Sickle cell disease

Question 1

chronic dyspnea in SCD causes?

Answer 1

asthma, pulmonary hypertension, pulmonary fibrosis.

Question 2

asthma

Answer 2

sx: intermittent/chronic wheezing, may be worse at night or with exercise or upper respiratory infection.
dx: pulmonary function testing shows reversible airway obstruction.

Question 3

pulmonary hypertension

Answer 3

sx: exertional dyspnea, signs of right heart failure(ex: jugular venous distention, hepatomegaly, edema).
dx: tricuspid regurgitation on echo, increased pulmonary arterial pressure on right sided heart catheterization.

Question 4

pulmonary fibrosis

Answer 4

sx: exertional dyspnea, progressive.
dx: honeycomb pattern on chest CT scan, pulmonary function testing showing restrictive pattern.

Question 5

pulmonary hypertension pathogenesis in SCD is multifactorial?

Answer 5

intravascular hemolysis, leading to chronic inflammation and endothelial dysfunction, causes hyperplasia and hypertrophy of small pulmonary vessels (vascular remodeling) and increased pulmonary vascular resistance (PVR).
increased PVR leads to decreased right ventricular output and decreased left ventricular filling. cardiac output can’t rise during exercise enough hence exertional dyspnea and fatigue. elevated right-sided heart pressures can increase the intensity of the pulmonic component of the second heart sound (loud S2).