A Difficult and Rare Case of Warfarin Refractory Antiphospholipid Syndrome Presenting With Catastrophic Antiphospholipid Syndrome Complicated by Gastrointestinal Bleeding

Abstract

Antiphospholipid syndrome (APS) is managed with warfarin for secondary prophylaxis in patients who have had a thrombotic event in the past. Warfarin has been deemed superior to novel oral anticoagulants in preventing venous and arterial thrombosis in conjunction with aspirin. The catastrophic variant of APS (CAPS) is very rarely reported, especially in those who have been on a therapeutic dose of warfarin therapy. We present a rare case of CAPS in a patient with a history of APS who had been on a therapeutic dose of warfarin along with aspirin therapy. The patient is a 70-year-old male with APS diagnosed 30 years prior when he presented with a pulmonary embolism; aspirin was added to warfarin two years ago when he had a cerebrovascular accident (CVA). He presented to the hospital with acute onset right-sided weakness and aphasia, left lower extremity pain. He had ischemic CVA, acute deep vein thrombosis (DVT), acute renal failure with a creatinine of 2.8, anemia with hemoglobin of 3.8, gastrointestinal bleed (GIB) on EGD, with INR of 3.48 cardiolipin IgM of >140g/L. He was transfused packed red blood cells, fresh frozen plasma, and provided Vit K. Subsequently, he had a cardiac arrest and was intubated and placed on a mechanical ventilator. Given simultaneous multiorgan involvement, acute arterial and venous thrombosis, the patient was diagnosed with CAPS. The patient was started on high-dose dexamethasone, intravenous immunoglobulin (IVIG), and underwent plasma exchange with significant improvement in symptoms, laboratory parameters; and was extubated with near normalization of his speech and motor deficits. He was discharged on enoxaparin and prednisone with sustained clinical improvement two months following discharge. This patient was on the recommended treatment for APS. However, he had presented with a CAPS. This is the first reported case of warfarin refractory CAPS. This case highlights that there might be a subgroup of the population in whom warfarin is not an effective form of treatment modality for an unknown cause, and in fact, it could potently expose a patient to the adverse events related to warfarin therapy as it did in our patient who had significant GIB. This case also highlights the uncommon scenario of spontaneous CAPS with no inciting event as previously reported in the literature, such as infection, recent surgeries, or trauma.

An Unusual Case of Catastrophic Antiphospholipid Syndrome in an Elderly Man

“…Regarding treating CAPS, the consensus is to employ anticoagulants and corticosteroids in all patients [8]. While there are no randomized-controlled trials (RCTs) on specific types of anticoagulants in CAPS specifically due to low prevalence, there have been several recent studies on APS in general showing that warfarin prevents anticoagulation more efficaciously than direct oral anticoagulants (DOACs) and that DOACs increase the risk of thromboses compared to warfarin [8,9,10]. Hence, the patient was switched from apixaban to warfarin upon the suspected diagnosis of CAPS.…”