Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, VA.
Advanced Lung Disease and Lung Transplant Program, Inova Fairfax Hospital, Falls Church, VA.
Chest. 2011 Jul;140(1):221-229. doi: 10.1378/chest.10-2572.
The American Thoracic Society and European Respiratory Society guidelines for the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) have been published recently. However, the influence, practical application, and utility of the prior consensus statement for IPF have never been evaluated. Demographics, diagnostic criteria, pulmonary function data, and disposition of patients with IPF evaluated at an interstitial lung disease center between 2000 and 2009 were analyzed. Enrollment in clinical drug trials, lung transplantation, and mortality also were assessed. A total of 521 patients with IPF were evaluated, with pulmonary function testing available in 446. In the 64% of patients without surgical lung biopsy, the most common major criterion not fulfilled was bronchoscopy. Lung transplantation was performed in 16.1% of patients, whereas 27.4% of prescreened patients were enrolled in a prospective drug study. Patients with mild, moderate, and severe disease categorized by FVC % predicted had median survivals of 55.6, 38.7, and 27.4 months, respectively. The attrition rate of patients who survived beyond 5 years was attenuated in subsequent years. IPF remains a deadly disease with a poor prognosis. Bronchoscopy does not appear to be required for an accurate diagnosis. A minority of patients were accommodated within a clinical trial or with transplantation. Categorization by baseline FVC % predicted effectively discriminates groups with different long-term outcomes. Our analysis supports the view that the value of statements also can be realized in the subsequent demonstration of their impact on patient management, which might enable further refinements in a continuous, iterative rediscovery process.
美国胸科学会和欧洲呼吸学会最近发布了特发性肺纤维化 (IPF) 的诊断和治疗指南。然而，之前的 IPF 共识声明的影响、实际应用和实用性从未得到过评估。分析了 2000 年至 2009 年间在间质性肺病中心评估的 IPF 患者的人口统计学、诊断标准、肺功能数据和患者处理情况。还评估了临床试验、肺移植和死亡率的登记情况。共评估了 521 例 IPF 患者，其中 446 例有肺功能检测。在没有外科肺活检的 64%的患者中，最常见的未满足主要标准是支气管镜检查。16.1%的患者进行了肺移植，而 27.4%的筛选患者参加了前瞻性药物研究。根据 FVC %预测值，轻度、中度和重度疾病患者的中位生存期分别为 55.6、38.7 和 27.4 个月。在 5 年以上存活的患者中，每年的病死率都会降低。IPF 仍然是一种致命疾病，预后不良。支气管镜检查似乎不是准确诊断所必需的。少数患者可以在临床试验或移植中得到治疗。根据基线 FVC %预测值进行分类，可以有效地区分具有不同长期预后的组。我们的分析支持这样一种观点，即声明的价值也可以通过随后证明其对患者管理的影响来实现，这可能会在一个持续的、迭代的重新发现过程中进一步完善。
