Central cyanosis due to severe pulmonary hypertension combined with pericarditis as the initial manifestation of systemic lupus erythematosus.

ABSTRACT

Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. The initial manifestation complicating any organ system either singly or in combination is protean. Herein we report a 26-year-old female patient with fresh SLE whose initial manifestation was central cyanosis caused by severe pulmonary hypertension and acute pericarditis. The symptoms were relieved dramatically after treatment with steroid and bosentan. Accurate and timely diagnosis in SLE-associated pulmonary hypertension may be life saving.