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The Esophageal Lesions in Dermatomyositis

1967; Radiological Society of North America; Volume: 89; Issue: 1 Linguagem: Inglês

10.1148/89.1.27

1527-1315

James M. O'Hara, George C. Szemes, Robert M. Lowman,

Skin Diseases and Diabetes

The occurrence of the phenomenon of pseudodiverticulation or sacculation of segments of the gastrointestinal tract found in cases of scleroderma has not been previously reported in dermatomyositis. Few well documented correlations of the radiologic and pathologic lesions of the esophagus in dermatomyositis have appeared in the literature. Feldman and Marshak (5) were able to find only 6 cases with radiologic abnormalities of the esophagus (3, 6, 11), to which they added one case of their own. These authors noted that esophageal changes in these 7 cases were minimal in character, and they stressed the occurrence of small bowel dilatation. Subsequently, additional series of patients with this condition demonstrating abnormal esophageal function have been reported (4, 13). Histologic descriptions of the esophagus in dermatomyositis have rarely appeared in the literature (8, 11, 12, 14, 17), and since the lesions to be described here have not been previously documented and represent major abnormalities, we consider it important to report this case. It provides an unusually good correlation of the physiologic, radiologic, and pathologic changes in the esophagus in dermatomyositis. The case is unique among the 49 examples of this disease encountered at this institution in the last ten years. Clinical History J. B., a 43-year-old white female, was first seen at the Yale-New Haven Medical Center with fulminating symptoms of dermatomyositis. One year previously a clinical diagnosis of lupus erythematosus had been made, and treatment with systemic steroids instituted. This produced little relief of the cutaneous symptoms, and there subsequently developed arthralgia, generalized skeletal muscle pain, weakness, and atrophy. Development of severe dysphagia necessitated the first hospitalization. Subsequent admissions for regulation of steroid dosage and for bacterial pneumonias with abscess formation as a result of aspiration were required. Diabetes developed. Terminally, this became poorly controlled, muscular weakness became very severe, and oral candidiasis developed in association with a persistent Gram-negative pneumonia. The patient died four years after the initial symptoms of her disease. The presence of a neoplastic process was never established, although numerous attempts to discover one were made. The lupus erythematosus preparation, serology, and rheumatoid factors were always negative. SGOT levels reached a high of 150 units early in the course of the disease. Roentgen Studies of the Esophagus Three conventional examinations and cinefluorographic studies of the esophagus were completed at approximately yearly intervals, demonstrating progressive increases in the abnormalities of the esophagus. On March 20, 1963, the examination showed both nasal reflux (Fig. 1, A and B) and aspiration of barium into the tracheobronchial tree. In 1964 (Fig. 2, A) defects in the mid portion and right lateral aspect of the esophagus were seen.