Introduction Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics and impact of AEs on children's interstitial lung disease (chILD).
Methods The Kids Lung Register collected data on AEs, the clinical course and quality of life (patient-reported outcomes - PRO) of rare paediatric lung diseases. Characteristics of AEs were obtained.
Results Data of 2822 AEs and 2887 register visits of 719 patients with chILD were recorded. AEs were characterised by increased levels of dyspnoea (74.1%), increased respiratory rate (58.6%) and increased oxygen demand (57.4%). Mostly, infections (94.4%) were suspected causing an AE. AEs between two register visits revealed a decline in predicted FEV1 (median −1.6%, IQR −8.0 to 3.9; p=0.001), predicted FVC (median −1.8%, IQR −7.5 to 3.9; p=0.004), chILD-specific questionnaire (median −1.3%, IQR −3.6 to 4.5; p=0.034) and the physical health summary score (median −3.1%, IQR −15.6 to 4.3; p=0.005) compared with no AEs in between visits. During the median observational period of 2.5 years (IQR 1.2–4.6), 81 patients died. For 49 of these patients (60.5%), mortality was associated with an AE.
Conclusion This is the first comprehensive study analysing the characteristics and impact on the clinical course of AEs in chILD. AEs have a significant and deleterious effect on the clinical course and health-related quality of life in chILD.
- paediatric interstitial lung disease
Data availability statement
Data are available upon reasonable request.
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Collaborators The chILD-EU collaborators areTugba Sysmanlar (Ankara Gazi University, Turkey), Ayse Aslan (Ankara Gazi University, Turkey), Susanne Zeidler (Asklepios Sankt Augustin Krankenhaus, Germany), Veronika Baumeister (Bochum Ruhr Universität, Germany), Folke Brinkmann (Bochum Ruhr Universität, Germany), Isabelle Rochat (Centre hospitalier universitaire vaudois Lausanne, Switzerland), Sebastian Becker (Darmstädter Kinderkliniken, Germany), Meike Köhler (Dr. von Hauner Children’s Hospital, University Hospital LMU Munich, Germany), Andrea Schams (Dr. von Hauner Children’s Hospital, University Hospital LMU Munich, Germany), Waltraud Wesselak (Dr. von Hauner Children’s Hospital, University Hospital LMU Munich, Germany), Mandeep Kaur (Dr. von Hauner Children’s Hospital, University Hospital LMU Munich, Germany), Silvia Castillo Corullón (Fundación de Investigación del Hospital Clínico Universitario de Valencia, Spain), Lale Bayir (Hannover Medical School, Germany), Nagehan Ciftci (Hannover Medical School, Germany), Steve Cunningham (Centre for Inflammation Research, University of Edinburgh, UK), Lauren McCann (Imperial College London, United Kingdom), Chiara Carolina Abbas (Inselspital Bern, Switzerland), Tobias Ehrenreich (Inselspital Bern, Switzerland), Philipp Latzin (Inselspital Bern, Switzerland), Ayse Aizit Kilinc (Istanbul Cerrahpasa University, Medicine Faculty Hospital, Turkey), Tobias Hübner (Kinderklinik Oldenburg, Germany), Holger Köster (Kinderklinik Oldenburg, Germany), Martin Schebek (Klinikum Kassel, Germany), Matteo Fontana (Luzerner Kantonsspital, Switzerland), Nicolas Regamey (Luzerner Kantonsspital, Switzerland), Stanislaw Boguslawski (Medical University of Warsaw, Poland), Honorata Marczak (Medical University of Warsaw, Poland), Deborah Adelsberger (Oldenburg Zentrum für Kinder- und Jugendmedizin, Germany), Achim Freihorst (Ostalab Klinikum Aalen, Germany), Frederik Buchvald (Rigshospitalet Copenhagen, Denmark), Gemma Donohoe (Royal Children’s Hospital Manchester, United Kingdom), Morag MacLean (Royal Hospital for Sick Children Edinburgh, United Kingdom), Fiona Redmond (Royal Hospital for Sick Children Edinburgh, United Kingdom), Cordula Koerner-Rettberg (Ruhr University-Bochum, Germany), Abigel Kolonics-Farkas (Semmelweis University, Hungary), Sarah Shortland (Sheffield Children’s Hospital, United Kingdom), Amy Collier (Southampton General Hospital, United Kingdom), Kathrin Seidemann (Städtische Klinikum Karlsruhe, Germany), Lutz Nährlich (Universität Giessen, Germany), Peter Meißner (Universität Ulm, Germany), Susanne Hämmerling (Universitätsklinikum Heidelberg, Germany), Benjamin Hanebeck (Universitätsklinikum Heidelberg, Germany), Dominik Schöndorf (Universitätsklinikum Heidelberg, Germany), Olaf Sommerburg (Universitätsklinikum Heidelberg, Germany), Winfried Baden (Universitätsklinikum Tübingen, Germany), Sune Rubak (University Aarhus, Denmark), Steve Turner (University Aberdeen, United Kingdom), Martin Rosewich (University Frankfurt/Main, Germany), Stefan Zielen (University Frankfurt/Main, Germany), Johannes Schulze (University Frankfurt/Main, Germany), Christiane Lex (University Göttignen, Germany), Frans De Baets (University Hospital Gent, Belgium), Edyta Glowacka (University Krakau, Poland), Freerk Prenzel (University Leipzig, Germany), Nazan Cobanoglu (University of Ankara, Turkey), Gisela Anthony (University of Marburg, Germany), Hans Rock (University of Marburg, Germany), Angelo Barbato (University of Padua, Italy), Deborah Snijders (University of Padua, Italy), Andrea Heinzmann (Universtätsklinikum Freiburg, Germany), Dora Krikovszky (Semmelweis University, Budapest, Hungary).
Contributors ES developed the theoretical framework, processed the analytic calculations, performed the data interpretation and wrote the manuscript. MG is full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish. He is responsible for the overall content as the guarantor. MG designed the project, organised and developed the platform and reviewed the cases. Clinical principal site investigators of the project were ES, NK, JC, MW, NE, NK, JL, KK, ZS, FS, WB, SH, S-PJ, MP, NU, FB, KK, MK and MG. All authors participated in discussions for conclusion of the project and reviewed the manuscript.
Funding MG is supported by DFG (Gr 970/9-1), FP7 Coordination of Non‐Community Research Programmes (Gr FP7‐305653‐chILD‐EU), Cost CA (Gr 16125 ENTeR-chILD) and European Respiratory Society Clinical Research Collaboration (Gr not applicable).
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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