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Updated: Mar 22, 2026

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Published on: May 11, 2015
Challenges and opportunities in treating inflammation associated with pulmonary hypertension
Norbert F Voelkel1, Rasa Tamosiuniene2, Mark R Nicolls2
1a School of Pharmacy , Virginia Commonwealth University , Richmond , VA , USA.
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.
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