Pathologic observations in systemic sclerosis (scleroderma): A study of fifty-eight autopsy cases and fifty-eight matched controls

Fifty-eight autopsy cases of systemic sclerosis were compared with fifty-eight matched controls to determine the extent, frequency and type of involvement in various organs. The organs found to be frequently and significantly involved by this disease were the skin, gastrointestinal tract, lungs, kidneys, skeletal muscle and pericardium. Myocardial and pleural involvement was also present, but was less easy to separate from similar lesions seen in matched controls. Specific involvement of the brain, anterior pituitary, cardiac valves, liver, spleen, gallbladder, urinary bladder, pancreas and peritoneum could not be demonstrated. In organs containing smooth muscle, such as the gastrointestinal tract, and in skeletal muscle, atrophy was usually a more prominent finding than was fibrosis. Vascular lesions suggestive of severe hypertension were seen in the kidneys of patients with scleroderma in the absence of hypertension. Moreover, vascular lesions suggestive of pulmonary hypertension were found in the lungs of patients without either clinical or pathologic evidence of pulmonary hypertension. Similar vascular lesions were also noted in other organs.