Critical Care Medicine Physician Anne Arundel Medical Center
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Introduction: Catastrophic antiphospholipid syndrome (CAPS) is a rare, fulminant form of antiphospholipid syndrome characterized by rapid multiorgan failure due to widespread thrombosis. Hepatic involvement is uncommon but can present with acute liver failure (ALF), especially when complicated by portal vein thrombosis (PVT). In patients with renal impairment on anticoagulation, drug accumulation may further contribute to hepatic injury.
Description: A 74-year-old man with coronary artery disease, heart failure, diabetes, chronic kidney disease, and home use of dabigatran presented with hypoxia, hypotension, and left leg pain. Labs showed severe transaminitis (AST 2465 U/L, ALT 1655 U/L), acute kidney injury (Cr 3.9), lactic acidosis (lactate 9.2), and coagulopathy (INR 8.9). Workup for infection, viral hepatitis, and autoimmune etiologies was negative. Imaging revealed hepatomegaly, and duplex ultrasound showed occlusion of the right and left portal veins and partial occlusion of the main portal vein. Lower extremity arterial duplex ultrasound showed left infrapopliteal runoff occlusion. Cardiolipin antibodies were pending, but CAPS was suspected due to the presence of systemic thrombosis, liver failure, and multiorgan dysfunction. Despite being on dabigatran, renal dysfunction raised concern for drug accumulation contributing to liver injury. The patient was started on intravenous heparin and supportive care, including vasopressors and continuous renal replacement therapy. Despite initial stabilization, he developed refractory shock, worsening hepatic dysfunction, and multiorgan failure. After multidisciplinary discussion, goals of care were shifted to comfort measures.
Discussion: This case underscores the importance of considering CAPS in patients with unexplained thromboses and multiorgan dysfunction. CAPS diagnosis requires persistent positivity of antiphospholipid antibodies on two or more occasions, at least 12 weeks apart, and a single negative anticardiolipin antibody test does not exclude the diagnosis of CAPS. Early empiric anticoagulation and multidisciplinary input are critical for suspected CAPS, even before confirmatory testing. The conclusion reflects the authors’ interpretation, with assistance from AI tools.
