ROENTGEN FEATURES OF SCLERODERMAL PERICARDITIS WITH EFFUSION.

Abstract

In recent years it has become generally accepted that scleroderma is not limited to the skin, but is a diffuse connective-tissue disease in which generalized mesenchymal fibrosis with proliferative hyaline vascular lesions occurs in practically every body tissue or organ. Although the skin and subcutaneous tissue changes are conspicuous, they are overshadowed by the more serious visceral lesions of the cardiovascular, pulmonary, gastrointestinal, and renal systems. The disease is prone to remissions and relapses, but is eventually fatal with a fulminating or chronic course; hence the terminology, progressive systemic sclerosis. Oram and Stokes (1) point out that in scleroderma the heart is involved much less often than the esophagus and lungs and that the frequency of clinical cardiac involvement in generalized scleroderma is difficult to estimate because reports in the literature vary from none at all to 2 to 3 per cent. In an autopsy series collected from the literature, however, Oram and Stokes found myocardial involvement in 25 of 28 cases and pericardial involvement, with and without effusion, in 22 of 31. Only Meitzer (2) and the authors (3) have published papers describing pericardial effusion in scleroderma. Recently, 2 cases of scleroderma with pericardial effusion and cor pulmonale were reported from this center (3). Since then, 3 additional cases have been studied. Roentgenography, especially angiocardiography, established the diagnosis of cor pulmonale in 2 of these patients. The third died of peritonitis and hemorrhage from perforation of a gastric ulcer while on adrenocorticosteroid therapy, and autopsy revealed isolated sclerodermal pericarditis. Report of Cases Case I. Pericardial Effusion and Cor Pulmonale. A 33-year-old Negro woman was first seen at Memorial Hospital in 1945 because of fibroadenoma of the breast. Surgical removal was followed by radiation therapy. At clinic visits, signs and symptoms of scleroderma gradually developed. These were generalized arthralgias, muscle aches, tightening of the skin over the finger joints of both hands, Raynaud's phenomenon, dysphagia, pulmonary fibrosis, cardiomegaly, and dryness of the mouth and diminution of tearing (Sjögren's syndrome). Biopsies of the skin from the wrist and the lower leg were consistent with scleroderma. The electrocardiogram showed right axis deviation and right ventricular hypertrophy. Because pericardial effusion was a possible explanation of the cardiomegaly (Fig. 1, A), angiocardiographic studies were performed at The New York Hospital on April 28, 1961. The superior vena cava, the right atrium, ventricle, and pulmonary artery were dilated. Branches of the pulmonary artery were diminished in caliber in the middle and outer zones (Fig. 1, B). The pulmonary veins, left atrium, and ventricle were normal in size. Modified circulation time (Decholin) was eighteen seconds, twice normal.