Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil

BMJ Case Rep. 2019 Apr 23;12(4):e228523. doi: 10.1136/bcr-2018-228523.

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.

Keywords: drugs: respiratory system; interstitial lung disease.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Lung Diseases / congenital*
  • Lung Diseases / diagnostic imaging
  • Lung Diseases / drug therapy*
  • Lung Diseases / pathology
  • Lymphangiectasis / congenital*
  • Lymphangiectasis / diagnostic imaging
  • Lymphangiectasis / drug therapy
  • Lymphangiectasis / pathology
  • Lymphangioleiomyomatosis / congenital
  • Lymphangioleiomyomatosis / diagnostic imaging
  • Lymphangioleiomyomatosis / drug therapy*
  • Lymphatic Abnormalities / diagnostic imaging
  • Male
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Rare Diseases
  • Respiratory Function Tests / methods
  • Sildenafil Citrate / administration & dosage
  • Sildenafil Citrate / therapeutic use*
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome
  • Young Adult

Substances

  • Phosphodiesterase 5 Inhibitors
  • Sildenafil Citrate

Supplementary concepts

  • Lymphangiectasia, pulmonary, congenital