Diffuse alveolar haemorrhage in children: an international multicentre study
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Methods A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years.
Results Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0–12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function.
Conclusions Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.
Originalsprog | Engelsk |
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Artikelnummer | 00733-2022 |
Tidsskrift | ERJ Open Research |
Vol/bind | 9 |
Udgave nummer | 2 |
Antal sider | 13 |
ISSN | 2312-0541 |
DOI | |
Status | Udgivet - 2023 |
Bibliografisk note
Funding Information:
Conflicts of interest: T. Bandeira reports personal fees from Sanofi and other support from Boehringer Ingelheim, outside the submitted work. E.D. Manali reports other from Boehringer Ingelheim, other from Bering, other from Hoffman la Roche, outside the submitted work. S. Papiris reports grants and other support from Boehringer Ingelheim and Hoffman la Roche, and other support from Savara, outside the submitted work. M. Griese reports grants, personal fees and nonfinancial support from Boehringer Ingelheim for an advisory board on nintedanib, outside the submitted work. The remaining authors have nothing to disclose.
Funding Information:
Support statement: This work was supported by the COST Action CA16125 European Network for Translational Research in Children’s and Adult Interstitial Lung Disease, by the Clinical Research Collaboration for chILD of the European Respiratory Society and by the Children’s Lung Foundation Denmark.
Publisher Copyright:
© The authors 2023.
ID: 366379982