Systemic Lupus Erythematosus Complicated by HELLP Syndrome

Abstract

We describe a pregnant woman with systemic lupus erythematosus, complicated by severe pre-eclampsia (the HELLP syndrome) and adult respiratory distress syndrome, who died in the intensive care unit. A 33-year-old multigravid woman was referred to our university hospital at 17 weeks' gestation because of an exacerbation of systemic lupus erythematosus with elevated liver enzymes and thrombocytopenia. At the time of admission, on physical examination she had revealed a butterfly rash over the cheeks, purpura, acute synovitis and oedema of the legs. Her blood pressure was 180/100 mmHg. The initial laboratory tests and immunological evaluation confirmed active systemic lupus erythematosus complicated by severe pre-eclampsia. One week after admission, she became suddenly confused and had a convulsion with Glascow Coma Score 10 (3+3+4). Therapeutic abortion was induced in the Obstetric Department. She was transferred to the intensive care unit with a diagnosis of respiratory failure, probably due to acute respiratory distress syndrome, and was intubated and ventilated. Dialysis was instituted on two consecutive days from the eighth day. In total she received over 20 units of red blood cells and large quantities of fresh frozen plasma and platelets. On the 24th day her Glascow Coma Score was 2 (1+1+E) and severe hypotension developed. She died from worsening acute respiratory distress syndrome on the 25th day. Women with systemic lupus erythematosus should be advised to become pregnant when the disease is inactive and should be observed at an appropriate centre using a multidisciplinary approach. Therapeutic abortion is an acceptable option if active nephropathy and severe pre-eclampsia are present in early pregnancy.