Challenges and opportunities in treating inflammation associated with pulmonary hypertension.
BACKGROUND
Inflammatory cells are present in the lungs from patients with many, if not all, forms of severe pulmonary hypertension.


METHODS
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.
a School of Pharmacy , Virginia Commonwealth University , Richmond , VA , USA.
Norbert F
NF
Voelkel
b Pulmonary and Critical Care Medicine Division , Stanford University , Palo Alto , CA , USA.
Rasa
R
Tamosiuniene
b Pulmonary and Critical Care Medicine Division , Stanford University , Palo Alto , CA , USA.
Mark R
MR
Nicolls
D000818:Animals; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D065627:Familial Primary Pulmonary Hypertension / Q000628:therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000628:therapy*; D007249:Inflammation / Q000628:therapy*; D008168:Lung / Q000473:pathology
D000893:Anti-Inflammatory Agents
Severe pulmonary hypertension; anti-CD20 antibodies; autoimmunity; cytokines; inflammation; leukotrienes; metabolic syndrome; wound healing