BACKGROUND: Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. 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.

2nd Pulmonary Medicine Department General University Hospital Athens Medical School National and Kapodistrian University of Athens Athens Greece

CHI at Tallaght University Hospital Dublin Ireland

Clinic for Paediatric Pneumology Allergology and Neonatology Hannover Medical School German Center for Lung Research Hannover Germany

Clinic for Paediatrics 3 University Hospital Essen Essen Germany

Danish Center of Pediatric Pulmonology and Allergology Department of Pediatrics and Adolescents Medicine University Hospital of Aarhus Aarhus Denmark

Departamento de Ginecologia Obstetrícia e Pediatria Faculdade de Medicina Universidade do Porto and Serviço de Pediatria Centro Hospitalar Universitário de S João Porto Portugal

Department of Clinical Medicine University of Copenhagen Copenhagen Denmark

Department of Pediatric Pneumonology and Allergy Medical University of Warsaw Warsaw Poland

Department of Pediatrics Pediatric Pulmonology Division Ankara University Faculty of Medicine Ankara Turkey

Department of Pediatrics Respiratory Unit Hospital de Santa Maria Centro Hospitalar Universitário Lisboa Norte Lisbon Portugal

Department of Pediatrics University Hospital Olomouc Olomouc Czech Republic

Dept of Pediatric Pulmonology and Allergy Children's University Hospital Toulouse France

Devision of Paediatrics Drottning Silvias Barn och Ungdomssjukhus University of Gothenburg Gothenburg Sweden

Dipartimento Salute della Donna e del Bambino Università degli Studi di Padova Padua Italy

Division of Paediatric Pulmonology Marmara University Faculty of Medicine Istanbul Turkey

Gazi University Faculty of Medicine Department of Pediatric Pulmonology Ankara Turkey

Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology Ankara Turkey

Imperial College London and Paediatric Respiratory Medicine Royal Brompton and Harefield NHS Foundation Trust London UK

Paediatric Pulmonary Service Dept of Paediatrics and Adolescent Medicine Copenhagen University Hospital Rigshospitalet Copenhagen Denmark

Pediatric Department Makassed Hospital East Jerusalem Palestine

Pediatric Pulmonology and Allergology Department Parc Taulí Hospital Universitari Institut d'Investigació i Innovació Parc Taulí Universitat Autónoma de Barcelona Barcelona Spain

Pediatric Pulmonology Clinical Hospital of Valencia Valencia Spain

Pediatric Pulmonology Department CHU Bordeaux Bordeaux France

Pediatric Pulmonology Department Son Espases University Hospital Palma de Mallorca Spain

Pediatric Pulmonology KUL UZ Gasthuisberg Leuven Belgium

Respiratory Unit Great Ormond Street Hospital for Children NHS Foundation Trust London UK

Royal Brompton Hospital London UK

Sorbonne Université Pediatric Pulmonology and Reference Center for rare lung diseases RespiRare Inserm U933 Laboratory of childhood genetic diseases Armand Trousseau Hospital APHP Paris France

Strasbourg University Hôpital de Hautepierre Pediatric Pulmonology Department Strasbourg France

These authors contributed equally

Université de Lyon Hôpital Femme Mère Enfant Pediatric Pulmonology Department Lyon France

ERJ Open Res. 2023 May 30;9(3):50733-2022. doi: 10.1183/23120541.50733-2022. PubMed

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