Raghu Ganesh, Chang Jacqueline
University of Washington Medical Center, 1959 N.E. Pacific, Campus Box 356522, Seattle, WA 98195-6522, USA.
Clin Chest Med. 2004 Dec;25(4):621-36, v. doi: 10.1016/j.ccm.2004.08.002.
Idiopathic pulmonary fibrosis (IPF) is a chronic scarring illness limited to the lung and characterized by progressive dyspnea, restrictive pulmonary physiology, and radiographic diffuse lung disease. During the past few years, significant progress has been made, particularly in diagnosis: a stricter case definition has been outlined by consensus among international experts. Insights into the pathophysiology have revealed novel areas for therapeutic intervention. These advances have helped clarify many important diagnostic and therapeutic issues in this disease. An effective treatment regimen is yet to be determined. Significant new insights in the natural course and monitoring disease course have provided use of appropriate endpoints to test the safety and efficacy of new treatment regimens. Several multicenter clinical trials are underway and several more are being planned. It is hoped that ongoing molecular genetic studies and results of clinical trials will improve outcome of patients and families affected with this disease in the near future.
特发性肺纤维化（IPF）是一种局限于肺部的慢性瘢痕性疾病，其特征为进行性呼吸困难、限制性肺生理功能以及影像学上的弥漫性肺部疾病。在过去几年中取得了重大进展，尤其是在诊断方面：国际专家已就更严格的病例定义达成共识。对病理生理学的深入了解揭示了治疗干预的新领域。这些进展有助于阐明该疾病许多重要的诊断和治疗问题。尚未确定有效的治疗方案。对自然病程和疾病进程监测的重大新见解为测试新治疗方案的安全性和有效性提供了合适的终点指标。多项多中心临床试验正在进行中，还有几项正在计划中。希望正在进行的分子遗传学研究和临床试验结果能在不久的将来改善受该疾病影响的患者及其家庭的预后。
