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Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil
  1. Arnaud Maurac1,
  2. Marie-Pierre Debray2,
  3. Bruno Crestani3,4 and
  4. Camille Taillé3,4
  1. 1 Centre Hospitalier Universitaire de Nancy, Département de pneumologie, CHRU de Nancy, Vandoeuvre-lès-Nancy, France
  2. 2 Service de Radiologie, Hôpital Bichat, Assistance Publique - Hôpitaux de Paris, Paris, France
  3. 3 Service de Pneumologie et Centre de Référence Constitutif des Maladies Pulmonaires Rares, Hôpital Bichat, Assistance Publique - Hôpitaux de Paris, Paris, France
  4. 4 Département Hospitalo-Universitaire FIRE; INSERM UMR 1152; LabEx Inflamex, Université Paris Diderot, UFR de Médecine Site Xavier-Bichat, Paris, France
  1. Correspondence to Professor Camille Taillé, camille.taille{at}


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.

  • interstitial lung disease
  • drugs: respiratory system

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  • Contributors AM and CT: concept, design, supervision, processing, writing manuscript and critical analysis. BC: critical analysis. M-PD: CT scan analysis and approved the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.