• Media type: E-Article
  • Title: Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil
  • Contributor: Maurac, Arnaud; Debray, Marie-Pierre; Crestani, Bruno; Taillé, Camille
  • imprint: BMJ, 2019
  • Published in: BMJ Case Reports
  • Language: English
  • DOI: 10.1136/bcr-2018-228523
  • ISSN: 1757-790X
  • Keywords: General Medicine
  • Origination:
  • Footnote:
  • Description: <jats:p>General lymphatic anomaly (GLA) is a very rare disorder, characterised by multifocal lymphatic malformations into various tissues that is due to congenital abnormalities of lymphatic development. No treatment has ever proved its efficiency.</jats:p><jats:p>We report a 22-year-old man with recurrent bronchial casts due to thoracic involvement of GLA. After a 6-month treatment with sildenafil (20 mg three times a day), a phosphodiesterase 5 inhibitor, chest CT scan showed a complete regression of ground-glass opacities and lung function test results improved substantially and remained stable for 1 year. The treatment was well tolerated.</jats:p><jats:p>This observation suggests that sildenafil may be a therapeutic approach to be tested in thoracic involvement of GLA.</jats:p>
  • Access State: Open Access