Basic and Clinical Sciences (BCSE) Practice Exam

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What is the histological hallmark of plexiform lesions found in pulmonary arterial hypertension (PAH)?

  1. Abnormal endothelial cells

  2. Intimal hyperplasia

  3. Luminal obliteration

  4. Medial hypertrophy

The correct answer is: Abnormal endothelial cells

The histological hallmark of plexiform lesions found in pulmonary arterial hypertension (PAH) is primarily the abnormal proliferation of endothelial cells, leading to the formation of complex vascular structures that can resemble a network of tangled vessels. These lesions result from a combination of endothelial dysfunction and excessive growth and migration of smooth muscle and endothelial cells in the pulmonary vasculature. In PAH, the formation of plexiform lesions typically signifies advanced disease and is associated with severe vascular remodeling. It is characterized by areas where the normal architecture of the pulmonary artery is disrupted, resulting in a proliferation of atypical endothelial cells, which can lead to further restriction of blood flow through the pulmonary arteries. Other pathological features, such as intimal hyperplasia, luminal obliteration, and medial hypertrophy, are present in PAH but do not exclusively define the plexiform lesions as effectively as the alteration of endothelial cells does. Therefore, the abnormal endothelial cells are recognized as the primary histological feature that distinguishes plexiform lesions in this condition.