Logo Logo
Switch Language to German

Seidl, Elias ORCID logoORCID: https://orcid.org/0000-0001-6610-3756; Schwerk, Nicolaus ORCID logoORCID: https://orcid.org/0000-0002-5496-7749; Carlens, Julia ORCID logoORCID: https://orcid.org/0000-0001-8578-864X; Wetzke, Martin ORCID logoORCID: https://orcid.org/0000-0002-1462-9569; Emiralioğlu, Nagehan ORCID logoORCID: https://orcid.org/0000-0002-1405-8401; Kiper, Nural ORCID logoORCID: https://orcid.org/0000-0003-1261-7393; Lange, Joanna ORCID logoORCID: https://orcid.org/0000-0003-4165-6455; Krenke, Katarzyna ORCID logoORCID: https://orcid.org/0000-0002-8370-784X; Szepfalusi, Zsolt ORCID logoORCID: https://orcid.org/0000-0003-4852-3102; Stehling, Florian ORCID logoORCID: https://orcid.org/0000-0002-2417-2048; Baden, Winfried; Hämmerling, Susanne; Jerkic, Silvija-Pera ORCID logoORCID: https://orcid.org/0000-0003-2972-649X; Proesmans, Marijke ORCID logoORCID: https://orcid.org/0000-0002-5576-9517; Ullmann, Nicola ORCID logoORCID: https://orcid.org/0000-0003-1111-5690; Buchvald, Frederik ORCID logoORCID: https://orcid.org/0000-0003-4196-3893; Knoflach, Katrin; Kappler, Matthias ORCID logoORCID: https://orcid.org/0000-0003-3889-9552 and Griese, Matthias ORCID logoORCID: https://orcid.org/0000-0003-0113-912X (2022): Acute exacerbations in children’s interstitial lung disease. In: Thorax, Vol. 77, No. 8: pp. 799-804 [PDF, 1MB]


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.

Actions (login required)

View Item View Item