S3815 Macrophage Activation Syndrome Complicated By Fatal Gastrointestinal Bleed

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Introduction: Macrophage Activation Syndrome (MAS) is a rare, potentially fatal complication of systemic inflammatory disorders that belongs to a group of disorders called Hemophagocytic Lymphohistiocytosis (HLH). It is characterized by inappropriate activation of T lymphocytes and macrophages resulting in fevers, hepatosplenomegaly, pancytopenia, hypertriglyceridemia, and hyperferritinemia. We discuss a case of a young female with recently diagnosed Adult-Onset Still’s Disease (AoSD) who presented with MAS complicated by a fatal gastrointestinal bleed. Case Description/Methods: A 27 year old female with recently diagnosed AoSD presented to the emergency department (ED) with worsening rash, joint pain, and fatigue. Initial labs were significant for AST 1203 U/L, ALT 590 U/L, ALK 914 U/L, Total Bilirubin 4.9 mg/dL, Ferritin >16,500 ng/mL, LDH >4,200 u/L, Triglycerides 512 mg/dL, and Fibrinogen 155 mg/dL. She tested positive for rhinovirus on a respiratory pathogen PCR panel. Imaging was notable for hepatomegaly on Computed tomography (CT) Abdomen and prominent bilateral axillary and mediastinal lymphadenopathy on CT Chest. She was started on Intravenous (IV) methylprednisolone followed by Anakinra per rheumatology’s recommendations given concern for MAS. She became acutely thrombocytopenic and developed hematemesis and hematochezia. She required transfer to the Intensive care unit for vasopressors and emergency transfusion protocol due to hemorrhagic shock. Esophagogastroduodenoscopy showed an actively bleeding erosion with large adherent clot limiting visualization in the gastric antrum unable to be cleared. She subsequently went into Pulseless electrical activity arrest, unable to achieve Return of spontaneous circulation. Autopsy was performed with the probable cause of death hemorrhagic shock secondary to gastric ulceration. Discussion: Macrophage activation syndrome (MAS) is a life-threatening complication of autoimmune diseases that is often complicated by pancytopenia and coagulopathy associated with inappropriate activation of macrophages and cytokine deregulation. Precipitating factors often include certain medications and infectious agents. Gastrointestinal complications include uncontrollable bleeding thought secondary to transmural lymphohistiocytic infiltration of macrophages which may also lead to perforation. There are few cases of gastrointestinal bleeding in association with MAS and Hemophagocytic lymphohistiocytosis, although mortality is very high and almost always secondary to uncontrollable bleeding. While there are no validated treatment protocols, treatment of MAS often includes IV methylprednisolone and Intravenous immune globulin as first-line treatment with Anakinra as second-line treatment.

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