Challenges and opportunities in treating inflammation associated with pulmonary hypertension

Expert Rev Cardiovasc Ther. 2016 Aug;14(8):939-51. doi: 10.1080/14779072.2016.1180976. Epub 2016 May 4.


Introduction: Inflammatory cells are present in the lungs from patients with many, if not all, forms of severe pulmonary hypertension.

Areas covered: Historically the first inflammatory cell identified in the pulmonary vascular lesions was the mast cell. T and B lymphocytes, as well as macrophages, are present in and around the pulmonary arterioles and many patients have elevated blood levels of interleukin 1 and 6; some patients show elevated levels of leukotriene B4. An overlap between collagen-vascular disease-associated pulmonary arterial hypertension (PAH) and idiopathic PAH exists, yet only a few studies have been designed that evaluate the effect of anti-inflammatory treatments. Here we review the pertinent data that connect PAH and inflammation/autoimmune dysregulation and evaluate experimental models of severe PAH with an emphasis on the Sugen/athymic rat model of severe PAH. Expert commentary: We postulate that there are several inflammatory phenotypes and predict that there will be several anti-inflammatory treatment strategies for severe PAH.

Keywords: Severe pulmonary hypertension; anti-CD20 antibodies; autoimmunity; cytokines; inflammation; leukotrienes; metabolic syndrome; wound healing.

Publication types

  • Review

MeSH terms

  • Animals
  • Anti-Inflammatory Agents / therapeutic use
  • Familial Primary Pulmonary Hypertension / therapy*
  • Humans
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy*
  • Inflammation / therapy*
  • Lung / pathology


  • Anti-Inflammatory Agents