Autoimmune pancreatitis with biliary tree and liver involvement as a part of IgG4-related autoimmune sclerosing disease. Case report
Honsová, E.; Lodererová, A.; Kostolná, E.; Oliverius, M.
Ceskoslovenska Patologie 46(3): 65-67
We report a rare case of autoimmune pancreatitis associated with sclerosing cholangitis, kidney, and salivary glands involvement. Abdominal ultrasound and CT scan showed enlarged pancreas, multiple nodular mass in the liver and kidney. ERCP showed features compatible with primary sclerosing cholangitis. The working clinical diagnosis considered malignant tumor with liver metastases. Histological examination of the liver biopsy sample revealed inflammatory process with numerous IgG4 positive plasma cells. The diagnostic conclusion of IgG4-related autoimmune sclerosing disease was drawn. The serum IgG level was elevated. Treatment with steroids improved the clinical course, all masses in the liver and kidneys disappeared, and laboratory tests were normalized. Now, 4 years after diagnosis the patient is free of all symptoms.