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Diffuse alveolar haemorrhage in children: an international multicentre study
Astrid Madsen Ring1, Nicolaus Schwerk2, Nural Kiper3
1Paediatric Pulmonary Service, Dept of Paediatrics and Adolescent Medicine, Copenhagen, University Hospital, Rigshospitalet, Copenhagen, Denmark.
Insights
Paediatric diffuse alveolar haemorrhage (DAH) is a rare, heterogeneous lung condition in children. This study highlights its varied causes, severe outcomes, and the need for evidence-based treatments.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Disease
Background:
- Diffuse alveolar haemorrhage (DAH) in children is a rare and poorly understood condition.
- Limited data exists on the clinical presentation, treatment strategies, and patient outcomes.
- Understanding DAH is crucial for improving pediatric respiratory care.
Purpose of the Study:
- To investigate the clinical characteristics, causes, treatments, and outcomes of pediatric DAH.
- To establish a comprehensive overview of this rare condition across multiple European centers.
- To identify areas for future research and evidence-based guideline development.
Main Methods:
- A retrospective, descriptive, multicenter follow-up study.
- Data collected from 117 pediatric patients diagnosed with DAH before age 18.
- Initiated through the European network for translational research in interstitial lung disease.
Main Results:
- The study identified 117 pediatric DAH cases with diverse etiologies, including idiopathic pulmonary haemosiderosis and autoimmune disorders.
- Common symptoms were anemia, hemoptysis, dyspnea, and cough, with 23% presenting without respiratory symptoms.
- Systemic corticosteroids were the most frequent treatment (93%), with a 13% overall mortality rate and persistent radiological/functional deficits.
Conclusions:
- Pediatric DAH is a heterogeneous condition with significant clinical variability and severe, often chronic, outcomes.
- The high mortality and persistent disease burden underscore the severity of pediatric DAH.
- This study provides a foundation for future prospective trials to establish evidence-based treatment and follow-up recommendations.
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.
