Abstract
Although the causal pathomechanisms contributing to remodelling of the pulmonary vascular bed in pulmonary arterial hypertension (PAH) are still unclear, several analogous features with carcinogenesis have led to the emergence of the cancer-like concept. The major similarities concern the altered crosstalk between cells from different tissue types, unexplained proliferation and survival of pulmonary smooth muscle and endothelial cells, the metabolic (glycolytic) shifts, and the association with the immune system. However, major differences between PAH and cancer exist, including the absence of invasion and metastasis, as well as the pathogenic genes involved and the degrees of angiogenesis impairment and genetic instability. It is clear that PAH is not a cancer, but this cancer-like concept has opened a new field of investigation and raises the possibility that antiproliferative and/or oncological drugs may exert therapeutic effects not only in cancer, but also in PAH. Such analogies and differences are discussed here.
Footnotes
Support Statement: This work was funded, in part, by grants from the French National Institute for Health and Medical Research (INSERM), the Legs Poix (Chancellerie des Universités de Paris, Paris, France), and the French National Agency for Research (grant no. ANR_12_JSV1_0004_01). A. Huertas is supported by the 2010 Josso Award from the French Medical Research Foundation (Paris, France). A. Seferian is supported by the French PAH patient association HTAP France (Asnières Lès Dijon, France). C. Sattler is supported by the Fonds de Dotation "Recherche en Santé Respiratoire" (Paris, France).
Conflict of interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com
Provenance: Submitted article, peer reviewed.
- Received September 28, 2013.
- Accepted October 14, 2013.
- ©ERS 2013
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