Diffuse alveolar haemorrhage in children: an international multicentre study

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.