Costabel U, Bonella F
Abteilung Pneumologie/Allergologie, Ruhrlandklinik-Universitätsklinik, Tüschener Weg 40, Essen, Germany.
Internist (Berl). 2011 Dec;52(12):1422-8. doi: 10.1007/s00108-011-2926-0.
Idiopathic pulmonary fibrosis is a chronic progressive lung disease with poor prognosis. The IFIGENIA trial showed that antioxidative therapy with N-acetylcysteine versus placebo for patients under treatment with prednisone plus azathioprine significantly slowed the deterioration of pulmonary function after 12 months. A number of other drugs have recently been evaluated in large multicenter placebo-controlled trials. Etanercept, interferon-γ, bosentan, ambrisentan, imatinib, and sildenafil did not show efficacy. The antifibrotic active ingredient pirfenidone is the first drug approved for the treatment of adult patients with mild to moderate idiopathic pulmonary fibrosis in the European Union. Approval was based on the results of 4 randomized, placebo-controlled clinical trials including more than 1,100 patients. Pirfenidone slowed the decline in lung function and reduced the risk of disease progression. Side effects include gastrointestinal discomfort, skin reactions, including photosensitivity, and rarely increased liver enzymes.
特发性肺纤维化是一种预后较差的慢性进行性肺部疾病。IFIGENIA试验表明，对于接受泼尼松加硫唑嘌呤治疗的患者，使用N - 乙酰半胱氨酸进行抗氧化治疗与使用安慰剂相比，在12个月后显著减缓了肺功能的恶化。最近，许多其他药物已在大型多中心安慰剂对照试验中进行了评估。依那西普、干扰素 - γ、波生坦、安立生坦、伊马替尼和西地那非均未显示出疗效。抗纤维化活性成分吡非尼酮是欧盟首个被批准用于治疗轻度至中度特发性肺纤维化成年患者的药物。批准基于4项随机、安慰剂对照临床试验的结果，这些试验纳入了1100多名患者。吡非尼酮减缓了肺功能下降，并降低了疾病进展的风险。副作用包括胃肠道不适、皮肤反应（包括光敏反应），以及罕见的肝酶升高。
