Severe autoimmune diffuse alveolar hemorrhage in children; early diagnosis and initiation of therapeutic plasma exchange may improve clinical outcomes

Introduction: Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening pulmonary complication in children, presenting with hemoptysis, anemia, diffuse infiltrates, and respiratory failure. Autoimmune diseases such as systemic lupus erythematosus (SLE) and ANCA-associated vasculitis (AAV) account for 30%-40% of pediatric DAH cases. Pediatric evidence is limited to case reports and small series. We aimed to characterize the clinical course, management, and outcomes of pediatric patients with autoimmune DAH requiring intensive care. Methods: We conducted a retrospective cohort study at a single tertiary pediatric institution, identifying 6 patients admitted to the ICU from 2013 to 2024 with DAH secondary to SLE or AAV. Results: sepsis post-decannulation. The remaining patients survived to discharge. Conclusion: Autoimmune DAH in pediatric patients carries high morbidity, often requiring advanced respiratory support and multimodal immunosuppression. Early diagnostics work up and initiation of TPE may improve outcomes. Further prospective pediatric study would be necessary to guide standardized treatment protocols.

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Publication Details

Journal
Frontiers in Pediatrics
Published
2026-04-07
DOI
https://doi.org/10.3389/fped.2026.1799535
Primary Topic
Vasculitis and related conditions
Type
article
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0.00
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article

Severe autoimmune diffuse alveolar hemorrhage in children; early diagnosis and initiation of therapeutic plasma exchange may improve clinical outcomes

Dai Kimura, Jack Steven Thomas
Frontiers in Pediatrics
Vasculitis and related conditions
article

Severe autoimmune diffuse alveolar hemorrhage in children; early diagnosis and initiation of therapeutic plasma exchange may improve clinical outcomes

Dai Kimura, Jack Steven Thomas
article en

Abstract

Introduction: Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening pulmonary complication in children, presenting with hemoptysis, anemia, diffuse infiltrates, and respiratory failure. Autoimmune diseases such as systemic lupus erythematosus (SLE) and ANCA-associated vasculitis (AAV) account for 30%-40% of pediatric DAH cases. Pediatric evidence is limited to case reports and small series. We aimed to characterize the clinical course, management, and outcomes of pediatric patients with autoimmune DAH requiring intensive care. Methods: We conducted a retrospective cohort study at a single tertiary pediatric institution, identifying 6 patients admitted to the ICU from 2013 to 2024 with DAH secondary to SLE or AAV. Results: sepsis post-decannulation. The remaining patients survived to discharge. Conclusion: Autoimmune DAH in pediatric patients carries high morbidity, often requiring advanced respiratory support and multimodal immunosuppression. Early diagnostics work up and initiation of TPE may improve outcomes. Further prospective pediatric study would be necessary to guide standardized treatment protocols.

Frontiers in PediatricsVol. 14
Le Bonheur Children's Hospital (US)
Good health and well-being
Openalex Percentile: Top 29%
Vasculitis and related conditions
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