PRIMARY PULMONARY ARTERIOLOSCLEROSIS

Abstract

THE PURPOSE of this paper is to describe the disease known as primary pulmonary arteriolosclerosis, to review the important literature, and to report three additional cases. Primary pulmonary arteriolosclerosis may be defined as a rare, progressive, fatal disease of unknown causation characterized by an extensive narrowing of the pulmonary arterioles by an arteriosclerotic process. The disease is uniformly associated with right ventricular enlargement. Recognition is difficult because, by definition, all pathologic processes now known to cause pulmonary arteriolosclerosis must be eliminated by detailed clinical and pathologic examination. Interest in the problem of pulmonary arteriosclerosis was evident as early as 1706 in the writings of Vieussens.¹ However, it was not until the early 19th century that the subject attracted sufficient attention to appear again in the literature in the writings of Kreysig² and Andral.³ This entity actually was not appreciated until the latter part of that century, when