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2019, Number 1

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Ann Hepatol 2019; 18 (1)

Sclerosing Cholangitis Related to IgG4: Not Always a Curable Entity

Martínez-Valle F, Riveiro-Barciela M, Salcedo María-Teresa, Merino-Casabiel X, Fernández-Codina A, de Torres I, Esteban R, Buti M

Language: English
References: 18
Page: 215-219
PDF size: 267.06 Kb.


ABSTRACT

IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. One out of three patients with IgG4 sclerosing cholangitis also presents autoimmune pancreatitis, although it can be associated with manifestations in other organs. One of the main features of IgG4-related disease is its good prognosis due to the great response to glucocorticoid therapy. However, relapse of the disease is not uncommon, especially when steroid therapy is decreased or stopped. Rituximab seems to be an effective treatment to achieve remission of the disease. We report the case of a 74 year-old man diagnosed with IgG4-related disease based on increase of serum IgG4 levels, imaging and histopathological findings, with systemic involvement including sclerosing cholangitis. Despite the absence of liver fibrosis at onset, the early use of glucocorticoids and rituximab therapy, the patient presented clinical and analytical deterioration, leading to secondary biliary cirrhosis. In conclusion, this clinical case highlights the importance of prompt diagnosis and therapeutics for sclerosing cholangitis secondary to IgG4-related disease in order to avoid progression of the disease and development of liver cirrhosis, as well as the refractory, aggressive nature of the disease in some cases as this one.


REFERENCES

  1. Kamisawa T, Zen Y, Pillai S, Stone JH. Igg4- related disease. Lancet 2015; 385: 1460-71.

  2. Martinez-Valle F, Fernandez-Codina A, Pinal-Fernandez I, Orozco-Galvez O, Vilardell-Tarres M. Igg4-related disease: Evidence from six recent cohorts. Autoimmunity reviews 2017; 16: 168-72.

  3. Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, Chari ST. Rituximab therapy for refractory biliary strictures in immunoglobulin g4-associated cholangitis. Clin Gastroenterol Hepatol 2008; 6: 364-6.

  4. Stone JH, Zen Y, Deshpande V. Igg4- related disesae. N Engl J Med 2012; 366: 539-51.

  5. Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T. Diagnosis of igg4-related sclerosing cholangitis. World J Gastroenterol 2013; 19: 7661-70.

  6. Ohara H, Okazaki K, Tsubouchi H, Inui K, Kawa S, Kamisawa T, Tazuma S, et al. Clinical diagnostic criteria of igg4- related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 2012; 19: 536-42.

  7. Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR, Levy MJ, et al. Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: The mayo clinic experience. Gut 2013; 62: 1607-15.

  8. Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, Desspande V, et al. Rituximab for igg4- related disease: A prospective, open-label trial. Ann Rheum Dis 2015; 74: 1171-7.

  9. Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, Klöppel G, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012 ; 25: 1181-92.

  10. Fernandez-Codina A, Martinez-Valle F, Pinilla B, Lopez C, DeTorres I, Solans-Laque R, Fraile-Rodriguez G, et al. Igg4- related disease: Results from a multicenter spanish registry. Medicine 2015; 94: e1275.

  11. EASL clinical practice guidelines: Management of cholestatic liver diseases. J Hepatol 2009; 51: 237-67.

  12. Lindor KD, Kowdley KV, Harrison ME. ACG clinical guideline: Primary sclerosing cholangitis. Am J Gastroenterol 2015; 110: 646-59.

  13. Karlsen TH, Schrumpf E, Boberg KM. Primary sclerosing cholangitis. Best Pract Res Clin Gastroenterol 2010; 24: 655-66.

  14. Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010; 51: 660-78.

  15. Li Y, Zhou L, Zhao X, Song W, Karunaratna N, Wang B. The importance of igg4 screening in patients diagnosed with primary sclerosing cholangitis in the past: A case rediagnosed as igg4-sc after 10 years. Medicine 2016; 95: e5628.

  16. Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, Chari ST, et al. International consensus guidance statement on the management and treatment of igg4- related disease. Arthritis Rheumatol 2015; 67: 1688-99.

  17. Nakazawa T, Shimizu S, Naitoh I. Igg4-related sclerosing cholangitis. Semin Liver Dis 2016; 36: 216-28.

  18. Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, Nakai Y, et al. Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut 2007; 56: 1719-24.




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