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Long term imatinib treatment in pulmonary arterial hypertension
  1. R Souza,
  2. O Sitbon,
  3. F Parent,
  4. G Simonneau,
  5. M Humbert
  1. Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, INSERM U764, IFR13, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Université Paris-Sud, Assistance Publique - Hôpitaux de Paris, Clamart, France
  1. Correspondence to:
    Professor M Humbert
    Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, INSERM U764, IFR 13, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Assistance Publique - Hôpitaux de Paris, Université Paris-Sud 11, 157 rue de la Porte de Trivaux, 92140 Clamart, France; marc.humbert{at}abc.aphp.fr

https://doi.org/10.1136/thx.2006.064097

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    Pulmonary arterial hypertension (PAH) is a life threatening condition characterised by progressive obliteration of the small pulmonary arteries leading to increased pulmonary arterial resistance and right heart failure. Treatment for PAH has developed in the last few years since the description of new pathways related to the disease.1 Recently, short term (6 months) use of imatinib, a platelet derived growth factor (PDGF) receptor antagonist, in combination with maximal PAH treatment (prostacyclin derivative, endothelin receptor antagonist, and type 5 phosphodiesterase inhibitor) has been shown to improve the haemodynamics and functional capacity in a single case of severe PAH.2 We here report the first two cases of the long term (3 years or more) use of imatinib, as monotherapy or in combination with bosentan, a dual endothelin receptor antagonist. …

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    Footnotes

    • Funding: Chancellerie des Universités de Paris (Legs Poix), Université Paris-Sud 11 and European Respiratory Society, Fellowship number 192.