2017
DOI: 10.1183/16000617.0053-2017
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When to start and when to stop antifibrotic therapies

Abstract: Idiopathic pulmonary fibrosis (IPF) is characterised by progressive changes of the lung architecture causing cough and dyspnoea and ultimately leading to lung failure and death. Today, for the first time, two drugs that may reduce the inexorable progression of the disease are available, suggesting that treatment with specific drugs for IPF should be started as soon as diagnosis is made. This applies to any disease and particularly to IPF, which is marked by a 5-year survival comparable or even worse than many … Show more

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Cited by 46 publications
(32 citation statements)
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“…Interestingly, as some patients may also be classified as IPAF, antifibrotics could represent a valid therapeutic option also for this specific group [22][23][24]. Furthermore, as documented in IPF patients, both pirfenidone and nintedanib were generally well tolerated, with the need of a dose reduction only in a few cases [12][13][14][15][16][17]. The one-year survival of PF-ILDs looks incredibly similar to IPF making the two diseases very similar (Fig.…”
Section: Discussionmentioning
confidence: 99%
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“…Interestingly, as some patients may also be classified as IPAF, antifibrotics could represent a valid therapeutic option also for this specific group [22][23][24]. Furthermore, as documented in IPF patients, both pirfenidone and nintedanib were generally well tolerated, with the need of a dose reduction only in a few cases [12][13][14][15][16][17]. The one-year survival of PF-ILDs looks incredibly similar to IPF making the two diseases very similar (Fig.…”
Section: Discussionmentioning
confidence: 99%
“…In recent years, undeniable progress has been achieved in understanding the pathogenic mechanisms of IPF. This has progressively led to the advent of pirfenidone and nintedanib, the first two drugs able to reduce lung function decline [12][13][14][15][16][17]. Comparable to IPF, some PF-ILDs are triggered by repetitive lung parenchymal injuries and demonstrate TGFβ-mediated fibroblast activation and myofibroblast accumulation that may lead to a progressive phenotype [18][19][20][21].…”
Section: Discussionmentioning
confidence: 99%
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“…MILANI et al [5] tackle the subject of a little known but clinically relevant condition, lung involvement in amyloidosis, with regards to the possible clinical consequences for the affected patients. TORRISI et al [6] take on the difficult task of exploring the indications and contraindications of antifibrotic therapies in IPF, a topical issue of great relevance, while CAMINATI et al [7] discuss how to deal with the most advanced phases of this disease. Finally, RAGHU [8], a worldwide expert on IPF and other interstitial lung diseases, discusses the future landscapes for the treatment of IPF and analyses the most important and promising trials in the pharmacological therapy of this disease.…”
mentioning
confidence: 99%