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

Challenges and opportunities in treating inflammation associated with pulmonary hypertension.

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a School of Pharmacy , Virginia Commonwealth University , Richmond , VA , USA.
b Pulmonary and Critical Care Medicine Division , Stanford University , Palo Alto , CA , USA.



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


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.


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

[Indexed for MEDLINE]
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Conflict of interest statement

Declaration of interest This paper was funded by grants from the National Instituted of Health (grant numbers 5PO1HL014985, R01HLI22887). M Nicolls is the cofounder of Eiccose LLC, a company investigating targeting LTB4 in pulmonary hypertension. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

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