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Case Records of the Massachusetts General Hospital
Weekly Clinicopathological Exercises
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Volume 333:369-377 August 10, 1995 Number 6
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Case 24-1995— A 46-year-old woman with dermatomyositis, increasing pulmonary insufficiency, and terminal right ventricular failure
D.S. Lazarus, and E.J. Mark

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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 muscle–biopsy specimen disclosed . . . [Full Text of this Article]

Differential Diagnosis

Clinical Diagnosis

Dr. David S. Lazarus's Diagnoses

Pathological Discussion

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