Overt pulmonary hypertension in systemic lupus erythematosus (SLE) is rarely reported. We have documented 4 such patients, all of whom had severe extrapulmonary manifestations of the disease and also Raynaud's phenomenon. The relationship between Raynaud's phenomenon and pulmonary hypertension in SLE is borne out by a review of the literature. A common vasospastic aetiology is suggested.