Department of Internal Medicine V, University of Munich, Comprehensive Pneumology Center, Marchioninistr, 15, 81377 Munich, Germany.
Respir Res. 2013;14 Suppl 1(Suppl 1):S6. doi: 10.1186/1465-9921-14-S1-S6. Epub 2013 Apr 16.
Patient management in Idiopathic Pulmonary Fibrosis (IPF) is largely based on societal guidelines and recommendations. A recent update by the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) provided updated guidance on the diagnosis and management of IPF, along with recommendations on pharmacologic and non-pharmacologic approaches to patient management. The treatment guidance is based on GRADE criteria, which rates the quality of evidence according to previously published methodology. Here we discuss how to interpret the recent guideline updates and the implications of this guidance for clinical practice. In addition we discuss the assessment and recommendations for a number of pharmacological agents that have been the focus of clinical trials over the past years. Although no single pharmacological agent was recommended by the guidelines committee, we discuss how since then, more recent data have resulted in the approval of pirfenidone in Europe, and preliminary negative findings regarding the safety of a triple therapy regimen consisting of prednisone, azathioprine and N-acetylcysteine have raised the question of whether it is no longer a treatment option. As clinicians, we must interpret the available guidance and recommendations as we consider each individual patient and as we discuss the available clinical data and the patient's own preferences in our approach to the management of this disease.
特发性肺纤维化(IPF)患者的管理主要基于社会指南和建议。美国胸科学会(ATS)、欧洲呼吸学会(ERS)、日本呼吸学会(JRS)和拉丁美洲胸科协会(ALAT)最近的更新提供了 IPF 诊断和管理的更新指南,以及对患者管理的药物和非药物方法的建议。治疗指南基于 GRADE 标准,该标准根据先前发表的方法对证据质量进行评级。在这里,我们讨论如何解释最近的指南更新,以及该指南对临床实践的影响。此外,我们还讨论了过去几年临床试验关注的一些药物的评估和建议。尽管指南委员会没有推荐任何单一的药物,但我们讨论了自那时以来,更多的最新数据如何导致吡非尼酮在欧洲获得批准,以及关于泼尼松、硫唑嘌呤和 N-乙酰半胱氨酸三联疗法安全性的初步负面发现,这使得我们不得不质疑它是否不再是一种治疗选择。作为临床医生,我们必须在考虑每个患者时解释可用的指导和建议,并在我们讨论可用的临床数据和患者自身对疾病管理的偏好时,考虑到这一点。