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      Physiology of the lung in idiopathic pulmonary fibrosis

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          Abstract

          The clinical expression of idiopathic pulmonary fibrosis (IPF) is directly related to multiple alterations in lung function. These alterations derive from a complex disease process affecting all compartments of the lower respiratory system, from the conducting airways to the lung vasculature. In this article we review the profound alterations in lung mechanics (reduced lung compliance and lung volumes), pulmonary gas exchange (reduced diffusing capacity, increased dead space ventilation, chronic arterial hypoxaemia) and airway physiology (increased cough reflex and increased airway volume), as well as pulmonary haemodynamics related to IPF. The relative contribution of these alterations to exertional limitation and dyspnoea in IPF is discussed.

          Abstract

          Physiological impairment in IPF is complex and involves all compartments of the respiratory system http://ow.ly/gyao30hdHUb

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          An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management

          Ganesh Raghu, Harold Collard, Jim J. J. Egan … (2011)
          American Journal of Respiratory and Critical Care Medicine, 183(6), 788-824
          Article 0 comments Cited 1117 times – based on 0 reviews     Review now
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            Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.

            Luca Richeldi, Roland M du Bois, Ganesh Raghu … (2014)
            Nintedanib (formerly known as BIBF 1120) is an intracellular inhibitor that targets multiple tyrosine kinases. A phase 2 trial suggested that treatment with 150 mg of nintedanib twice daily reduced lung-function decline and acute exacerbations in patients with idiopathic pulmonary fibrosis. We conducted two replicate 52-week, randomized, double-blind, phase 3 trials (INPULSIS-1 and INPULSIS-2) to evaluate the efficacy and safety of 150 mg of nintedanib twice daily as compared with placebo in patients with idiopathic pulmonary fibrosis. The primary end point was the annual rate of decline in forced vital capacity (FVC). Key secondary end points were the time to the first acute exacerbation and the change from baseline in the total score on the St. George's Respiratory Questionnaire, both assessed over a 52-week period. A total of 1066 patients were randomly assigned in a 3:2 ratio to receive nintedanib or placebo. The adjusted annual rate of change in FVC was -114.7 ml with nintedanib versus -239.9 ml with placebo (difference, 125.3 ml; 95% confidence interval [CI], 77.7 to 172.8; P<0.001) in INPULSIS-1 and -113.6 ml with nintedanib versus -207.3 ml with placebo (difference, 93.7 ml; 95% CI, 44.8 to 142.7; P<0.001) in INPULSIS-2. In INPULSIS-1, there was no significant difference between the nintedanib and placebo groups in the time to the first acute exacerbation (hazard ratio with nintedanib, 1.15; 95% CI, 0.54 to 2.42; P=0.67); in INPULSIS-2, there was a significant benefit with nintedanib versus placebo (hazard ratio, 0.38; 95% CI, 0.19 to 0.77; P=0.005). The most frequent adverse event in the nintedanib groups was diarrhea, with rates of 61.5% and 18.6% in the nintedanib and placebo groups, respectively, in INPULSIS-1 and 63.2% and 18.3% in the two groups, respectively, in INPULSIS-2. In patients with idiopathic pulmonary fibrosis, nintedanib reduced the decline in FVC, which is consistent with a slowing of disease progression; nintedanib was frequently associated with diarrhea, which led to discontinuation of the study medication in less than 5% of patients. (Funded by Boehringer Ingelheim; INPULSIS-1 and INPULSIS-2 ClinicalTrials.gov numbers, NCT01335464 and NCT01335477.).
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              A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.

