Classification and natural history of the idiopathic interstitial pneumonias.
Classification and natural history of the idiopathic interstitial pneumonias.
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DOI：
10.1513/pats.200601-005tk
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发表时间：
2006-06-01
期刊：
影响因子：
--
通讯作者：
King, Talmadge E Jr
中科院分区：
文献类型：
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作者：
Kim, Dong Soon;Collard, Harold R;King, Talmadge E Jr
在美国胸科学会/欧洲呼吸学会共识分类中，特发性间质性肺炎分为7种临床病理实体。分类主要基于组织病理学，但取决于临床医生，放射科医生和病理学家的密切互动。准确的诊断可能非常困难，特别是在决定特发性肺纤维化和纤维化非特异性间质性肺炎时;需要更好的诊断标志物。特发性肺纤维化的预后非常差，诊断后的中位生存期为2-4年，但个体患者的病程差异很大。预测个体患者的预后具有挑战性，但已确定了各种临床和放射学变量。根据最近的几项临床试验，这种疾病的自然史可能包括相对稳定的时期，其间会出现导致大量发病或死亡的疾病急性加重。非特异性间质性肺炎的特征是具有独特的组织病理学表现，其预后优于特发性肺纤维化。然而，特发性肺纤维化与纤维化非特异性间质性肺炎的关系仍存在混乱和争议。
In the American Thoracic Society/European Respiratory Society consensus classification, idiopathic interstitial pneumonias are classified into seven clinicopathologic entities. The classification is largely based on histopathology, but depends on the close interaction of clinician, radiologist, and pathologist. An accurate diagnosis can be very difficult, especially when deciding between idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia; better diagnostic markers are needed. The prognosis of idiopathic pulmonary fibrosis is very poor, with median survival of 2-4 yr after the diagnosis, yet the course of individual patients is highly variable. Predicting prognosis in the individual patient is challenging but various clinical and radiologic variables have been identified. According to several recent clinical trials, the natural history of this disease may involve periods of relative stability punctuated by acute exacerbations of disease that result in substantial morbidity or death. Nonspecific interstitial pneumonia is characterized by a distinct histopathologic appearance and a better prognosis than idiopathic pulmonary fibrosis. However, there is still confusion and controversy over the relationship between idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia.