Design of a Study Assessing Disease Behaviour During the Peri-Diagnostic Period in Patients with Interstitial Lung Disease: The STARLINER Study

Marlies Wijsenbeek, Elisabeth Bendstrup, Claudia Valenzuela, Michael T Henry, Catharina Moor, Monica Bengus, Andras Perjesi, Frank Gilberg, Klaus-Uwe Kirchgaessler, Carlo Vancheri, Marlies Wijsenbeek, Elisabeth Bendstrup, Claudia Valenzuela, Michael T Henry, Catharina Moor, Monica Bengus, Andras Perjesi, Frank Gilberg, Klaus-Uwe Kirchgaessler, Carlo Vancheri

Abstract

Background/objectives: This study will aim to characterise disease behaviour during the peri-diagnostic period in patients with suspected interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF), using daily home spirometry and accelerometry. Additionally, this study will aim to increase collaboration between secondary and tertiary centres using a digital collaboration platform.

Methods: The STARLINER study (NCT03261037) will enrol approximately 180 symptomatic patients aged 50 years or more with radiological evidence of ILD/IPF from community and tertiary centres in Canada and Europe. Approximately two-thirds of sites will be community centres. Patients will be followed during pre-diagnosis (inclusion to diagnosis; up to a maximum of 12 months) and post-diagnosis (diagnosis to treatment initiation; up to a maximum of 6 months). The study will be facilitated by a digital ecosystem consisting of the devices used for home-based assessments and a digital collaboration platform enabling communication between community and tertiary centres, and between clinicians and patients.

Planned outcomes: The primary endpoint will be time-adjusted semi-annual change in forced vital capacity (FVC; in millilitres) during the peri-diagnostic period. Physical functional capacity and patient-reported outcomes (PROs) will also be assessed. FVC and physical functional capacity will be measured using daily home spirometry and accelerometry, and at site visits using spirometry and the 6-min walk test. PROs will be assessed prior to, or during, site visits and will always be completed in the same order.

Conclusions: Findings from this study may help to facilitate the early and accurate diagnosis of ILDs by increasing knowledge about disease progression, enabling collaboration between community and tertiary centres and improving communication between clinicians and patients.

Trial registration number: NCT03261037.

Funding: F. Hoffmann-La Roche, Ltd., Basel, Switzerland. Plain language summary available for this article.

Keywords: Digital ecosystem; Disease behaviour; Home-based assessment; Idiopathic pulmonary fibrosis; Interstitial lung disease; Pulmonary/respiratory; Virtual multidisciplinary team.

Figures

Fig. 1
Fig. 1
Study procedures. ILD interstitial lung disease, IPF idiopathic pulmonary fibrosis. aPatients will leave the study if there is no diagnosis within 12 months of inclusion or a non-ILD diagnosis
Fig. 2
Fig. 2
Digital ecosystem. FVC forced vital capacity, ILD interstitial lung disease, IPF idiopathic pulmonary fibrosis, MDT multidisciplinary team

