A 45-year-old African-American woman presented to the pulmonary medicine clinic for evaluation of progressive dyspnea and pulmonary hypertension. She had a 3-month history of fatigue, progressive dyspnea on exertion, and orthopnea associated with a feeling of fullness in the neck, chest pressure, palpitations, and an infrequent dry cough. She had a medical history of systemic lupus erythematosus, superior vena cava obstruction due to antiphospholipid antibody syndrome (requiring warfarin therapy for the last 4 years), rheumatoid arthritis, asthma, complete heart block status-post pacemaker placement, and atrial fibrillation. The symptoms gradually started to interfere with her daily activities. Three weeks before her pulmonary appointment, she was evaluated by her primary care provider who prescribed an inhaled corticosteroid in addition to her usual β-agonist with no relief. The patient denied tobacco, alcohol, or other recreational drug use.