Which of the following connective tissue disease is most commonly associated with pulmonary hypertension for this patient?
A 36-year-old woman presents to your office for continuing management of progressive dyspnea on exertion, which she has been experiencing for the past 4 months. A transthoracic echocardiogram demonstrates pulmonary hypertension, and a CT scan of the chest shows pulmonary fibrosis. She tells you she is currently being evaluated by a rheumatologist for arthralgias and myalgias. Her rheumatologist mentioned that her dyspnea could be caused by the same problem that is causing her arthralgias.
A. Dermatomyositis B. CREST syndrome C. Systemic lupus erythematosus D. Rheumatoid arthritis
Crest syndrome-pulmonary hypertension with little or no parenchymal lung disease is a life-threatening manifestation of the connective tissue diseases. patients with scleroderma, particularly those with crest syndrome (calcinosis, raynaud phenomenon, esophageal involvement, sclerodactyly, and telangiectasias) have a high incidence of pulmonary hypertension. the prevalence ranges from 2% to 35% in patients with scleroderma to 50% in those with crest syndrome. the prevalence in patients with other disorders varies from 23% to 53% in those with mixed connective tissue disease to 0.5% to 14% in those with systemic lupus erythematosus; it is less common in those with rheumatoid arthritis, sjogren syndrome, and dermatomyositis. treatment of pulmonary hypertension in patients with connective tissue diseases should be patterned after the protocol for ipah.