A 45-year-old Caucasian female comes to the office due to progressive dyspnea. She also wears warm gloves indoors because her fingers turn blue when they are cold. She also complains of retrosternal burning and regurgitation, especially when supine. Cardiac examination reveals an accentuated S2 over the upper left sternal border. The abdomen is soft with mild hepatomegaly. Pulmonary function test results are as follows: Which of the following is the most likely cause of this patient's dyspnea?

Question

A 45-year-old Caucasian female comes to the office due to progressive dyspnea.  She also wears warm gloves indoors because her fingers turn blue when they are cold.  She also complains of retrosternal burning and regurgitation, especially when supine.  Cardiac examination reveals an accentuated S2 over the upper left sternal border. The abdomen is soft with mild hepatomegaly.   Pulmonary function test results are as follows:  Which of the following is the most likely cause of this patient's dyspnea?

Quizplus · Accepted Answer

Eductional Objective: Pulmonary arterial hypertension is a common complication of systemic sclerosis, likely resulting from proliferation of T cells with release of cytokines (eg, TGF-beta) and consequent progressive thickening and occlusion of the small and medium-sized pulmonary arteries/arterioles.  Patients typically have progressive dyspnea and a loud pulmonic component of S2 and may develop signs of right-sided heart failure (eg, hepatomegaly, peripheral edema).

A 45-Year-Old Caucasian Female Comes to the Office Due to Progressive