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Catastrophic Antiphospholipid Syndrome in a Patient With Upper Gastrointestinal Bleed, Portal Hypertension, Deep Venous Thrombosis, and Cardiogenic Shock FREE TO VIEW

Mubdiul Ali Imtiaz, MD; Peter Ricketti, DO; Dina Khateeb, DO; Eugenio Capitle, MD; Andrew Berman, MD
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Internal Medicine, Rutgers University - New Jersey Medical School, Newark, NJ

Chest. 2014;146(4_MeetingAbstracts):304A. doi:10.1378/chest.1992223
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Catastrophic antiphospholipid syndrome (CAPS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis associated with the presence of antiphospholipid antibodies (aPL), resulting in multi-organ failure. Splenic infarction is uncommon. We report an unusual case of CAPS involving a patient who presented with hematemesis secondary to portal hypertension from splenic vein thrombosis.

CASE PRESENTATION: A 70-year-old man with a history of diabetes, systemic lupus erythematosus (SLE) with known aPL, and chronic kidney disease, presented with multiple episodes of hematemesis. Physical examination was notable for a distended abdomen with a fluid wave and bilateral lower extremity (LE) edema. Esophagogastroduodenoscopy revealed gastric varices, and abdominal imaging revealed splenic vein thrombosis causing portal hypertension. A LE Doppler ultrasound revealed deep vein thrombosis (DVT) in bilateral femoral and popliteal veins. Empiric antibiotics were initiated. The patient underwent angioplasty and stent placement to the splenic vein, and an inferior vena cava filter was placed due to active bleeding and an elevated international normalized ratio (INR). On hospital day 4, new EKG changes, a troponin of 2.60 ng/mL, and new wall motion abnormalities on echocardiogram were noted, with an ejection fraction (EF) of 25% (EF was 55% one week earlier). CAPS was diagnosed with clinical evidence of vessel occlusions affecting the spleen, liver, heart, and bilateral LE. He was subsequently managed with high-dose glucocorticoids, plasma exchange, intravenous immune globulin (IVIG), intravenous heparin and dobutamine drip. The patient was discharged in stable condition on prednisone and hydroxychloroquine. Warfarin was held due to persistently elevated INR.

DISCUSSION: CAPS is an uncommon condition with a poor prognosis if untreated. Clinical manifestations of CAPS are secondary to thrombosis in various organs. Simultaneous presentation of thrombotic and hemorrhagic events is rare and complicates clinical decision making. Treatment of CAPS is not standardized, though the combination of anticoagulation and corticosteroids plus plasma exchange with or without IVIG has the highest survival rate. The successful outcome of our case illustrates the importance of considering CAPS in patients who present with multiple thrombotic events so that appropriate and potentially life-saving treatment is initiated.

CONCLUSIONS: A high degree of clinical suspicion is required to diagnose CAPS. Though uncommon, CAPS should be considered in patients who present with multiple thrombotic events over a short time period.

Reference #1: Asherson RA, Espinosa G, Cervera R, Font J, Reverter JC. Catastrophic antiphospholipid syndrome: proposed guidelines for diagnosis and treatment. J Clin Rheumatol. 2002 Jun;8(3):157-65.

DISCLOSURE: The following authors have nothing to disclose: Mubdiul Ali Imtiaz, Peter Ricketti, Dina Khateeb, Eugenio Capitle, Andrew Berman

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