Peripheral Phenomena in a Woman With Calcinosis, Raynaud Phenomenon, Esophageal Dysmotility, Sclerodactyly, and Telangiectasia (CREST) Syndrome–Associated Pulmonary Hypertension
An 84-year-old woman with a history of CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) variant scleroderma and gastroesophageal reflux disease presented with progressive shortness of breath over 2 months. Her previous surgeries included a bilateral sympathotomy for the Raynaud phenomenon. Physical examination findings included clear lungs, subcutaneous calcinosis over the first 3 fingers and elbows bilaterally, as well as sclerodactyly, Raynaud phenomenon, and diffuse telangiectasias (Figure 1). There was no lower-extremity edema.
Laboratory testing was significant for the presence of the anticentromere antibody. A transthoracic echocardiogram revealed an ejection fraction of 60%, pulmonary artery systolic pressure of 85 mm Hg, right-to-left shunting through a patent foramen ovale, and severe tricuspid valve regurgitation. A right heart catheterization confirmed pulmonary hypertension (Figure 2) and a decrease in her pulmonary systolic pressure from 89/22 mm Hg (49 mm Hg) to 73/14 mm Hg (41 mm Hg) after the administration of inhaled nitric oxide. Afterward, treatment with sildenafil 20 mg daily was started, and 1 month later she reported a mild improvement in her dyspnea and inhaled iloprost was added to her regimen.
- © 2012 American Heart Association, Inc.