The Pulmonary Manifestations of Generalised Scleroderma (Progressive Systemic Sclerosis)

¹ The University of Cape Town and the Groote Schuur Hospital.

Attention is focused on the various pulmonary manifestations of generalised scleroderma (progressive systemic sclerosis). These were investigated in a series of 12 patients suffering from this condition.

Pleural involvement is uncommon in systemic sclerosis, in contrast to its frequency in other collagen diseases.

Pulmonary fibrosis due to systemic sclerosis, although a specific lesion, has no pathognomonic radiological appearance. Three illustrative cases are presented, in one of which sarcoidosis was closely simulated. The true cause of the radiological alterations may be established by giving attention to other stigmata of systemic sclerosis. The value of examination of the fingers for Raynaud's phenomenon, calcinosis, ulceration and sclerodactyly is stressed.

Secondary broncho-pulmonary infection, a frequent complication of systemic sclerosis, may be favoured by several different factors which are listed.

Pulmonary hypertension may be caused either by pulmosclerosis or sclerodermatous endarteritis. Cases are presented to illustrate these two mechanisms.