              Talmadge E King, Williamson Bradford, Socorro Castro-Bernardini … (2014)
              In two of three phase 3 trials, pirfenidone, an oral antifibrotic therapy, reduced disease progression, as measured by the decline in forced vital capacity (FVC) or vital capacity, in patients with idiopathic pulmonary fibrosis; in the third trial, this end point was not achieved. We sought to confirm the beneficial effect of pirfenidone on disease progression in such patients. In this phase 3 study, we randomly assigned 555 patients with idiopathic pulmonary fibrosis to receive either oral pirfenidone (2403 mg per day) or placebo for 52 weeks. The primary end point was the change in FVC or death at week 52. Secondary end points were the 6-minute walk distance, progression-free survival, dyspnea, and death from any cause or from idiopathic pulmonary fibrosis. In the pirfenidone group, as compared with the placebo group, there was a relative reduction of 47.9% in the proportion of patients who had an absolute decline of 10 percentage points or more in the percentage of the predicted FVC or who died; there was also a relative increase of 132.5% in the proportion of patients with no decline in FVC (P<0.001). Pirfenidone reduced the decline in the 6-minute walk distance (P=0.04) and improved progression-free survival (P<0.001). There was no significant between-group difference in dyspnea scores (P=0.16) or in rates of death from any cause (P=0.10) or from idiopathic pulmonary fibrosis (P=0.23). However, in a prespecified pooled analysis incorporating results from two previous phase 3 trials, the between-group difference favoring pirfenidone was significant for death from any cause (P=0.01) and from idiopathic pulmonary fibrosis (P=0.006). Gastrointestinal and skin-related adverse events were more common in the pirfenidone group than in the placebo group but rarely led to treatment discontinuation. Pirfenidone, as compared with placebo, reduced disease progression, as reflected by lung function, exercise tolerance, and progression-free survival, in patients with idiopathic pulmonary fibrosis. Treatment was associated with an acceptable side-effect profile and fewer deaths. (Funded by InterMune; ASCEND ClinicalTrials.gov number, NCT01366209.).
              Article 0 comments Cited 729 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Eur Respir Rev
                Journal ID (iso-abbrev): Eur Respir Rev
                Journal ID (publisher-id): ERR
                Journal ID (hwp): errev
                Title: European Respiratory Review
                Publisher: European Respiratory Society
                ISSN (Print): 0905-9180
                ISSN (Electronic): 1600-0617
                Publication date Collection: 31 March 2018
                Publication date (Electronic): 24 January 2018
                Volume: 27
                Issue: 147
                Electronic Location Identifier: 170062
                Affiliations
                [1 ]Service de Pneumologie et Explorations Fonctionnelles Respiratoires, Hôpital Bretonneau, Tours, France
                [2 ]Université François Rabelais, Tours, France
                [3 ]CEPR/INSERM UMR1100, LabexMabImprove, Tours, France
                [4 ]AP-HP, Hôpital Bichat, Service d'Anatomie Pathologique, Paris, France
                [5 ]Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France
                [6 ]INSERM UMR1152, LabexInflamex, Paris, France
                [7 ]AP-HP, Hôpital Cochin, Service de Physiologie-Explorations Fonctionnelles, Paris, France
                [8 ]Université Paris Descartes, Paris, France
                [9 ]AP-HP, Hôpital Bichat, Service de Physiologie-Explorations Fonctionnelles, Paris, France
                [10 ]AP-HP, Hôpital Bichat, Service de Pneumologie A, DHU FIRE, Paris, France
                Author notes
                Bruno Crestani, Hôpital Bichat, Service de Pneumologie A, 45 rue Henri Huchard, 75018 Paris, France. E-mail: bruno.crestani@ 123456aphp.fr
                Article
                Publisher ID: ERR-0062-2017
                DOI: 10.1183/16000617.0062-2017
                PMC ID: 9489199
                PubMed ID: 29367408
                SO-VID: ae01055c-59b7-4d0b-96f5-e4e1c7432d60
                Copyright © Copyright ©ERS 2018.
                License:

                ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

                History
                Date received : 22 May 2017
                Date accepted : 15 October 2017
                Categories
                Subject: Review
                Subject: 10
                Subject: 13

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