Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil

Abstract

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.

Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report

“…Because there are very few published studies on DPL, data on its clinical course and prognosis are available only from previous case reports. Multiple medications for DPL have been tried, including bevacizumab, propranolol, sirolimus, interferon-alfa 2b, glucocorticoids, bisphosphates, thalidomide [5,10], and sildenafil [15]. Other forms of treatment, such as surgery, sclerotherapy, radiotherapy, and lung transplantation have also been reported [12,[16][17][18].…”