TY - JOUR
T1 - Acute exacerbations in children's interstitial lung disease
AU - Seidl, Elias
AU - Schwerk, Nicolaus
AU - Carlens, Julia
AU - Wetzke, Martin
AU - Emiralioğlu, Nagehan
AU - Kiper, Nural
AU - Lange, Joanna
AU - Krenke, Katarzyna
AU - Szepfalusi, Zsolt
AU - Stehling, Florian
AU - Baden, Winfried
AU - Hämmerling, Susanne
AU - Jerkic, Silvija-Pera
AU - Proesmans, Marijke
AU - Ullmann, Nicola
AU - Buchvald, Frederik
AU - Knoflach, Katrin
AU - Kappler, Matthias
AU - Griese, Matthias
AU - chILD EU collaborators
N1 - © Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.
PY - 2022/8
Y1 - 2022/8
N2 - 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.
AB - 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.
KW - Child
KW - Humans
KW - Lung
KW - Lung Diseases, Interstitial
KW - Quality of Life
KW - Surveys and Questionnaires
UR - http://www.scopus.com/inward/record.url?scp=85127427260&partnerID=8YFLogxK
U2 - 10.1136/thoraxjnl-2021-217941
DO - 10.1136/thoraxjnl-2021-217941
M3 - Journal article
C2 - 35149584
VL - 77
SP - 799
EP - 804
JO - Thorax
JF - Thorax
SN - 0040-6376
IS - 8
M1 - 217941
ER -