Case 24-1995: A 46-year-old woman with dermatomyositis, increasing pulmonary insufficiency, and terminal right ventricular failure

Presentation of Case A 46-year-old woman was admitted to the hospital for consideration of lung transplantation because of severe pulmonary hypertension. The patient had been well and working as a secretary until 16 months earlier, when proximal-muscle weakness developed, accompanied by myalgia, Raynaud's phenomenon, scaling plaques over the distal interphalangeal joints, and a maculopapular rash over the face, neck, and anterior chest that spared the eyelids and nasolabial folds; she began to have dyspnea. Evaluation elsewhere showed that the creatine kinase level was approximately 3000 U per liter. Electromyographic studies revealed evidence of muscle disease. Microscopical examination of a quadriceps . . .