Pulmonary capillaritis in children: a review of eight cases with comparison to other alveolar hemorrhage syndromes

J Pediatr. 2005 Mar;146(3):376-81. doi: 10.1016/j.jpeds.2004.10.025.


Objective: To review clinical, laboratory, and outcome characteristics of children diagnosed with pulmonary capillaritis (PC), a small-vessel vasculitis, presenting as diffuse alveolar hemorrhage (DAH), and to compare these findings with those for children with other alveolar hemorrhage syndromes.

Study design: A retrospective chart review of patients who underwent a lung biopsy because of a clinical suggestion of pulmonary hemorrhage.

Results: PC was identified in 8 of 23 patients. In these patients, cough, crackles, and hypoxia were common. Alveolar infiltrates on radiography and anemia were present in 7 of 8 cases. Serologic evidence of a systemic vasculitis was present in 50% of patients. High-dose corticosteroids proved effective in controlling alveolar hemorrhage in all cases. There were no presenting signs or symptoms that could differentiate patients with PC from those with non-immune-mediated alveolar hemorrhage. In general, patients with PC had a lower hematocrit and higher erythrocyte sedimentation rate (ESR).

Conclusion: Children presenting with lower respiratory tract symptoms, chest x-ray abnormalities, and anemia should undergo evaluation for PC, as early initiation of immunosuppression can be lifesaving and organ sparing. No clinical signs to differentiate immune and non-immune-mediated alveolar hemorrhage were evident in this study.

Publication types

  • Comparative Study

MeSH terms

  • Biopsy
  • Blood Sedimentation
  • Capillaries / pathology*
  • Child
  • Female
  • Hematocrit
  • Hemorrhage / etiology*
  • Hemorrhage / pathology
  • Humans
  • Lung / blood supply*
  • Lung Diseases / diagnosis*
  • Lung Diseases / etiology
  • Male
  • Pulmonary Alveoli / pathology*
  • Retrospective Studies
  • Vasculitis / diagnosis*