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Review
. 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

Norbert F Voelkel  1 , Rasa Tamosiuniene  2 , Mark R Nicolls  2
Affiliations
Review

Challenges and opportunities in treating inflammation associated with pulmonary hypertension

Norbert F Voelkel et al. Expert Rev Cardiovasc Ther. 2016 Aug.

Abstract

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.

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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.

References

    1. Voelkel NF, Gomez-Arroyo J, Abbate A, Bogaard HJ, Nicolls MR. Pathobiology of pulmonary arterial hypertension and right ventricular failure. Eur Respir J. 2012;40:1555–65. - PMC - PubMed
    1. Stein PD, Matta F, Hughes PG. Scope of problem of pulmonary arterial hypertension. Am J Med. 2015;128:844–51. - PubMed
    1. Haddad F, Spruijt OA, Denault AY, Mercier O, Brunner N, Furman D, Fadel E, Bogaard HJ, Schnittger I, Vrtovec B, Wu JC, de Jesus Perez V, Vonk-Noordegraaf A, Zamanian RT. Right Heart Score for Predicting Outcome in Idiopathic, Familial, or Drug- and Toxin-Associated Pulmonary Arterial Hypertension. JACC Cardiovasc Imaging. 2015;8:627–38. - PMC - PubMed
    1. Latus H, Delhaas T, Schranz D, Apitz C. Treatment of pulmonary arterial hypertension in children. Nat Rev Cardiol. 2015;12:244–54. - PubMed
    1. Heath D, Edwards JE. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958;18:533–47. - PubMed

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