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Respirology ; 22(7): 1436-1458, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28845557

RESUMO

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Indóis/uso terapêutico , Pneumologia , Piridonas/uso terapêutico , Sociedades Médicas , Austrália , Comorbidade , Progressão da Doença , Medicina Baseada em Evidências , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Nova Zelândia , Ensaios Clínicos Controlados Aleatórios como Assunto
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