References

    1. Ryerson CJ, Collard HR. Update on the diagnosis and classification of ILD. Curr Opin Pulm Med. 2013;19(5):453–459. doi: 10.1097/MCP.0b013e328363f48d.
    1. Skolnik K, Ryerson CJ. Unclassifiable interstitial lung disease: a review. Respirology. 2016;21(1):51–56. doi: 10.1111/resp.12568.
    1. European Respiratory Society. Interstitial lung diseases. In: Gibson GJ, Loddenkemper R, Sibille Y, Lundbäck B, Fletcher M, editors. European lung white book. Wakefield: Charlesworth; 2013. . Accessed 26 Oct 2018.
    1. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis. 2008;3:8. doi: 10.1186/1750-1172-3-8.
    1. Margaritopoulos GA, Harari S, Caminati A, Antoniou KM. Smoking-related idiopathic interstitial pneumonia: a review. Respirology. 2016;21(1):57–64. doi: 10.1111/resp.12576.
    1. Prasad R, Gupta N, Singh A, Gupta P. Diagnosis of idiopathic pulmonary fibrosis: current issues. Intractable Rare Dis Res. 2015;4(2):65–69. doi: 10.5582/irdr.2015.01009.
    1. Caminati A, Cassandro R, Torre O, Harari S. Severe idiopathic pulmonary fibrosis: what can be done? Eur Respir Rev. 2017;26(145):170047. doi: 10.1183/16000617.0047-2017.
    1. King TE, Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–2092. doi: 10.1056/NEJMoa1402582.
    1. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–1769. doi: 10.1016/S0140-6736(11)60405-4.
    1. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–2082. doi: 10.1056/NEJMoa1402584.
    1. Cottin V, Richeldi L. Neglected evidence in idiopathic pulmonary fibrosis and the importance of early diagnosis and treatment. Eur Respir Rev. 2014;23(131):106–110. doi: 10.1183/09059180.00008613.
    1. Thickett DR, Kendall C, Spencer LG, et al. Improving care for patients with idiopathic pulmonary fibrosis (IPF) in the UK: a round table discussion. Thorax. 2014;69(12):1136–1140. doi: 10.1136/thoraxjnl-2014-206284.
    1. Cosgrove GP, Bianchi P, Danese S, Lederer DJ. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018;18(1):9. doi: 10.1186/s12890-017-0560-x.
    1. Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med. 2011;184(7):842–847. doi: 10.1164/rccm.201104-0668OC.
    1. Russell AM, Ripamonti E, Vancheri C. Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients’ perspectives of the disease and treatment. BMC Pulm Med. 2016;16:10. doi: 10.1186/s12890-016-0171-y.
    1. Bonella F, Wijsenbeek M, Molina-Molina M, et al. European IPF patient charter: unmet needs and a call to action for healthcare policymakers. Eur Respir J. 2016;47(2):597–606. doi: 10.1183/13993003.01204-2015.
    1. De Sadeleer LJ, Meert C, Yserbyt J, et al. Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: a retrospective observational study of 938 cases. Chest. 2018;153(6):1416–1423. doi: 10.1016/j.chest.2018.03.026.
    1. Walsh SLF, Wells AU, Desai SR, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study. Lancet Respir Med. 2016;4(7):557–565. doi: 10.1016/S2213-2600(16)30033-9.
    1. De Boer K, Aravena C, Sood R, et al. Virtual multidisciplinary discussion: feasibility and diagnostic concordance with face-to-face multidisciplinary discussion. QJM Int J Med. 2016;109(1):S43–S44.
    1. Wells AU. Managing diagnostic procedures in idiopathic pulmonary fibrosis. Eur Respir Rev. 2013;22(128):158–162. doi: 10.1183/09059180.00001213.
    1. Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708–719. doi: 10.1016/S2213-2600(16)30152-7.
    1. Bahmer T, Kirsten AM, Waschki B, et al. Clinical correlates of reduced physical activity in idiopathic pulmonary fibrosis. Respiration. 2016;91(6):497–502. doi: 10.1159/000446607.
    1. Bahmer T, Kirsten AM, Waschki B, et al. Prognosis and longitudinal changes of physical activity in idiopathic pulmonary fibrosis. BMC Pulm Med. 2017;17(1):104. doi: 10.1186/s12890-017-0444-0.
    1. Moor CC, van Manen MJG, Tak NC, van Noort E, Wijsenbeek MS. Development and feasibility of an eHealth tool for idiopathic pulmonary fibrosis. Eur Respir J. 2018;51(3):1702508. doi: 10.1183/13993003.02508-2017.
    1. Moor CC, Wapenaar M, Miedema JR, Geelhoed JJM, Chandoesing PP, Wijsenbeek MS. A home monitoring program including real-time wireless home spirometry in idiopathic pulmonary fibrosis: a pilot study on experiences and barriers. Respir Res. 2018;19(1):105. doi: 10.1186/s12931-018-0810-3.
    1. Russell AM, Adamali H, Molyneaux PL, et al. Daily home spirometry: an effective tool for detecting progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;194(8):989–997. doi: 10.1164/rccm.201511-2152OC.
    1. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6(2):138–153. doi: 10.1016/S2213-2600(17)30433-2.
    1. Johannson KA, Vittinghoff E, Morisset J, Lee JS, Balmes JR, Collard HR. Home monitoring improves endpoint efficiency in idiopathic pulmonary fibrosis. Eur Respir J. 2017;50(1):1602406. doi: 10.1183/13993003.02406-2016.
    1. Maher TM, Molina-Molina M, Russell AM, et al. Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries. BMC Pulm Med. 2017;17(1):124. doi: 10.1186/s12890-017-0468-5.
    1. Collard HR, Tino G, Noble PW, et al. Patient experiences with pulmonary fibrosis. Respir Med. 2007;101(6):1350–1354. doi: 10.1016/j.rmed.2006.10.002.
    1. Wuyts WA, Cavazza A, Rossi G, Bonella F, Sverzellati N, Spagnolo P. Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic? Eur Respir Rev. 2014;23(133):308–319. doi: 10.1183/09059180.00004914.
    1. Irish Lung Fibrosis Association. National patient charter for idiopathic pulmonary fibrosis; 2015. . Accessed 26 Oct 2018.
    1. van der Heijden M, Lucas PJ, Lijnse B, Heijdra YF, Schermer TR. An autonomous mobile system for the management of COPD. J Biomed Inform. 2013;46(3):458–469. doi: 10.1016/j.jbi.2013.03.003.
    1. Mohktar MS, Redmond SJ, Antoniades NC, et al. Predicting the risk of exacerbation in patients with chronic obstructive pulmonary disease using home telehealth measurement data. Artif Intell Med. 2015;63(1):51–59. doi: 10.1016/j.artmed.2014.12.003.
    1. Carvajalino S, Reigada C, Johnson M, Dzingina M, Bajwah S. Symptom prevalence of patients with fibrotic interstitial lung disease: a systematic literature review. BMC Pulm Med. 2018;18(1):78. doi: 10.1186/s12890-018-0651-3.
    1. De Kleijn W, De Vries J, Lower E, Elfferich M, Baughman R, Drent M. Fatigue in sarcoidosis: a systematic review. Curr Opin Pulm Med. 2009;15(5):499–506. doi: 10.1097/MCP.0b013e32832d0403.
    1. van Manen MJ, Geelhoed JJ, Tak NC, Wijsenbeek MS. Optimizing quality of life in patients with idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2017;11(3):157–169. doi: 10.1177/1753465816686743.
    1. Wijsenbeek M, van Manen M, Bonella F. New insights on patient-reported outcome measures in idiopathic pulmonary fibrosis: only PROMises? Curr Opin Pulm Med. 2016;22(5):434–441. doi: 10.1097/MCP.0000000000000294.

Source: PubMed

Patrocinadores e Colaboradores

Condições médicas

Intervenções de drogas

3
Se inscrever