval of the offending agent, when known, and early identification andaggressive suppression of the acute and chronic inflammatory process,thereby reducing further lung damage. In this article,we made an analysisfrom hundreds of Chinese and English literature in2011in order toexplore new ideas for new research.更多还原