sildenafil-citrate and Lymphangioleiomyomatosis

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.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.This observation suggests that sildenafil may be a therapeutic approach to be tested in thoracic involvement of GLA.

Topics: Humans; Lung Diseases; Lymphangiectasis; Lymphangioleiomyomatosis; Lymphatic Abnormalities; Male; Phosphodiesterase 5 Inhibitors; Rare Diseases; Respiratory Function Tests; Sildenafil Citrate; Tomography, X-Ray Computed; Treatment Outcome; Young Adult