Abstract

Pulmonary hypertension is known to develop in patients with connective tissue diseases. We report a case with systemic lupus erythematosus, in which the natural course of pulmonary hypertension due to systemic lupus erythematosus was observed. This female patient was diagnosed with systemic lupus erythematosus at 24 years of age, but had refused to take steroids or immunosuppressants. A diagnosis of pulmonary hypertension due to systemic lupus erythematosus was made at 42 years old; the pulmonary artery increased in size over a decade. At 59 years old, the patient was transferred to the emergency department of our hospital in a state of shock, at which time chest computed tomography showed a pulmonary artery aneurysm of 82 mm. She died hours after admission, with no response to cardiopulmonary resuscitation. Autopsy showed an enlarged right ventricle and pulmonary alveolar hemorrhage with the formation of a plexiform lesion, accompanied by thrombi, in the branches of the pulmonary artery.