Pulmonary vasculitis is an uncommon complication of systemic lupus erythematosus (SLE), and has not been previously documented in pregnancy. This case report describes the rapidly fatal course of a pregnant SLE patient, whose terminal illness was manifested by severe pulmonary hypertension and unexpected autopsy findings of medium and large vessel vasculitis. The relationship of these unusual manifestations to other clinical and serologic features of SLE, as well as novel therapeutic options, are discussed.