Medical College of Georgia Philadelphia, PA, United States
Michael Coles, MD, Pooja Mude, MD, Robert Kennedy, MD Medical College of Georgia, Augusta, GA
Introduction: Unexplained pancreatic exo/endocrine insufficiency should prompt evaluation for autoimmune pancreatitis.
Case Description/Methods: 54-year-old African-American male with past medical history significant for prior DVT was admitted for initial episode of Hyperosmolar Hyperglycemic state (HHS) after complaining of general malaise and polydipsia. Further history taking revealed acute on chronic steathorea and a subacute weight loss of fifteen pounds. Patient's HHS was managed with fluid resuscitation and an insulin infusion. Work-up of his steathorea revealed an undetectable fecal elastase level. His labs were significant for AST 94 U/L, ALT/143 U/L ALP of 144 U/L. A CT of his abdomen and pelvis with contrast demonstrated an atrophic pancreas with diffuse calcifications (Figure 1A/B). Follow up MRCP demonstrated irregular pancreatic duct with strictures and dilated side branches with mild dilation of the common bile duct. Initial etiology of the patient's presentation with HHS was thought to be secondary to new diagnosis of Latent Autoimmune diabetes (LADA), given the presence of positive Glutamic Acid Decarboxylase-65 antibodies, and low C-peptide levels in the context of hyperglycemia. However, in view the of the symptoms of steatorhea, additional investigations were sent which demonstrated elevated IgG4 at 106.40 mg/dL concerning for Autoimmune Pancreatitis (AIP) and Type 3c (pancreatogenic) diabetes mellitus. He denied any history tobacco/alcohol abuse. The patient was treated with pancrealipase and insulin therapy which resulted in an improvement in symptoms.
Discussion: The 2011 International Consensus Diagnostic Criteria for AIP confirms an acute state of the disease. However, literature had validated that approximately 40% of patients with AIP experienced pancreatic stone formation, pancreatic atrophy, and/or irregular dilatation of the main pancreatic duct over a long-term course. Patients with AIP generally respond favorably to Prednisolone therapy in the acute setting and utilized to prevent pancreatic stone formation with progression to chronic pancreatitis and subsequent endo/exocrine deficiency. Our patients clinical and radiologic findings were more consistent with an advanced disease state limiting the utility of steroid administration. Undifferentiated pancreatitis without typical risk factor exposures should prompt Gastroenterologist's suspicion of AIP to prevent progression to advanced disease states as exemplified in our patient with pancreatogenic Type 3c Diabetes mellitus.
Figure: Figure 1A: Coronal section of a CT Abdomen and Pelvis with IV contrast demonstrating an atrophic pancreas with diffuse calcifications (demarcated by the red outline) consistent with chronic pancreatitis and no acute stigmata. Figure 1B: Sagittal section of a CT Abdomen and Pelvis with IV contrast demonstrating an atrophic pancreas diffuse pancreatic calcifications (demarcated by the red outline) consistent with chronic pancreatitis and no acute stigmata. Incidental finding of a large stool ball within the rectum.
Disclosures: Michael Coles indicated no relevant financial relationships. Pooja Mude indicated no relevant financial relationships. Robert Kennedy indicated no relevant financial relationships.
Michael Coles, MD, Pooja Mude, MD, Robert Kennedy, MD. P2144 - Chronic Autoimmune Pancreatitis Presenting With Exocrine Deficiency and Type 3c Diabetes Mellitus, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.