A case of HELLP syndrome at 34w-pregnancy with systemic lupus erythymatosus and antiphospholipid antibody syndrome; Importance of measurement of VW factor

Abstract

Summary: A 39-year-old woman was diagnosed with Systemic lupus erthymatosus (SLE) in 1993, and initially received 30 mg of prednisolone (PSL) daily as treatment. In 2012, the patient was diagnosed with pregnancy-induced hypertension (PIH) complecated with proteinurea, hypertension and pretibial edema at 24 weeks of gestation. At onset, protein urea was 1.6 g/day and she was given bed rest in the hospital with a protein-restricted and low salt diet, which led to a decrease in protein urea to approximately 1 g/day. At 34 weeks of gestation epigastric pain developed, and laboratory examinations showed liver dysfunction and low platelets. We made a diagnosis of hemolysis, elevated liver enzymes, low platelet (HELLP) syndrome and performed an emergency cesarean procedure. Thereafter blood pressure was elevated, protein urea was 3.2 g/daily, anti-ds-DNA antibody level was elevated and serum C3/C4/CH50 was reduced, thus we gave. plasma exchange therapy, along with immunoadsorption and steroid pulse therapy (methyl-prednisolone 500 mg/daily for 3 days), as well as PSL at 30 mg/day. Overtime clinical symptoms and laboratory data gradually improved. Conclusion: Some reports suggest that SLE during pregnancy is a risk factor for hypertension, nephritis, SLE relapse and HELLP syndrome. In the patient, ADAMTS13 activity did not decrease, while there was an increase in VW factor level. We assessed this case was as atypical thrombotic microangiopathy. And herein report HELLP syndrome during pregnancy associated with SLE in our patient.