Pulmonary Scleroderma

¹ Mayo Graduate School of Medicine (University of Minnesota), Rochester: Resident in Medicine
² Mayo Clinic and Mayo Foundation (Rochester, Minnesota): Section of Medicine
³ Mayo Clinic and Mayo Foundation (Rochester, Minnesota): Experimental and Anatomic Pathology

Clinical records, autopsy protocols, and pathologic specimens in 28 cases of scleroderma were reviewed from the standpoint of the pulmonary features. Dyspnea on exertion was the most common respiratory complaint. Pulmonary scleroderma usually was a late feature of widespread systemic disease, but was infrequently the primary cause of death which was usually due to cardiac or renal failure. Physical findings in pulmonary scleroderma were few, but bilateral rales at the base of the lungs were common. The most frequent roentgenographic finding was diffuse linear and nodular fibrosis in the lower two thirds of the lung fields. Extensive pathologic changes were present in the lungs of patients with or without clinical or roentgenographic abnormalities. In all 28 cases there were morphologic pulmonary abnormalities which were frequently nonspecific. Progressive nonspecific interstitial fibrosis with bronchiolectasis and cyst formation was the outstanding pathologic feature and intimal proliferation occurred in small pulmonary arteries and arterioles.