Managing pregnancy-associated clinical emergencies in systemic lupus erythematosus: a case-based approach

ABSTRACT

Introduction: Systemic lupus erythematosus (SLE)-related thrombocytopenia during pregnancy and the postpartum period have been associated with adverse pregnancy outcomes and perinatal complications. In this case report, we present two SLE patients with thrombocytopenia emergencies secondary to HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome and thrombotic thrombocytopenic purpura (TTP).

Areas covered: The first case involved a 26-year-old woman, G1P0 at 26 weeks gestation (GA), with high-titer antiphospholipid antibodies (aPL) (positive lupus anticoagulant, anti-beta 2 glycoprotein-1 (aβ2GP1), anti-cardiolipin) and non-criteria aPL to phosphatidylserine/prothrombin complex and anti-domain 1 β2GP1. This case highlights the risks associated with aPL in pregnancy, considers management issues relating to anticoagulation during pregnancy and highlights the importance of maintaining a high index of suspicion for diagnosis of HELLP in SLE patients. The second case was a 36-year-old female, G3P2 at 32 weeks GA, with class III lupus nephritis (LN) who developed severe pre-eclampsia, which included mild thrombocytopenia. This case illustrates the challenges in identifying and differentiating between three pregnancy emergencies that can be seen in SLE patients (pre-eclampsia, LN, and TTP) and presents the management of TTP in peripartum SLE.

Expert opinion: These two cases remind us of the importance of timely diagnosis and management of thrombocytopenia in this population.

KEYWORDS:

• SLE
• pregnancy
• antiphospholipid syndrome
• thrombotic thrombocytopenic purpura

Article Highlights

• Patients with SLE are at higher maternal and fetal morbidity compared to a non-SLE population.

• Pre-pregnancy counselling of patients with SLE is important and should involve an expert multi-disciplinary team, with close surveillance during pregnancy.

• It can be challenging to differentiate between different etiologies of thrombocytopenia or thrombotic microangiopathy in pregnant patients with SLE. Therefore, timely and broad diagnostic investigations including biomarkers of disease activity, anti-phospholipid antibodies, and ADAMTS-13 testing are critical.

• The treatment for antiphospholipid syndrome during pregnancy varies between different guidelines and specialties, highlighting the need for more research to determine the optimal management approach.

• TTP remains a rare condition with high mortality and morbidity despite plasmapheresis, especially in SLE pregnancy; therefore, early recognition and treatment are critical.

Declaration of interest

L Skeith has received honoraria from LEO Pharma and grant funding from CSL Behring. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.