The clinical course of patients with idiopathic pulmonary fibrosis
The clinical course of patients with idiopathic pulmonary fibrosis
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DOI：
10.7326/0003-4819-142-12_part_1-200506210-00005
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发表时间：
2005-06-21
影响因子：
39.2
通讯作者：
Brown, KK
中科院分区：
文献类型：
--
作者：
Martinez, FJ;Safrin, S;Brown, KK
背景：明确特发性肺纤维化(IPF)临床病程的前瞻性数据很少。目的：分析轻、中度特发性肺间质纤维化患者的临床病程。设计：来自安慰剂组的数据分析，随机对照试验，评估干扰素-γ1b。地点：学术和社区医疗中心。患者：在一项评估干扰素-伽马1b的试验的安慰剂组中，168名患者。测量：生理学和呼吸困难的测量，每12周评估一次；住院；恶化速度和死亡原因的中位数为76周。结果：生理变量在研究期间变化最小。然而，23%的患者因呼吸系统疾病而需要住院治疗，21%的患者死亡。特发性肺纤维化是89%的死亡患者的主要死亡原因，其中47%的患者在死亡之前出现明显的急性临床恶化。限制：用于确定恶化速度和死亡原因的工具被回顾性应用。结论：认识到轻中度IPF患者常见的急性致命恶化对监测患者具有重要意义，并支持肺移植的早期转诊。
Background: Prospective data defining the clinical course in idiopathic pulmonary fibrosis (IPF) are sparse. Objective: To analyze the clinical course of patients with mild to moderate IPF.Design: Analysis of data from the placebo group of a randomized, controlled trial evaluating interferon-gamma 1b. Setting: Academic and community medical centers.Patients: 168 patients in the placebo group of a trial evaluating interferon-gamma 1b.Measurements: measures of physiology and dyspnea assessed at 12-week intervals; hospitalizations; and the pace of deterioration and cause of death over a median period of 76 weeks.Results: Physiologic variables changed minimally during the study. However, 23% of patients required hospitalization for a respiratory disorder and 21% died. Idiopathic pulmonary fibrosis was the primary cause of death in 89% of patients who died, and an apparent acute clinical deterioration preceded death in 47% of these patients.Limitations: The instrument used to define the pace of deterioration and cause of death was applied retrospectively.Conclusions: Recognition of the common occurrence of acute fatal deterioration in patients with mild to moderate IPF has important implications for monitoring patients and supports early referral for lung transplantation.