Immunoglobulin G4-associated cholangitis

Česká verzia

Authors: Petr Dítě ; L. Husová; Z. Lukáš; Marie Přecechtělová; S. Štěpánková
Authors place of work: Interní hepatogastroenterologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jan Lata, CSc. ¹; Ústav patologie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Josef Feit, CSc. ²
Published in the journal: Vnitř Lék 2010; 56(8): 824-826
Category: Case Reports

Summary

Autoimmune IgG4- associated cholangitis is a new entity among the liver and biliary tree disorders, classified among the so-called IgG4-related diseases. Even though prognosis of this disease is unclear, this type of sclerosing cholangitis is not being linked to a carcinoma. Clinical and laboratory data differ slightly from the findings associated with the usual primary sclerosing cholangitis and it is mainly the high IgG4 level and hyperbilirubinaemia that supports the diagnosis of autoimmune disease. Unlike primary sclerosing cholangitis, this disease is not associated with a malignant prognosis and steroids represent an effective treatment. Combination of steroids with azathioprin is a possible alternative in case of a relapse. Patient’s response to steroid therapy is a diagnosis-supporting criterion. This disease should always be considered as part of differential diagnosis of primary sclerosing cholangitis, especially when autoimmune aberrations or other autoimmune diseases are present. Long-term evaluations of these patients are so far lacking and thus studies on larger patient samples are required.

Key words:
immunoglobulin G4 – sclerosing cholangitis – autoimmune diseases

Zdroje

1. Bartholomew G, Cain JC, Woolner LB et al. Sclerosing cholangitis: its possible association with Riedél’s struma and fibrous retroperitonitis. Report of two case. N Engl J Med 1963; 269: 8–12.

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

3. van Buuren HR, Vleggaar FP, Willemien Erkelens G et al. Autoimmune pancreatocholangitis: a series of ten patients. Scand J Gastroenterol Suppl 2006; 243: 70–78.

4. Takikawa H, Takamori Y, Tanaka A et al. Analysis of 388 cases of primary sclerosing cholangitis in Japan: Presence of a subgroup without pancreatic involvement in older patients. Hepatol Res 2004; 29: 153–159.

5. Björnsson E, Chari ST, Smyrk T et al. Immunoglobulin IgG4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 2007; 45: 1547–1554.

6. Hamano H, Kawa S, Uehara T et al. Immunoglobulin G4‑related lymphoplasmatic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointest Endosc 2005; 62: 152–157.

7. Sood GK, Vij JC, Mohapatra JR et al. Primary sclerosing cholangitis with chronic pancreatitis. Indian Clin Gastroenterol 1999; 9: 309–310.

8. 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.

9. ZenY, Harada K, Sasaki M et al. IgG4‑related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis‑associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol 2004; 28: 1193–1203.

10. 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.

11. Mendes FD, Jorgensen R, Keach J et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101: 2070–2075.

12. Björnsson E, Kilander A, Olsson R. CA 19-9 and CEA are unreliable markers for cholangiocarcinoma in patients with primary sclerosing cholangitis. Liver 1999; 19: 501–508.

13. Erkelens GW, Vleggaar FP, Lesterhuis W et al. Sclerosing pancreato-cholangitis responsive to steroid therapy. Lancet 1999; 354: 43–44.

14. Stathopoulos G, Nourmand AD, Blackstone M et al. Rapidly progressive sclerosing cholangitis following surgical treatment of pancreatic pseudotumor. J Clin Gastroenterol 1995; 21: 143–148.

15. Uehara T, Hamano H, Kawa S et al. Distinct clinicopathological entity “autoimmune pancreatitis‑associated sclerosing cholangitis”. Pathol Int 2005; 55: 405–411.

16. Kuroiwa T, Suda T, Takahashi T et al. Bile duct involvement in a case of autoimmune pancreatitis successfully treated with an oral steroid. Dig Dis Sci 2002; 47: 1810–1816.

17. Kyokane K, Ichihara T, Horisawa M et al. Successful treatment of primary sclerosing cholangitis with cyclosporine and corticosteroids. Hepatogastroenterology 1994; 41: 449–452.