title: Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.
abstract: 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.
authors.affiliation: Department of Respiratory Medicine, Children's University Hospital, Dublin, Ireland. fraflanagan@gmail.com
authors.forename: Frances
authors.initials: F
authors.lastname: Flanagan
authors.forename: Louisa
authors.initials: L
authors.lastname: Glackin
authors.forename: Dubhfeasa M
authors.initials: DM
authors.lastname: Slattery
mesh_terms: D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000627:therapeutic use; D006486:Hemosiderosis / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D007262:Infusions, Intravenous; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D000097545:Hemosiderosis, Pulmonary
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide