The Impact of Lung Cancer on Survival of Idiopathic Pulmonary Fibrosis

Tomassetti, Sara; Gurioli, Carlo; Ryu, Jay H.; Decker, Paul A.; Ravaglia, Claudia; Tantalocco, Paola; Buccioli, Matteo; Piciucchi, Sara; Sverzellati, Nicola; Dubini, Alessandra; Gavelli, Giampaolo; Chilosi, Marco; Poletti, Venerino

doi:10.1378/chest.14-0359

Cited by 271 publications

(288 citation statements)

References 30 publications

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“…The specific molecular and cellular mechanisms and causes of disease onset and progression are still unknown [3]. However, a growing body of evidence suggests that IPF is caused by an aberrant wound healing response to recurrent alveolar epithelial cell injury causing accelerated lung senescence and an increased fibrosis [4][5][6]. Cigarette smoking, exposure to organic and inorganic dust, and genetic factors have been shown to play a central role in disease pathogenesis [7].…”

Section: Introductionmentioning

confidence: 99%

The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

Tomassetti¹,

Piciucchi

Tantalocco³

et al. 2015

Eur Respir Rev

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Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases. @ERSpublicationsThe strengths and limitations of IPF management by a multidisciplinary team are highlighted in two patient cases http://ow.ly/H04Xe

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Section: Introductionmentioning

confidence: 99%

The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

Tomassetti¹,

Piciucchi

Tantalocco³

et al. 2015

Eur Respir Rev

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“…Le conseguenze di ognuna di queste comorbidità sulla storia e la prognosi dell'IPF non sono ancora del tutto chiarite, ma l'ipertensione polmonare, il cancro del polmone, oltre all'enfisema combinato alla IPF (Sindrome CPFE) hanno un documentato impatto negativo sulla sopravvivenza [26,27]. Il rischio relativo di un paziente con IPF di sviluppare un cancro del polmone, rispetto alla popolazione generale, è di 7,31 [28] e tale rischio risulta maggiore nei maschi fumatori.…”

Section: Comorbiditàunclassified

“…Il protocollo ASCEND prevedeva inoltre, come analisi pre-specificata dello studio, la valutazione della mortalità nell'intera coorte dei pazienti trattati negli studi ASCEND, CAPACITY 004 e CAPACITY 006 (1.247 pazienti, in totale). Una pooled analysis prespecificata ha riportato, rispetto al placebo, una riduzione della mortalità ad un anno del 48% per qualunque causa (HR = 0,52; IC95% = 0,31-0,87; p = 0,01) e del 68% per IPF (HR = 0,32; IC95% = 0,14-0,76; p = 0,006) [27]. A seguito dell'analisi cumulata, EMA ha rilasciato l'autorizzazione all'immissione in commercio.…”

Section: Trattamento Farmacologico Della Ipfunclassified

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Belisari¹,

Bettoni

Cortesi

et al. 2017

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To date, there are few therapeutic answers for Idiopathic pulmonary fibrosis (IPF) and only two pharmacological treatments have a marketing authorization for this disease. Recently nintedanib (Ofev®) has been authorized as a new therapeutic option and its economic profile has been evaluated by international Health Technology Assessment (HTA) bodies. IPF has important implications for everyday life of patients and their carers, negatively influencing their quality of life and bringing heavy economic burden to the NHS and to the entire society. It is, therefore important to consider these aspects for the Italian environment and to perform a pharmacoeconomic evaluation to define the efficiency of nintedanib in IPF by means of a Cost-Utility Analysis (CUA). As IPF is a chronic and progressive disease, a lifetime Markov model has been therefore developed with health states describing the patient’s condition as a combination of lung function and exacerbation history. The cohort entered in the model at different Forced Vital Capacity (FVC%) predicted health states, without exacerbation. The Clinical data used to perform this CUA were derived from clinical trials and the relative efficacy of nintedanib versus the comparator (pirfenidone) was then obtained from a Network Meta-Analysis (NMA) combining data reported in each primary study (INPULSIS 1-2 and TOMORROW trials for nintedanib, and CAPACITY and ASCEND trials for pirfenidone, respectively). At base-case, treatment with nintedanib resulted in a slightly lower estimated total cost vs pirfenidone, better safety profile and lower risk of acute exacerbations with an advantage in Quality Adjusted Life years (QALYs) gained. These results were confirmed by the sensitivity analysis. Although nintedanib appears to be a valuable option for the NHS to treat IPF patients, future data evidence, as long-term or real-life data, will help to confirm these results.[In Italian]

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“…In particular, Watanabe and colleagues suggest surgical treatment of cancer lesions in IPF patients affected by stage I LC although long-term results of these patients are worse than patients without IPF [Watanabe et al 2008]. Tomassetti and colleagues suggest sublobar resection as a safer surgical treatment of stage I LC in mild IPF patients compared with lobectomy even if the risk of LC recurrence is high [Tomassetti et al 2015]. Therefore, to treat LC in a minimally invasive way, early diagnosis of LC by HRCT scan would be necessary.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

New perspectives on management of idiopathic pulmonary fibrosis

Puglisi

Torrisi

Vindigni

et al. 2016

Therapeutic Advances in Chronic Disease

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Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive parenchymal lung disease characterized by a median survival of 3-5 years following diagnosis. The diagnosis is based on clinical, radiological and histopathological evaluation. Therefore, a multidisciplinary team is needed to reach the correct diagnosis. For a long time, supportive care and lung transplantation in selected cases, have been considered the only possible treatments for IPF. In the last decade many studies have investigated IPF pathogenesis, leading to an improved knowledge of the mechanisms underlying the disease and to the approval of two new drugs for IPF treatment (pirfenidone and nintedanib). The therapeutic approach of IPF cannot be limited to the administration of antifibrotic drugs, but it is necessary for improving the quality of life of patients and for facilitating, as far as possible, the performance of normal daily activities and relationships. IPF patients are also afflicted by disease-related complications such as gastroesophageal reflux, pulmonary hypertension, acute exacerbations and an increased risk of developing lung cancer. The clinician who treats IPF patients, should also treat these possible complications to slow disease progression, thus maintaining the possibility of a pulmonary transplantation.

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The Impact of Lung Cancer on Survival of Idiopathic Pulmonary Fibrosis

Cited by 271 publications

References 30 publications

The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

New perspectives on management of idiopathic pulmonary fibrosis

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