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Catastrophic Antiphospholipid Syndrome Presenting in the Puerperium Period
Abstract Number: SU-03
Abstract Type: Case Report/Case Series
Introduction: Catastrophic Antiphospholipid Syndrome (CAPS) was first defined in 1992 by Asherson and is characterized by small vessel occlusive disease with high associated mortality around 50% . Diagnosis is difficult, especially during pregnancy, as it can mimic or coexist with other more common microangiopathic syndromes . Although rare, CAPS should be considered in pregnant women with microangiopathy and a history of antiphospholipid syndrome (APS).
Case: A 26-year-old G2P0010 at 25 weeks presented with acute onset RUQ and epigastric pain. PMH was significant for APS diagnosed in the setting of recurrent venous thrombosis, later serologically confirmed. The patient was maintained on warfarin anticoagulation and transitioned to LMWH prior to becoming pregnant. Initial laboratory tests were unremarkable and RUQ ultrasound was negative for gallbladder pathology. On hospital day 2, she had a witnessed tonic-clonic seizure. A brain MRV showed posterior reversible encephalopathy syndrome (PRES) with no evidence of hemorrhage, infarct, or thrombosis. Labs revealed thrombocytopenia to 22,000 and AST and ALT elevations greater than 1000. Initial data was concerning for eclampsia/HELLP syndrome, and the patient underwent a C-section under general anesthesia. Postpartum, the platelet count recovered to 102,000 and liver enzymes began to normalize. The patient’s abdominal pain resolved. On POD3 the patient complained of new back pain; she was treated with analgesics and discharged home on POD4. She was readmitted within 24 hours for severe back pain, fever, and tachypnea. On POD5, lab testing revealed thrombocytopenia again (42,000), and hematology was consulted. A V/Q scan showed chronic pulmonary emboli, and MRI of the abdomen revealed small wedge shaped infarcts of the liver. No infectious etiology was found, and given her history of APL, the diagnosis of CAPS was suspected. The patient was started on high-dose steroids and plasma exchange on POD9. She had marked improvement in clinical symptoms and her platelets recovered to normal range after 6 rounds of plasmapheresis.
Discussion: Venous thromboembolism risk increases significantly during pregnancy with up to a 20-fold increased risk reported in the puerperium period, likely even higher in patients with APS. Unlike APS, however, CAPS predominantly affects small vessels, resulting in multiple-organ failure, which leads to a systemic inflammatory response and organ dysfunction . The most common organ systems affected are renal, pulmonary, dermatologic, and cerebral. In the majority of the cases the trigger is unknown, but can be from infection or trauma. Here we describe a complicated case of eclampsia, HELLP, and CAPS and investigate the challenge of diagnosing a rare microangiopathic disease in the setting of a complex patient.
 Asherson, R. J Rheumatol 1992;19:508–12.
 Makatsariya, A et al. Autoimmun Rev 2006;6:89–93
 Cervera, R et al. J Autoimmun 2009;32:240-5