Clinical Reviews in Allergy & Immunology

, Volume 44, Issue 1, pp 31–38 | Cite as

The Role of Inflammation and Autoimmunity in the Pathophysiology of Pulmonary Arterial Hypertension

  • Nada Kherbeck
  • Mathieu C. Tamby
  • Guillaume Bussone
  • Hanadi Dib
  • Frederic Perros
  • Marc Humbert
  • Luc Mouthon


Pulmonary arterial hypertension is characterized by a remodeling of pulmonary arteries with endothelial cell, fibroblast, and vascular smooth muscle cell activation and proliferation. Since pulmonary arterial hypertension occurs frequently in autoimmune conditions such as systemic sclerosis, inflammation and autoimmunity have been suspected to play a critical role in both idiopathic pulmonary arterial hypertension and systemic sclerosis-associated pulmonary arterial hypertension. High levels of pro-inflammatory cytokines such as interleukin-1 and interleukin-6, platelet-derived growth factor, or macrophage inflammatory protein 1 have been found in lung samples of patients with pulmonary arterial hypertension, along with inflammatory cell infiltrates mainly composed of macrophages and dendritic cells, T and B lymphocytes. In addition, circulating autoantibodies are found in the peripheral blood of patients. Thus, autoimmunity and inflammation probably play a role in the development of pulmonary arterial hypertension. In this setting, it would be important to set-up new experimental models of pulmonary arterial hypertension, in order to define novel therapeutics that specifically target immune disturbances in this devastating condition.


Inflammation Autoimmunity Pulmonary arterial hypertension Systemic sclerosis Autoantibodies 



We thank Ms. Monisokha LY ( for the design of the figure.


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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Nada Kherbeck
    • 1
    • 2
    • 3
  • Mathieu C. Tamby
    • 1
    • 2
    • 3
    • 7
  • Guillaume Bussone
    • 1
    • 2
    • 3
    • 4
  • Hanadi Dib
    • 1
    • 2
    • 3
  • Frederic Perros
    • 5
    • 6
  • Marc Humbert
    • 5
    • 6
  • Luc Mouthon
    • 1
    • 2
    • 3
    • 4
  1. 1.Institut CochinINSERM U1016ParisFrance
  2. 2.CNRS UMR 8104ParisFrance
  3. 3.Université Paris DescartesParisFrance
  4. 4.Université Paris Descartes, Faculté de Médecine, pôle de Médecine Interne et centre de référence pour les vascularites nécrosantes et la sclérodermie systémiquehôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP)ParisFrance
  5. 5.Université Paris Sud, Faculté de Médecine, centre national de référence de l’hypertension artérielle pulmonaire sévère, Service de Pneumologie et Réanimation RespiratoireHôpital Antoine-Béclère, AP-HPClamartFrance
  6. 6.INSERM U999, Hypertension Artérielle Pulmonaire, Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie LannelongueLe Plessis-RobinsonFrance
  7. 7.Institut Cochin, Pavillon Gustave RoussyParisFrance

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