Abstract

Objectives We outline a case of a woman with lupus (SLE) and antiphospholipid syndrome (APLA) at 26 weeks’ gestation who develops a complex multiorgan condition with a broad differential. Methods Case report. Results A 34 year old woman, G1P0, presented at 26 weeks gestation with a 5 day history of epigastric pain and emesis. She has a history of lupus anticoagulant (LAC), anticardiolipin (ACL) and anti-dsDNA (ADD) positive SLE and APLA syndrome diagnosed at age 13 with multiple small cerebral infarcts. She was treated with ASA, warfarin and plaquenil; the latter two stopped by age 25, with no recurrent thrombosis. Her pregnancy was conceived through IVF. She continued on ASA and LWMH for prophylactic anticoagulation. Upon presentation, she was hypertensive (pressures >160/100) with thrombocytopenic at 47 × 109/L, creatinine rise from 40 to 93 μmol/L, high uric acid level at 518 μmol/L, and AST and ALT at 74 and 64 U/L respectively peaking in the 4800 s. HIT and SRA were negative, blood films showed schistocytes, positive DAT and C3/C4 levels were low at 0.7 and 0.06 g/L respectively. The patient was diagnosed with preeclampsia and HELLP syndrome. Immediate cesarean section was performed without delivery or hemostatic complications. Placental histology was consistent with preeclampsia. Postpartum, she had persistent severe hypertension with CT confirmed multiorgan infarcts. Anemia and thrombocytopenia also persisted (platelet nadir 4 × 109/L), complement levels remained low and creatinine peak at 485 μmol/L. ADAMTS13 level was 43%. The differential diagnosis included TTP, atypical HUS or SLE progression. High dose steroids, PLEX, dialysis and eculizumab were utilized with minimal response. A trial of vincristine and rituximab was initiated. Conclusions SLE and APLA predispose women to increased pregnancy related complications, including preeclampsia, however the pathogenesis is not well understood. This complex case highlights numerous challenges and addresses the utility of new targeted therapeutic interventions. Disclosures A.E. Lin: None. N. Shehata: None. H. Reich: None. D. Barth: None. C. Laskin: None. S. Lapinsky: None. S. Gandhi: None.

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