Immunoglobulin G4-related disease in gastroenterology
Authors:
Peter Mačinga; Jana Jarošová; Julius Špičák; Tomáš Hucl
Authors‘ workplace:
Klinika hepatogastroenterologie, Institut klinické a experimentální medicíny, Praha
Published in:
Vnitř Lék 2021; 67(2): 76-83
Category:
Main Topic
Overview
IgG4-related disease is a recently defined clinical entity that can manifest itself in any organ. The most common gastrointestinal manifestations are diseases of the pancreas (autoimmune pancreatitis type 1) and biliary tree (IgG4-associated cholangitis); involvement of liver parenchyma is uncommon and the affection of tubular organs is very rare. IgG4-related pancreatitis and cholangitis can mimic malignancies in their clinical presentation. Diagnosis is often difficult and requires careful evaluation of the combination of symptoms, serology and imaging findings, while adhering to the established diagnostic criteria. The first line of treatment is the administration of corticoids and the remission is achieved in the vast majority of patients. In case of contraindication, intolerance or failure of corticotherapy, patients should receive B cell depletion therapy (rituximab). Based on the available knowledge, monotherapy with other immunosuppressants is not considered to be sufficiently effective. Some patients may benefit from maintenance treatment to prevent relapse, which is otherwise common in both IgG4-related pancreatitis and cholangitis. Recognized IgG4-related disease has a good prognosis, but some patients develop irreversible fibrotic changes in the affected organ with consequent dysfunction; the possible association of the disease with a higher risk of malignancy has not yet been reliably elucidated.
Keywords:
IgG4-related disease – IgG4-related pancreatitis – autoimmune pancreatitis – IgG4-related cholangitis
Sources
1. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012; 22(1): 1–14.
2. Stone JH, Khosroshahi A, Deshpande V, Chan JK, Heathcote JG, Aalberse R et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum. 2012; 64(10): 3061–3067.
3. Uchida K, Masamune A, Shimosegawa T, Okazaki K. Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009. Int J Rheumatol. 2012; 2012: 358371.
4. Masamune A, Kikuta K, Hamada S, Tsuji I, Takeyama Y, Shimosegawa T et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2016. J Gastroenterol. 2020; 55(4): 462–470.
5. Karim F, Loeffen J, Bramer W, Westenberg L, Verdijk R, van Hagen M et al. IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatr Rheumatol Online J. 2016; 14(1): 18.
6. Miyabe K, Zen Y, Cornell LD, Rajagopalan G, Chowdhary VR, Roberts LR et al. Gastrointestinal and Extra‑ Intestinal Manifestations of IgG4-Related Disease. Gastroenterology. 2018; 155(4): 990–1003 e1.
7. Wallace ZS, Zhang Y, Perugino CA, Naden R, Choi HK, Stone JH et al. Clinical phenotypes of IgG4-related disease: an analysis of two international cross‑sectional cohorts. Ann Rheum, DiS. 2019; 78(3): 406–412.
8. Lanzillotta M, Campochiaro C, Mancuso G, Ramirez GA, Capurso G, Falconi M, et al. Clinical phenotypes of IgG4-related disease reflect different prognostic outcomes. Rheumatology (Oxford). 2020; 59(9): 2435–2442.
9. Kawa S, Ota M, Yoshizawa K, Horiuchi A, Hamano H, Ochi Y et al. HLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population. Gastroenterology. 2002; 122(5): 1264–1269.
10. Perugino CA, AlSalem SB, Mattoo H, Della‑Torre E, Mahajan V, Ganesh G et al. Identification of galectin-3 as an autoantigen in patients with IgG4-related disease. J Allergy Clin Immunol. 2019; 143(2): 736–745 e6.
11. Shiokawa M, Kodama Y, Sekiguchi K, Kuwada T, Tomono T, Kuriyama K et al. Laminin 511 is a target antigen in autoimmune pancreatitis. Sci Transl Med. 2018; 10(453).
12. Hubers LM, Vos H, Schuurman AR, Erken R, Oude Elferink RP, Burgering B et al. Annexin A11 is targeted by IgG4 and IgG1 autoantibodies in IgG4-related disease. Gut. 2018; 67(4): 728–735.
13. de Buy Wenniger LJ, Culver EL, Beuers U. Exposure to occupational antigens might predispose to IgG4-related disease. Hepatology. 2014; 60(4): 1453–1454.
14. Detlefsen S, Drewes AM. Autoimmune pancreatitis. Scand J Gastroenterol. 2009; 44(12): 1391–407.
15. Lohr JM, Beuers U, Vujasinovic M, Alvaro D, Frokjaer JB, Buttgereit F et al. European Guideline on IgG4-related digestive disease - UEG and SGF evidence‑based recommendations. United European Gastroenterol J. 2020; 8(6): 637–666.
16. Deshpande V. IgG4-Related Disease of the Gastrointestinal Tract: A 21st Century Chameleon. Arch Pathol Lab Med. 2015; 139(6): 742–749.
17. Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci. 1995; 40(7): 1561–1568.
18. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001; 344(10): 732–738.
19. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003; 38(10): 982–984.
20. Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003; 27(8): 1119–1127.
21. Zamboni G, Luttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P et al. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004; 445(6): 552–563.
22. Hart PA, Zen Y, Chari ST. Recent Advances in Autoimmune Pancreatitis. Gastroenterology. 2015; 149(1): 39–51.
23. de Pretis N, Vieceli F, Brandolese A, Brozzi L, Amodio A, Frulloni L. Autoimmune pancreatitis not otherwise specified (NOS): Clinical features and outcomes of the forgotten type. Hepatobiliary Pancreat Dis Int. 2019; 18(6): 576–579.
24. Kanno A, Masamune A, Okazaki K, Kamisawa T, Kawa S, Nishimori I et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2011. Pancreas. 2015; 44(4): 535–539.
25. Schneider A, Michaely H, Weiss C, Hirth M, Ruckert F, Wilhelm TJ et al. Prevalence and Incidence of Autoimmune Pancreatitis in the Population Living in the Southwest of Germany. Digestion. 2017; 96(4): 187–198.
26. Dítě P, Ševčíková A, Novotný I et al. Autoimunní pankreatitida v České republice – region jižní Morava. Čes a Slov Gastroent a Hepatol 2007; 61(2): 82–85.
27. Vujasinovic M, Valente R, Maier P, von Beckerath V, Haas SL, Arnelo U et al. Diagnosis, treatment and long‑term outcome of autoimmune pancreatitis in Sweden. Pancreatology. 2018; 18(8): 900–904.
28. Blaho M, Dite P, Kunovsky L, Kianicka B. Autoimmune pancreatitis - An ongoing challenge. Adv Med Sci. 2020; 65(2): 403–408.
29. Nagpal SJS, Sharma A, Chari ST. Autoimmune Pancreatitis. Am J Gastroenterol. 2018; 113(9): 1301.
30. Sah RP, Chari ST. Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol. 2011; 23(1): 108–113.
31. Frulloni L, Lunardi C, Simone R, Dolcino M, Scattolini C, Falconi M, et al. Identification of a novel antibody associated with autoimmune pancreatitis. N Engl J Med. 2009; 361(22): 2135–2142.
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