King Talmadge E
Interstitial Lung Disease Center, San Francisco General Hospital, San Francisco, CA 94110, USA.
Trans Am Clin Climatol Assoc. 2004;115:43-76; discussion 76-8.
The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of usual interstitial pneumonia suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. While the clinical and radiographic diagnosis of idiopathic interstitial pneumonias can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathology. A structured, clinical-radiological-pathological approach to the diagnosis of the idiopathic interstitial pneumonias, with particular attention to the identification of idiopathic pulmonary fibrosis, insures proper therapy, enhances prognostication, and allows for further investigation of therapies aimed at distinct pathophysiology.
特发性间质性肺炎是一组病因不明的异质性疾病，对患者往往造成严重后果。根据临床-放射学-病理学标准对特发性间质性肺炎进行亚分类，突出了其在发病机制、治疗和预后方面的重要意义。最关键的区别在于是否存在普通型间质性肺炎，这是特发性肺纤维化中所见的组织病理学模式。特发性肺纤维化对治疗的反应和预后较差。对普通型间质性肺炎病理生理学的新认识表明其是一种明显的纤维增殖过程，抗纤维化治疗显示出前景。虽然在某些情况下可以自信地做出特发性间质性肺炎的临床和影像学诊断，但许多患者需要进行外科肺活检以确定其潜在的组织病理学。一种结构化的临床-放射学-病理学方法用于诊断特发性间质性肺炎，尤其注重识别特发性肺纤维化，可确保正确治疗、改善预后，并允许对针对不同病理生理学的治疗方法进行进一步研究。
