P1015 - Catastrophic Antiphospholipid Syndrome Presenting as Hepatic Injury, Pancreatitis, and Rash

Vyshnavi Kodali, DO, Konstantinos Damiris, DO, Benyam Addissie, MD
Geisinger Medical Center, Danville, PA

Introduction: Catastrophic Antiphospholipid Syndrome (CAPS) is a rare and life-threatening emergency that occurs in up to 1% of patients with Antiphospholipid Syndrome (APS). CAPS may present in the setting of other prothrombotic factors such as infection and malignancy. Early recognition of CAPS is vital and should be considered when patients with known APS present with multisystem disease.

Case Description/Methods: 71-year-old man with history of APS presented with diffuse rash and jaundice. The maculopapular blanching rash started 5 days before presentation on his extremities and spread centrally (Image A & B). The jaundice started 1-2 days later. ROS was otherwise normal. Labs were notable for direct bilirubin >10, alkaline phosphatase 1,111, LDH 501, haptoglobin < 10, lipase 635, and creatinine 1.5. There was no recent exposure to known hepatotoxic medications. CT imaging showed acute pancreatitis and no hepatobiliary or vascular pathology. MRCP showed thickened CBD with upstream biliary dilation without mass, but subsequent EUS did not demonstrate any biliary pathology to warrant ERCP. Fluid resuscitation for pancreatitis was started. In search of a unifying diagnosis for the multiorgan involvement, multidisciplinary discussion was held which yielded concern for CAPS. Plasma exchange (PLEX) and high dose steroids were emergently initiated. With this, liver chemistry started to improve but did not normalize, so liver biopsy was performed. Histology did not reveal microthrombi but there was patchy necrosis (Image C). Skin biopsy favored drug-induced exanthem. Despite the treatment, his liver labs worsened after a significant initial improvement (Table 1). Unfortunately, he continued to decompensate, developed new pulmonary embolisms despite full dose anticoagulation, and passed away.

Discussion: CAPS is a rare disease characterized by complement mediated immune dysfunction, leading to widespread thrombus formation or signs of ischemia. Gastrointestinal involvement in CAPS is uncommon and was the initial presentation in our patient with liver injury and pancreatitis. Triggers do not have to occur simultaneously and this patient’s malignancy & recent infections (COVID-19, culture negative endocarditis) with known APS led to high suspicion for CAPS as a unifying diagnosis.




Disclosures:


Vyshnavi Kodali, DO, Konstantinos Damiris, DO, Benyam Addissie, MD. P1015 - Catastrophic Antiphospholipid Syndrome Presenting as Hepatic Injury, Pancreatitis, and Rash, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.