Abstract
Purpose: Schnitzler syndrome, a rare autoimmune inflammatory disorder, is characterized by chronic urticaria, IgM gammopathy, and systemic inflammation, e.g., fever, leukocytosis, lymphadenopathy, and arthritis. We present a case of acute on chronic pancreatitis in a patient with Schnitzler syndrome, a condition not previously clearly associated with chronic pancreatitis. A 50-year-old white female with a complicated past medical history including recurrent abdominal pain, pancreatitis, leukocytosis, urticaria and IgM gammopathy presented with worsening abdominal pain. Pain started 4 days prior to admission and was located over the epigastric and periumbilical areas. Pain was sharp, without aggravating or alleviating factors. Associated symptoms included chronic diarrhea, fatigue, and weight loss. The diagnosis of Schnitzler syndrome was made three years ago at another hospital when the patient presented with, among other symptoms, chronic abdominal pain. Her condition resolved with IL-1R antagonist, Anakinra, injection. Unfortunately, she stopped Anakinra treatments due to financial constraints, resulting in frequent hospital admissions mostly for intractable abdominal pain and diarrhea. Past surgical history included pancreatic cyst drainage at age of 10. Patient denied alcohol use. Family history was positive for alcoholic pancreatitis. Physical examination revealed a stable but cachectic female. Abdomen was soft, mildly distended with normoactive bowel sounds, and tender to palpation. Relevant laboratory results included WBC 39,600, amylase 6, lipase 46, alkaline phosphatase 226, AST 8, ALT 22, and Clostridium difficile-negative stool. Abdominal CT showed a diffusely calcified pancreas consistent with chronic pancreatitis. The patient was managed conservatively for acute on chronic pancreatitis. Upon stabilization, she was arranged for transfer to a nearby university center for management of her underlying Schnitzler syndrome. IL-1, a key pathogenetic cytokine in Schnitzler syndrome, also contributes to the development of chronic pancreatitis. In mice, IL-1β overexpression in the pancreas induces chronic pancreatitis; IL-1R antagonist attenuates disease progression. IL-1β is processed by the inflammasome, an intracellular multiprotein complex. A gain-of-function mutation in NLRP3 inflammasome has been identified in a patient with Schnitzler syndrome. Therefore, therapy that targets the inflammasome/IL-1 pathway can be expected to ameliorate chronic pancreatitis secondary to Schnitzler syndrome. We also propose that Schnitzler syndrome be considered among the differential diagnoses for patients presenting with unexplained chronic pancreatitis.
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