Hughes Stovin: Sustained remission and regression of pulmonary aneurysms with anti–tumor necrosis factor treatment. Issue 6 (2nd April 2019)

• Record Type: Journal Article
• Authors: Ghirardo, Sergio; Pastore, Serena; Gortani, Giulia; Tommasini, Alberto; Taddio, Andrea
• Is Part Of: Pediatric pulmonology. Volume 54:Issue 6(2019)
• Page Start: E13
• Page End: E15
• Publication Date: 2019-04-02
• Subjects: Hughes Stovin -- pulmonary aneurisms -- antiTNF‐α -- treat ment; Pediatric respiratory diseases -- Periodicals; Pediatrics -- Periodicals; 618.922
• DOI: 10.1002/ppul.24298
• Languages: English
• ISSNs: 8755-6863

Abstract

Abstract: We report the first case of Hughes Stovin Syndrome successfully treated with long‐lasting tumor necrosis factor α (TNF‐α) blocker (infliximab) treatment. Because of the failure of the standard therapeutic regimen with steroids and cyclophosphamide, infliximab was started achieving a stable disease remission and a complete resolution of pulmonary aneurysms. Hughes Stovin Syndrome, although rare, is a life‐threatening condition that needs to be timely identified and treated aggressively. Our report underlines the importance of TNF‐α blocker treatment in Hughes Stovin Syndrome, suggesting its use as long‐term safe and useful.