#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Autoimmune Pancreatitis with Biliary Tree and Liver Involvement as a Part of IgG4-related Autoimmune Sclerosing Disease. Case Report


Authors: E. Honsová 1;  A. Lodererová 1;  E. Kostolná 2;  M. Oliverius 3
Authors‘ workplace: Transplantcentrum IKEM, Praha, 1Pracoviště klinické a transplantační patologie, 2Klinika hepatogastroenterologie, 3Klinika transplantační chirurgie
Published in: Čes.-slov. Patol., 46, 2010, No. 3, p. 65-67
Category:

Overview

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.

Key words:
autoimmune pancreatitis – IgG4 – primary sclerosing cholangitis


Sources

1. Detlefsen S, Mohr Drewes A, Vyberg M, Kloppel G.: Diagnosis of autoimmune pancreatitis by core biopsy: application of six microscopic criteria. Virchows Arch., 454, 2009, s. 531–539.

2. Fukui T, Okazaki K, Yoshizawa H, et al.: A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjogren,s syndrome. Pancreatology, 5, 2005, s. 86–91.

3. Ghazale A et al.: Immunoglobulin G4-associated cholangitis: clinical profile and response to the therapy. Gastroenterology, 134, 2008, s. 706–715.

4. Kamisawa T, Funata N, Hayashi Y et al.: A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol., 38, 2003, s. 982–984.

5. Kamisawa T, Okamoto A.: Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol., 41, 2006, s. 613–625.

6. Kamisawa T, Okazaki K, Kawa S.: Diagnostic criteria for autoimmune pancreatitis in Japan. Wold J Gastroenterol., 14, 2008, s. 34992–4994.

7. Kitawa S, Zen Y, Harada K et al.: Abundant IgG4-positive plasma celll infiltration characterizes chronic sclerosing sialadenitis (Kuttneręs tumor). Am J Surg Pathol., 29, 2005, s. 783–791.

8. Rudmik L, Trpkov K, Nash C, et al.: Autoimmune pancreatitis associated with renal lesions mimicking metastatic tumours. CMAJ., 15, 2006, s. 367–369.

9. Saeki T, Nishi S, Ito T, et al.: Renal lesions in IgG4-related systemic disease. Internal Medicine., 46, 2007, s. 1365–1371.

10. Sarles H, Sarles JC, Muratore et al.: Chronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease? Am J Dig Dis., 6, 1961, s. 688–698.

11. Watson S, Jenkins D, Bellamy Ch.: Nephropathy in IgG4-related systemic disease. Am J Surg Pathol., 30, 2006, s. 1472–1477.

12. Yamamoto M, Harada S, Oharaha M, Suzuki C, et al.: Clinical and pathological differences between Mikulitzęs disease and Sjőgrenęs syndrome. Rheumatology, 44, 2005, s. 227–234.

13. Yamamoto M, Takahashi H, Ohara M, et al.: A new conceptualization for Mikulitczęs disease as an IgG4-related placatic disease. Mod Rheumatol., 16, 2006, s. 335–340.

14. Yoshida K, Toki F, Takeuchi T et al.: Chronic pancreatitis caused by an autoimmune abnormality: proposal of the concept of autoimmune pancreatitis. Dig. Dis Sci., 40, 1995, s. 1561–1568.

15. Zandieh I, Byrne MF.: Autoimmune pancreatitis: A review. World J Gastroenterol., 47, 2007, s. 6327–6332.

16. Zhang L, Notohara K, Levy MJ et al.: IgG4- positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Modern Pathology, 20, 2007, s. 23–28.

Labels
Anatomical pathology Forensic medical examiner Toxicology
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#