Pulmonary hypertension in systemic autoimmune disease

Pulmonary hypertension occurs in systemic autoimmune diseases, specifically systemic lupus erythematosus, progressive systemic sclerosis, rheumatoid arthritis, polymyositis-dermatomyositis, and mixed connective tissue disease. This article discusses the histopathologic and clinical manifestations of this syndrome and its semblance to those of so-called ''primary'' pulmonary hypertension. An approach to diagnosis and treatment of pulmonary hypertension in collagen vascular disease is suggested, with specific focus on the use of vasodilator medications and transplantation.