Corticosteroid responsive pulmonary hypertension in systemic lupus erythematosus

Summary Primary pulmonary hypertension is an irreversible and fatal disorder. Every effort should therefore be made to discover all the other treatable diseases which may be associated with pulmonary hypertension. The association of systemic lupus erythematosus and pulmonary hypertension was rarely reported in the past. We add another case in which pulmonary hypertension was the presenting symptom of systemic lupus erythematosus (SLE). In contrast to the previously reported cases, our patient responded well to corticosteroids. It is assumed that this favorable response was due to the relatively early stage of the disease, when the histopathologic pulmonary changes were still in the reversible inflammatory stage.