Diffuse alveolar haemorrhage in children: an international multicentre study

Astrid Madsen Ring*, Nicolaus Schwerk, Nural Kiper, Ayse Tana Aslan, Paul Aurora, Roser Ayats, Ines Azevedo, Teresa Bandeira, Julia Carlens, Silvia Castillo-Corullon, Nazan Cobanoglu, Basil Elnazir, Nagehan Emiralioğlu, Tugba Sismanlar Eyuboglu, Michael Fayon, Tugba Ramaslı Gursoy, Claire Hogg, Karsten Kötz, Bülent Karadag, Vendula LátalováKatarzyna Krenke, Joanna Lange, Effrosyni D Manali, Borja Osona, Spyros Papiris, Marijke Proesmann, Philippe Reix, Lea Roditis, Sune Rubak, Nisreen Rumman, Deborah Snijders, Florian Stehling, Laurence Weiss, Ebru Yalcın, Fazilcan Zirek, Andrew Bush, Annick Clement, Matthias Griese, Frederik Fouirnaies Buchvald, Nadia Nathan, Kim Gjerum Nielsen

*Corresponding author af dette arbejde


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.

TidsskriftERJ Open Research
Udgave nummer2
StatusUdgivet - mar. 2023


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