Autoimmune pancreatitis and IgG-positive sclerosing cholangitis

Česká verzia

Authors: Petr Dítě ¹ ; I. Novotný ²; J. Lata ³; M. Růžička ⁴; E. Geryk ¹; Bohuslav Kianička ⁵
Authors place of work: Lékařská fakulta MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Aleš Hep, CSc. ¹; Masarykův onkologický ústav Brno, ředitel prof. MUDr. Jiří Vorlíček, CSc. ²; Lékařská fakulta Ostravské univerzity Ostrava, děkan doc. MUDr. Arnošt Martínek, CSc. ³; Sheikh Khalifa Med. City – Cleveland Clinic, Abu Dhabi, U. A. E. ⁴; Gastroenterologické oddělení II. interní klinika Lékařské fakulty MU a FN u sv. Anny Brno, přednosta prof. MUDr. Miroslav Souček, CSc. ⁵
Published in the journal: Vnitř Lék 2011; 57(3): 254-257
Category: 60th birthday of prof. Mudr. Jiřího Vítovce, CSc, FESC

Summary

Sclerosing cholangitis is a heterogenous disease. Sclerosing cholangitis with an unknown cause is abbreviated PSC. PSC affects extra- as well as intra-hepatic bile ducts and since this is a permanently progressing fibrous condition, it leads to liver cirrhosis. The disease is often associated with a development of cholangocarcinoma and idiopathic intestinal inflammation. Causal therapy does not exist; liver transplantation is indicated. IgG4 cholangitis differs from PSC in a number of features. This form is, unlike PSC, linked to autoimmune pancreatitis (AIP) as well as other IgG4 sclerosing diseases. Anatomically, distal region of ductus choledochus is most frequently involved. Icterus is, unlike in PSC, a frequent symptom of AIP. There also is a distinctive histological picture – significant lymphoplasmatic infiltration of the bile duct wall with abundance of IgG4 has been described, lymphoplasmatic infiltration with fibrosis in the periportal area and the presence of obliterating phlebitis is also typical. However, intact biliary epithelium is a typical feature. IgG4 can be diagnosed even without concurrent presence of AIP. IgG4 sclerosing cholangitis is a condition sensitive to steroid therapy. At present, there is no doubt that IgG4 sclerosing cholangitis is a completely different condition to primary sclerosing cholangitis. From the clinical perspective, these diseases should be differentiated in every clinician’s mind as (a) AIP is treated with corticosteroids and not with an unnecessary surgery, (b) IgG4 sclerosing cholangitis is mostly successfully treated with corticosteroids and the disease is not, unlike PSC, a risk factor for the development of cholangiocarcinoma.

Key words:
autoimmune pancreatitis – IgG4 sclerosing cholangitis – IgG4-Related Sclerosing Disease

Zdroje

1. Yoshida K, Toki, F, Takeuchi T et al. Chronic pancreatitis cause by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995; 40: 1561–1568.

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

3. Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol 2003; 98: 2811–2812.

4. Kamisawa T, Funata N, Hayashi Y et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 2003; 52: 683–687.

5. Kamisawa T, Egawa N, Nakajima H et al. Gastrointestinal findings in patients with autoimmune pancreatitis. Endoscopy 2005; 37: 1127–1130.

6. Kamisawa T, Egawa N, Nakajima H et al. Extrapancreatic lesions in autoimmune pancreatitis. J Clin Gastroenterol 2005; 39: 904–907.

7. Kamisawa T, Nakajima H, Egawa N et al. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology 2006; 6: 132–137.

8. Nakajo M, Jinnouchi S, Fukukura Y et al. The efficacy of whole-body PDG-PET or PET/CT for autoimmune pancreatitis and associated extrapancreatic autoimmune lesions. Eur J Nucl Med Mol Imaging 2007; 34: 2088–2095.

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

10. Comings DE, Skubi KB, Van Eyes J et al. Familial multifocal fibrosclerosis. Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel´s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med 1967; 66: 884–892.

11. Kamisawa T, Egawa N, Inokuma S et al. Pancreatic endocrine and exocrine function and salivary gland function in autoimmune pancreatitis before and after steroid therapy. Pancreas 2003; 27: 235–238.

12. Notohara K, Burgart LJ, Yadav D et al. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol 2003; 27: 1119–1127.

13. Zamboni G, Luttges J, Capelli P et al. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimen and 9 biopsy specimens. Virchows Arch 2004; 445: 552–563.

14. Kamisawa T, Wakabayashi T, Sawabu N Autoimmune pancreatitis in young patients. J Clin Gastroenterol 2006; 40: 847–850.

15. Okazaki K, Uchida K, Ohana M et al. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology 2000; 118: 573–581.

16. Zen Y, Fujii T, Harada K et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology 2007; 45: 1538–1546.

17. Frulloni CS, Scatolini C, Falconi M et al. Autoimmune pancreatitis: differences between the focal and difuse forms in 87 patients. Am J Gastroenterol 2009; 104: 1119–1127.

18. Pearson RK, Longnecker DS, Chari ST et al. Controversies in clinical pancreatology: autoimmune pancreatitis: does in exist? Pancreas 2003; 27: 1–13.

19. Okazaki K, Kawa S, Kamisawa T et al. Research Committee of Intractable Diseases of the Pancreas. Clinical diagnostic criteria of autoimmune pancreatitis: revise proposal. J Gastroenterol 2006; 41: 626–631.

20. Chari ST, Smyrk TC, Levy MJ et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006; 4: 1010–1016.

21. Kamisawa T, Egawa N, Nakajima H et al. Morphological changes after steroid therapy in autoimmune pancreatitis. Scand J Gastroenterol 2004; 39: 1154–1158.

22. Kamisawa T, Yoshiike M, Egawa N et al. Treating patients with autoimmune pancreatitis: results from a long-term follow-up study. Pancreatology 2005; 5: 234–238; discussion 238–240.

23. Kamisawa T, Okamoto A, Wakabayashi T et al. Appropriate steroid therapy for autoimmune pancreatitis based on long-term outcome. Scand J Gastroenterol 2008; 43: 609–613.

24. Takayama M, Hamano H, Ochi Y et al. Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation. Am J Gastroenterol 2004; 99: 932–937.

25. Kamisawa T, Okamoto A Prognosis of autoimmune pancreatitis. J Gastroenterol 2007; 42 (Suppl 18): 59–62.

26. LaRusso NF, Wiesner RH, Ludwig J et al. Current concepts. Primary sclerosing cholangitis. N Engl J Med 1984; 310: 899–903.

27. Wiesner RH, Grambsch PM, Dickson ER et al. Primary sclerosing cholangitis: natural history, prognostic factors and surfoval analysis. Hematology 1989; 10: 430–436.

28. Nakazawa T, Ohara H, Sano H et al. Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis. Pancreas 2005; 30: 20–25.

29. Nakanuma Y, Zen Y Pathology and immunopathology of immunoglobulin G4-related sclerosing cholangitis: The latest addition to the sclerosing cholangitis family. Hepatol Res 2007; 37 (Suppl 3): S478–S486.

30. Ghazale A, Chari ST, Zhang L et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134: 706–715.