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Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide

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Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide

Auteurs : Frances Flanagan [Irlande (pays)] ; Louisa Glackin [Irlande (pays)] ; Dubhfeasa M. Slattery [Irlande (pays)]

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RBID : ISTEX:42BD8BE56D88E616415C3A12F3B018304FC5E1DB

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. Pediatr Pulmonol. 2013; 48:303–305. © 2012 Wiley Periodicals, Inc.

Url:
DOI: 10.1002/ppul.22583

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ISTEX:42BD8BE56D88E616415C3A12F3B018304FC5E1DB

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<div type="abstract" xml:lang="en">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.</div>
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