Splenic infarct and SMA thrombosis as a manifestation of Seronegative Antiphospholipid Syndrome

Document Type

Conference Proceeding

Publication Date

10-1-2024

Publication Title

Am J Gastroenterol

Abstract

Introduction: Antiphospholipid Syndrome (APS) presents with a wide spectrum of clinical manifestations, ranging from asymptomatic cases to life-threatening catastrophic APS. Thrombosis in large or microvessels can affect virtually any organ system. Diagnostic criteria for APS have evolved, typically requiring the presence of 1 clinical criterion and 1 laboratory criterion. However, emerging research highlights cases where patients exhibit clinical features of APS despite transiently positive or persistently negative antiphospholipid antibody (aPL) titers, a condition termed seronegative APS (SN-APS). This diagnosis, often 1 of exclusion, is considered only after ruling out other inherited and acquired thrombophilic conditions Case Description/Methods: A 45-year-old man with a history of recurrent pulmonary emboli, pancreatitis with pseudocyst, and retinal artery occlusion presented with generalized abdominal pain. Laboratory findings revealed elevated white blood cell count of 25k and lipase 60, with other parameters within normal limits. Computed tomography abdomen showed a splenic abscess, and computed tomography angiography identified thrombosis in the splenic artery and superior mesenteric artery (SMA). Seronegative anti-phospholipid syndrome (SN-APS) was suspected due to recurrent thrombotic events despite initial negative aPL tests. He underwent abscess drainage and received empirical treatment with ceftriaxone and metronidazole. Management involved transitioning from direct oral anticoagulants to Vitamin K Antagonists for SN-APS, highlighting the importance of personalized therapy and ongoing research for optimal patient care. Discussion: The initial clinical diagnosis in this patient was based on a history of multiple thrombotic events, starting with pulmonary emboli, followed by ophthalmological complications, and subsequently a splenic abscess and infarct. Differential diagnoses included thrombophilia, malignancy, and disseminated intravascular coagulation. During hospitalization, aPL testing was initially negative, possibly due to consumption. Over a 6-month follow-up post-discharge, laboratory results remained negative for autoimmune diseases, thrombophilia, and infectious diseases. This case highlights the complexity of diagnosing SN-APS, characterized by recurrent thrombosis including involvement of the splenic and superior mesenteric arteries, and challenges in treatment with direct oral anticoagulants, emphasizing the importance of personalized therapeutic strategies and ongoing clinical monitoring (see Figure 1).

Volume

119

Issue

10

First Page

S3004

Last Page

S3005

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