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


METHODS
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).


CONCLUSIONS
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.
Fullmer
Jason J
JJ
Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston 77030, USA. jjfullme@texaschildrenshospital.org
Langston
Claire
C
Dishop
Megan K
MK
Fan
Leland L
LL
D001706:Biopsy; D001799:Blood Sedimentation; D002196:Capillaries / Q000473:pathology*; D002648:Child; D005260:Female; D006400:Hematocrit; D006470:Hemorrhage / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008168:Lung / Q000098:blood supply*; D008171:Lung Diseases / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D011650:Pulmonary Alveoli / Q000473:pathology*; D012189:Retrospective Studies; D014657:Vasculitis / Q000175:diagnosis*