Increased extracellular matrix deposition, increased pulmonary infiltrates, and an accumulation of inflammatory cells within the PA wall, leading to increased pulmonary vascular resistance, are the hallmarks of pulmonary arterial hypertension (PAH), a relatively uncommon chronic disease of the pulmonary vasculature. These traits may show up as fatigue, chest pain, or syncope as a clinical symptom. Increased pulmonary vascular resistance and elevated pulmonary arterial pressure can develop quickly, leading to right heart failure and death. Idiopathic PAH, heritable PAH (HPAH), drug- and toxin-induced PAH, associated PAH (APAH), and persistent PAH of the newborn (PPHN) are the five categories into which PAH is divided. Though the exact mechanism is unknown, inflammation is thought to play a major role in the development of PAH.
For more information: Macrophages in PAH
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