UnMASking the Diagnosis: Idiopathic Non-Cirrhotic Portal Hypertension Diagnosed in the Setting of Macrophage Activation Syndrome Complicated by Severe Pancreatitis: 2017 Presidential Poster Award

Abstract

Systemic Lupus Erythematosus (SLE) is a common rheumatologic condition with known GI involvement, most notably autoimmune hepatitis and lupus colitis. Acute pancreatitis (AP) is a rare complication of SLE and is typically associated with disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multiorgan dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare ultimately complicated by MAS in an adult patient and successfully treated with Anakinra. A 29 year-old female with SLE and non-adherence to medical therapy was admitted with severe necrotizing AP evidenced by epigastric pain, nausea, oral intolerance, lipase >3000 U/L, and CT scan demonstrating necrosis and inflammation in the pancreatic head. After admission, she developed tachycardia, hypothermia, lactate elevation of 17.2, and hypercarbic respiratory failure with pulmonary edema requiring intubation and mechanical ventilation with aggressive supportive care. Laboratory evaluation demonstrated evidence of DIC with pancytopenia, elevated ANA/ds DNA, and a ferritin of 1332 ng/mL. She was started on methylprednisolone for treatment of a SLE flare with clinical improvement leading to extubation. Following extubation, her ferritin level doubled in association with clinical deterioration raising suspicion for MAS. She was then started on Anakinra resulting in brisk clinical response and down trending ferritin. AP occurs in 1-8% of SLE patients and is proposed to be the result of vascular damage. MAS complicates approximately 5.5% of childhood SLE compared to 0.7% of adult SLE but is more commonly the result of AP in combination with SLE compared to SLE alone and is associated with higher mortality rates. Our case uniquely illustrates a rare etiology of AP and the relationship between systemic diseases and the GI system in a critically ill patient. Treatment of MAS is aimed at treating the underlying SLE; however, our patient required the addition of a chemotherapeutic agent. Anakinra is a novel agent which has been used to treat refractory childhood MAS and was successful in treating this adult patient. To the best of our knowledge, this is the only reported case of refractory MAS due to SLE induced AP necessitating treatment with Anakinra.