성인형 스틸병에 동반된 폐동맥 고혈압

Abstract

Pulmonary hypertension is one of the serious complications of autoimmune rheumatic disease, and it is becoming an important cause of morbidity and premature death. Pulmonary involvement occurs occasionally in adult-onset Still's disease (AOSD), but pulmonary hypertension has not been previously reported in Korea. We describe a 33-year-old woman with 5-year history of AOSD who presented with pulmonary hypertension, without evidences of pleural or parenchymal involvement of the lung, pulmonary embolism or any other obvious cause. Here, we report an AOSD patient with pulmonary hypertension with review of the literatures.