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

AbstractOBJECTIVE:
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.
AuthorsJason J Fullmer, Claire Langston, Megan K Dishop, Leland L Fan
JournalThe Journal of pediatrics (J Pediatr) Vol. 146 Issue 3 Pg. 376-81 (Mar 2005) ISSN: 0022-3476 [Print] United States
PMID15756223 (Publication Type: Comparative Study, Journal Article)
Topics
  • Biopsy
  • Blood Sedimentation
  • Capillaries (pathology)
  • Child
  • Female
  • Hematocrit
  • Hemorrhage (etiology, pathology)
  • Humans
  • Lung (blood supply)
  • Lung Diseases (diagnosis, etiology)
  • Male
  • Pulmonary Alveoli (pathology)
  • Retrospective Studies
  • Vasculitis (diagnosis)

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