IgG-4-Related Gallbladder Disease Mimicking Mirizzi Syndrome
IgG4 related disease (IgG4-RD) is an uncommon diagnosis that may involve multiple organ systems and now unifies many previously isolated diagnoses [1]. There is a growing body of literature describing the clinical presentation, diagnostic criteria, and demographic affected by this immune mediated condition. The most commonly recognized gastrointestinal manifestation of IgG4-RD is autoimmune pancreatitis. However, two additional gastrointestinal manifestations include cholangiopathy [2] and cholecystitis [3]. This case report describes a novel presentation of IgG4-RD involving the biliary tract and gallbladder appearing as a pseudo-Mirizzi type syndrome.

A woman in her sixth decade was referred to our gastroenterology clinic following a hospital admission for obstructive jaundice. Her symptoms began two months prior, when she presented to a community hospital emergency department (ED) reporting a three-day history of jaundice, right upper quadrant abdominal pain, and nausea. She was previously healthy, except for psoriasis and ongoing tobacco use.
In the outside hospital ED the patient was hemodynamically stable and afebrile. The physical exam was significant for jaundice, dry mucus membranes and right upper quadrant abdominal tenderness. Laboratory studies demonstrated elevated alkaline phosphatase 801 U/L (reference 40-150 U/L), elevated total bilirubin 17.7 mg/dL (reference 0.0-1.5 mg/dL) and elevated aspartate transaminase 150 U/L (reference 7-40 U/L) and alanine transaminase 159 U/L (reference 0-45 U/L). The white blood cell count was 12.62 k/uL (3.70-11.00 k/uL)....