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      Diurnal variation in forced vital capacity in patients with fibrotic interstitial lung disease using home spirometry

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          Abstract

          Forced vital capacity (FVC) is used as the routine physiological measure to assess disease progression in fibrotic interstitial lung diseases (f-ILDs) [1]. New drugs are currently being investigated on top of “standard care” with antifibrotic drugs in idiopathic pulmonary fibrosis (IPF) and other f-ILD, resulting in small margins of change in FVC [2, 3]. Recently, the first trial of antifibrotic medication in patients with systemic sclerosis-associated interstitial lung disease has shown a numerically small but significant lower annualised rate of FVC decline (41 mL) in patients treated with nintedanib compared with placebo [3].

          Abstract

          This study demonstrates a diurnal variation in FVC measured with home spirometry in patients with f-ILD, with a higher FVC in the morning than in the afternoon http://bit.ly/37SQtBK

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          Most cited references8

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          Nintedanib for Systemic Sclerosis–Associated Interstitial Lung Disease

          Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis and a leading cause of systemic sclerosis-related death. Nintedanib, a tyrosine kinase inhibitor, has been shown to have antifibrotic and antiinflammatory effects in preclinical models of systemic sclerosis and ILD.
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            Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial

            At present, no approved pharmacotherapies are available for unclassifiable interstitial lung disease (ILD), which is characterised by progressive fibrosis of the lung. We aimed to assess the efficacy and safety of pirfenidone in patients with progressive fibrosing unclassifiable ILD.
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              The development and validation of the King's Brief Interstitial Lung Disease (K-BILD) health status questionnaire.

              Health status is impaired in patients with interstitial lung disease (ILD). There is a paucity of tools that assess health status in ILD. The objective of this study was to develop and validate the King's Brief Interstitial Lung Disease questionnaire (K-BILD), a new health status measure for patients with ILD. Patients with ILD were recruited from outpatient clinics. The development of the questionnaire consisted of three phases: item generation; item reduction, allocation to domains by factor analysis, Rasch analysis to create unidimensional scales and validation; and repeatability testing. 173 patients with ILD (49 with idiopathic pulmonary fibrosis) completed a preliminary 71-item questionnaire. 56 items were removed due to redundancy, low factor loadings or poor fit to the Rasch model. The final version of the K-BILD questionnaire consisted of 15 items and three domains (breathlessness and activities, chest symptoms and psychological). Internal consistency assessed with Cronbach's α coefficient was 0.94 for the K-BILD total score. Concurrent validity of the K-BILD questionnaire was high compared with St George's Respiratory Questionnaire (r=0.90) and moderate with lung function (vital capacity, r=0.50). The K-BILD questionnaire was repeatable over 2 weeks (n=44), with intraclass correlation coefficients for domains and total score 0.86-0.94. The K-BILD construct validity for patients with idiopathic pulmonary fibrosis was similar to that of other ILDs. The K-BILD questionnaire is a brief, valid, self-completed health status measure for ILD. It could be used in the clinic to assess ILD from the patients' perspective.
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                Author and article information

                Journal
                ERJ Open Res
                ERJ Open Res
                ERJOR
                erjor
                ERJ Open Research
                European Respiratory Society
                2312-0541
                January 2020
                06 April 2020
                : 6
                : 1
                : 00054-2020
                Affiliations
                [1 ]Dept of Respiratory Medicine, Erasmus Medical Center, University Hospital Rotterdam, Rotterdam, The Netherlands
                [2 ]National Reference Coordinating Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Lyon, France
                [3 ]Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France
                Author notes
                Marlies S. Wijsenbeek, Dept of Respiratory Medicine, Erasmus Medical Center, University Hospital Rotterdam, 's Gravendijkwal 230, Rotterdam 3015 CA, The Netherlands. E-mail: m.wijsenbeek-lourens@ 123456erasmusmc.nl
                Author information
                https://orcid.org/0000-0002-5591-0955
                Article
                00054-2020
                10.1183/23120541.00054-2020
                7132034
                32280666
                2a147861-8424-499d-a1e3-15791138b364
                Copyright ©ERS 2020

                This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

                History
                : 3 February 2020
                : 18 February 2020
                Funding
                Funded by: Boehringer Ingelheim, open-funder-registry 10.13039/100001003;
                Categories
                Original Research Letters
                13

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