Cyclophosphamide Pulse Therapy for the Management of Pulmonary Hypertension Associated with Systemic Lupus Erythematosus / 대한류마티스학회지

ABSTRACT

Pulmonary hypertension is an uncommon but serious complication of systemic lupus erythematosis (SLE).Usually its outcomes are ominous and may progress to heart failure and even sudden death.The pathophysiology is still unknown, but several mechanisms,such as pneumonitis,vasculitis,thromboembolism or thrombosis in situ have been proposed.There is no definitive therapy for this condition.Although supportive measures with vasodilators remain the mainstay of treatment,the responses are generally disappointing. We describe a case of improvement of pulmonary hypertension in SLE treated with cyclophosphamide pulse therapy as documented by hemodynamic data. Immunosuppressive treatment with cyclophosphamide was effective in this condition,suggested an immune mediated pathogenesis.