Critical Care: Student/Resident Case Report Poster - Critical Care I |

Catastrophic Antiphospholipid Antibody Syndrome Presenting as Intraabdominal Abscess: An Unusual Case of Thrombotic Storm. FREE TO VIEW

Katelyn Donohue, MD; Samuel Minkove, MD; Bethany Weiler, MD; Susan Shyu, MD
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University of Maryland Medical Center, Baltimore, MD

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):377A. doi:10.1016/j.chest.2016.08.390
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare variant of antiphospholipid antibody syndrome (APL), occurring in less than 1% of APL cases, with an often perilous outcome. Triggered by trauma or infection, APL is a disease of microvascular thrombosis. Microvascular damage often occurs in the lungs, kidneys or skin. Rarely, patients with CAPS suffer thrombotic storm with large-vessel thrombosis of both arteries and veins in addition to microthrombi. Up to 25% of CAPS patients report gastrointestinal problems later in the disease course, but less than 1% of reported cases present with GI symptoms1. Below is an unusual case of a woman presenting with GI abscess from CAPS thrombotic storm.

CASE PRESENTATION: A 45-year-old female with a history of systemic lupus erythematosus and end-stage renal disease presented to an outside hospital with intractable vomiting. Computed tomography of the abdomen showed fluid collection in the left upper quadrant and partial thrombosis of the superior mesenteric vein (SMV). She then developed polymicrobial Gram-negative bacteremia (Proteus vulgaris, Klebsiella pneumonia) as well as thrombocytopenia, lower GI bleeding, and respiratory failure. She was transferred to our facility. On arrival, she was suspected to have CAPS with thrombotic storm, based on the presence of digital ischemia, right radial artery and SMV thrombus. She received intravenous immunoglobulin (IVIg) and steroids but did not tolerate anticoagulation due to GI bleeding. Blood and peritoneal fluid cultures grew Enterococcus faecium. Due to increasing vasopressor requirements and intra-abdominal pressures she was taken emergently for surgical washout to remove abundant intra-abdominal pus. Biopsies of black lesions on the colon revealed thrombosis of the microvasculature and necrosis. Beta-2 glycoprotein IgA was 52 GPI IGA units, and RNP Ab was 1.1 AI. Despite multiple washouts, steroids, IVIg and plasmapheresis, she died on day 21 of hospitalization.

DISCUSSION: This case illustrates an unusual presentation of CAPS as intra-abdominal abscess secondary to SMV occlusion and microthrombosis of the colon. Though less common than microthrombi, large vessel occlusion in the abdomen should prompt suspicion of CAPS. Mortality in these patients is high: 30-50%. Steroids, anticoagulation and IVIg has improved outcomes - however failure to recognize unusual presentations leads to poor outcomes.

CONCLUSIONS: Critical care providers must be aware of CAPS’ variable presenting symptoms. While primary GI manifestations are rare, high clinical suspicion of CAPS allows for timely treatment and improved mortality.

Reference #1: Cervera R, Bucciarelli S, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32:240-5.

Reference #2: Carvera R, Rodriguez-Pinto I, et al. 14th International Congress on Antiphospholipid Antibodies Task Force Report on CAPS. Autoimmunity Rev 2014;13:699-707.

DISCLOSURE: The following authors have nothing to disclose: Katelyn Donohue, Samuel Minkove, Bethany Weiler, Susan Shyu

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