Servicio de Neumología, Hospital Clínic, Barcelona, España.
Arch Bronconeumol. 2013 Aug;49(8):343-53. doi: 10.1016/j.arbres.2013.03.011. Epub 2013 Jun 4.
Idiopathic pulmonary fibrosis is defined as a chronic fibrosing interstitial pneumonia limited to the lung, of unknown cause, with poor prognosis and few treatment options. In recent years there has been an increase in their prevalence, probably due to the optimization of diagnostic methods and increased life expectancy. The ATS/ERS Consensus (2000) established the diagnostic criteria and recommendations for the assessment of the disease course and treatment. Later studies have helped to redefine diagnostic criteria and treatment options. In 2011, an international consensus was published, establishing diagnostic criteria and new treatment strategies. These guidelines have been updated with the newest aspects of diagnosis and treatment of idiopathic pulmonary fibrosis. A level of evidence has been identified for the most relevant questions, particularly with regard to treatment options.
特发性肺纤维化定义为一种局限于肺部的慢性纤维性间质性肺炎，病因不明，预后较差，治疗选择有限。近年来，其患病率有所增加，可能是由于诊断方法的优化和预期寿命的延长。美国胸科学会/欧洲呼吸学会共识（2000 年）确立了诊断标准和疾病进程及治疗评估的建议。后来的研究有助于重新定义诊断标准和治疗选择。2011 年，发表了一项国际共识，确立了诊断标准和新的治疗策略。这些指南已经根据特发性肺纤维化的最新诊断和治疗方面进行了更新。针对最相关的问题，特别是治疗选择问题，确定了证据级别。
