The Pulmonary Manifestations of Generalised Scleroderma

Two aspects of generalised scleroderma have received much attention in recent years. Firstly, the widespread and sclerotic nature of the disease process has been embodied in the term “progressive systemic sclerosis,” proposed by Goetz’ in 1945, and since adopted by other authors. Secondly, connective tissue alterations are now regarded as an integral part of this entity which answers all the criteria of a grave collagen disturbance. The purpose of this paper is to re-examine the known and possible pulmonary manifestations of systemic sclerosis, as the disease will here be termed, with reference to these newer concepts. Pleural and vascular involvement in allied collagen disorders (e.g. disseminated lupus erythematosus) are well established. The possibility of similar lesions in systemic sclerosis is therefore raised. Hypertension in the pulmonary circuit, an entity which has recently received wide attention, is known to result from both pulmonary fibrosis and pulmonary arteritis. Systemic sclerosis is an established cause of the former, and full evidence of pulmonary vascular involvement will be presented. The relationship between systemic sclerosis and pulmonary hypertension will accordingly be examined and amplified. The pulmonary manifestations of systemic sclerosis to be discussed in this paper are: