Conflict of interest: None.
Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide†
Article first published online: 9 MAY 2012
Copyright © 2012 Wiley Periodicals, Inc.
Volume 48, Issue 3, pages 303–305, March 2013
How to Cite
Flanagan, F., Glackin, L. and Slattery, D. M. (2013), Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide. Pediatr. Pulmonol., 48: 303–305. doi: 10.1002/ppul.22583
- Issue published online: 16 FEB 2013
- Article first published online: 9 MAY 2012
- Manuscript Accepted: 4 APR 2012
- Manuscript Received: 25 OCT 2011
- none reported
Idiopathic pulmonary hemosiderosis (IPH), a subtype of diffuse alveolar hemorrhage is a rare condition, first described by Virchow in 1864. Historically, it manifests in children in the first decade of life with the combination of hemoptysis, iron deficiency anemia, and alveolar infiltrates on chest radiograph. More recently, diffuse alveolar hemorrhage has been classified by the absence or presence of pulmonary capillaritis (PC), the latter carrying a potential for a poorer outcome. While systemic corticosteroids remain the first line treatment option, other immune modulators have been trailed including hydroxychloroquine, azathioprine, 6-mercaptopurine, and cyclophosphamide with varying results. Our case demonstrates for the first time, the successful use of intravenous cyclophosphamide in the management of chronic idiopathic PC. Pediatr Pulmonol. 2013; 48:303–305. © 2012 Wiley Periodicals, Inc.