Crozer Chester Medical Center, Department of Pediatrics, One Medical Center Blvd, Upland, PA 19013, United States.
Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA 95616, United States.
Autoimmun Rev. 2014 Apr-May;13(4-5):508-12. doi: 10.1016/j.autrev.2014.01.037. Epub 2014 Jan 11.
Idiopathic pulmonary fibrosis is a difficult disease to diagnose. Idiopathic pulmonary fibrosis is a member of a class of diseases known as idiopathic interstitial pneumonias. Other members include nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Usual interstitial pneumonia (UIP) is the pathological equivalent of idiopathic pulmonary fibrosis. Prior to 2011, the diagnosis was based on major and minor criteria, but because these criteria were not evidence based, the criteria were modified by consensus from the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. These new criteria now include satisfying three core requirements, including exclusion of other possible cause of interstitial lung disease, specific findings of usual interstitial pneumonia on high resolution computed tomography, and a combination of "possible UIP" findings on high resolution computed tomography and UIP findings on lung biopsy. Idiopathic pulmonary fibrosis is a severe, progressive disease with limited treatment options, and exacerbations are associated with a high degree of morbidity and mortality.
特发性肺纤维化难以诊断。特发性肺纤维化属于特发性间质性肺炎的一种疾病，其他成员包括非特异性间质性肺炎、隐源性机化性肺炎、急性间质性肺炎、呼吸性细支气管炎相关间质性肺病、脱屑性间质性肺炎和淋巴细胞性间质性肺炎。寻常间质性肺炎（UIP）是特发性肺纤维化的病理学等同物。在 2011 年之前，该诊断基于主要和次要标准，但由于这些标准没有依据，因此标准由美国胸科学会、欧洲呼吸学会、日本呼吸学会和拉丁美洲胸科协会的共识进行了修改。这些新标准现在包括满足三个核心要求，包括排除其他可能的间质性肺病的原因、高分辨率计算机断层扫描上的寻常间质性肺炎的特定发现，以及高分辨率计算机断层扫描上的“可能 UIP”发现与肺活检上的 UIP 发现的组合。特发性肺纤维化是一种严重的、进行性疾病，治疗选择有限，加重与高发病率和死亡率相关。
