Royal Prince Alfred Hospital, Sydney, NSW
Alfred Hospital, Melbourne, VIC.
Med J Aust. 2018 Feb 5;208(2):82-88. doi: 10.5694/mja17.00799.
Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.
特发性肺纤维化(IPF)是一种纤维性间质性肺疾病,与呼吸困难和咳嗽等衰弱症状相关,导致呼吸衰竭、生活质量受损,最终导致死亡。诊断 IPF 具有挑战性,因为它通常与其他间质性肺疾病具有许多共同特征。在本文中,我们总结了澳大利亚和新西兰胸科学会以及澳大利亚肺脏基金会最近发布的关于 IPF 的诊断和管理的联合立场声明,这些声明是专门为澳大利亚和新西兰的医生量身定制的。主要建议:建议进行全面的多学科团队会议,以快速准确地诊断 IPF。应考虑使用抗纤维化疗法来减缓疾病进展。然而,鉴于许多 IPF 亚组缺乏证据,特别是更广泛的疾病严重程度谱,因此需要谨慎使用。非药物干预措施,包括肺康复、补充氧气、适当治疗合并症和与疾病相关的症状,仍然是最佳管理的关键。尽管最近取得了进展,但 IPF 仍然是一种致命疾病,应将合适的患者转介进行肺移植评估。
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