Basic and Clinical Sciences (BCSE) Practice Exam

What type of diseases are associated with group I pulmonary hypertension?

1. Obstructive lung diseases

2. Connective tissue diseases

3. Restrictive lung diseases

4. Infectious diseases

The correct answer is: Connective tissue diseases

Group I pulmonary hypertension primarily refers to pulmonary arterial hypertension (PAH), which is a specific form of pulmonary hypertension characterized by increased pressure in the pulmonary arteries due to various causes. One of the most significant associations with group I pulmonary hypertension is connective tissue diseases, such as systemic sclerosis (scleroderma), rheumatoid arthritis, and lupus. These diseases can lead to inflammation and vascular remodeling, which contributes to the development of pulmonary arterial hypertension. Connective tissue diseases often involve systemic inflammation and affect the small blood vessels, leading to changes that cause increased vascular resistance in the pulmonary circulation. The pathophysiological mechanisms include endothelial dysfunction, vascular smooth muscle cell proliferation, and thrombosis within the pulmonary arteries, all of which are critical in the onset of PAH. In contrast, other types of diseases listed in the options typically do not cause the specific vascular changes associated with group I pulmonary hypertension. For example, obstructive lung diseases such as chronic obstructive pulmonary disease (COPD) primarily involve airways and lung parenchyma rather than the pulmonary vasculature. Restrictive lung diseases may affect lung volume but do not typically lead to the distinct vascular changes seen in pulmonary arterial hypertension. Infectious diseases can lead to respiratory issues, but they are not