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Presentation of Case
Dr. Robert H. Goldstein (Medicine): A 63-year-old woman was admitted to the intensive care unit (ICU) of this hospital because of fever, hypotension, and hypoxemia.
Five months before the current admission, weakness of the proximal muscles of the arms and dyspnea on exertion developed in the patient. One month later, she was admitted to this hospital with worsening symptoms. Follicular erythematous papules were present on the lateral aspect of both hips, and the blood creatine kinase level was 1856 U per liter (reference range, 40 to 150).
A skin biopsy of the lesions on the lateral hips revealed interface dermatitis. A diagnosis of dermatomyositis was made. An interferon-γ release assay for Mycobacterium tuberculosis was negative, and treatment with prednisone and azathioprine was initiated, along with trimethoprim–sulfamethoxazole for prophylaxis against Pneumocystis jirovecii pneumonia. An extensive evaluation for cancer — including mammography, computed tomography (CT) of the abdomen and pelvis, transvaginal ultrasonography, colonoscopy, and a Papanicolaou smear — was unrevealing. After the initiation of prednisone and azathioprine therapy, the blood creatine kinase level decreased to 683 U per liter, and the patient had a temporary mild increase in strength.
Despite the initial improvement of the patient’s symptoms, the proximal muscle weakness gradually worsened and the blood creatine kinase level increased during the next several weeks. Dyspnea on exertion progressed, and when the patient was chewing, she noted fatigue in the muscles of her jaw and neck. Three months before the current admission, the patient was evaluated by her rheumatologist and reported severe dyspnea at rest. She was readmitted to this hospital for further evaluation, and additional imaging studies were obtained.
Dr. Melissa C. Price: CT of the chest was performed during expiration and after the administration of intravenous contrast material. Bilateral ground-glass opacities in a mosaic distribution were present, and there was no consolidation (Figure 1).
Dr. Goldstein: To further assess the worsening dyspnea on exertion and the abnormalities seen on imaging studies of the chest, pulmonary-function testing was performed. The forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and ratio of FEV1 to FVC were normal, as were the total lung capacity, carbon monoxide diffusing capacity, and maximal respiratory muscle strength.
The patient reported difficulty swallowing, and...