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Pancreatic Disease in Sjögren’s Syndrome and IgG4-Related Disease

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Sjögren’s Syndrome

Abstract

The pancreas is a compound tubular-alveolar gland composed of an exocrine portion (serous acini) and an endocrine portion (islets of Langerhans). The islet cells make up only 1% of the total pancreatic mass.

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References

  1. Meijer JM, Meiners PM, Huddleston Slater JJ, Spijkervet FK, Kallenberg CG, Vissink A, et al. Health-related quality of life, employment and disability in patients with Sjogren’s syndrome. Rheumatology (Oxford). 2009;48(9):1077–82.

    Article  Google Scholar 

  2. Cardell BS, Gurling KJ. Observations on the pathology of Sjögren’s syndrome. J Pathol Bacteriol. 1964;68:137–46.

    Article  Google Scholar 

  3. Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ. Sjoegren’s syndrome. A clinical, pathological, and serological study of sixty-two cases. Medicine (Baltimore). 1965;44:187–231.

    Article  CAS  Google Scholar 

  4. Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas – an autonomous pancreatic disease? Am J Dig Dis. 1961;6:688–98.

    Article  PubMed  CAS  Google Scholar 

  5. Fenster F, Buchanan WW, Laster L, Bunim J. Studies of pancreatic function in Sjögren’s ­syndrome. Ann Intern Med. 1964;61:498–508.

    PubMed  CAS  Google Scholar 

  6. Wakfram R, Kopelman H, Tsantoufas D, Williams Ft. Chronic pancreatitis, sclerosing cholangitis, and sicca complex in two siblings. Lancet. 1975;1:550–2.

    Google Scholar 

  7. Sjogren I, Wengle B, Korsgren M. Primary sclerosing cholangitis associated with fibrosis of the submandibular glands and the pancreas. Acta Med Scand. 1979;205(1–2):139–41.

    PubMed  CAS  Google Scholar 

  8. Nieminen U, Koivisto T, Kahri A, Farkkila M. Sjogren’s syndrome with chronic pancreatitis, sclerosing cholangitis, and pulmonary infiltrations. Am J Gastroenterol. 1997;92(1):139–42.

    PubMed  CAS  Google Scholar 

  9. Fukui T, Okazaki K, Yoshizawa H, Ohashi S, Tamaki H, Kawasaki K, et al. A case of autoimmune pancreatitis associated with sclerosing cholangitis, retroperitoneal fibrosis and Sjogren’s syndrome. Pancreatology. 2005;5(1):86–91.

    Article  PubMed  Google Scholar 

  10. Pickartz T, Pickartz H, Lochs H, Ockenga J. Overlap syndrome of autoimmune pancreatitis and cholangitis associated with secondary Sjogren’s syndrome. Eur J Gastroenterol Hepatol. 2004;16(12):1295–9.

    Article  PubMed  Google Scholar 

  11. Eckstein RP, Hollings RM, Martin PA, Katelaris CH. Pancreatic pseudotumor arising in association with Sjogren’s syndrome. Pathology. 1995;27(3):284–8.

    Article  PubMed  CAS  Google Scholar 

  12. Nakamura M, Okumura N, Sakakibara A, Kawai H, Takeichi M, Kanzaki M. A case of Sjögren’s syndrome presenting pancreatitis symptoms which responded to steroid therapy. Proc Jpn Pancreas Soc. 1976;6:135–6.

    Google Scholar 

  13. 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):1561–8.

    Article  PubMed  CAS  Google Scholar 

  14. DiMagno EP, Go VL, Summerskill WH. Relations between pancreatic enzyme and malabsorption in severe pancreatic insufficiency. N Engl J Med. 1973;288(16):813–5.

    Article  PubMed  CAS  Google Scholar 

  15. Ramos-Casals M, Solans R, Rosas J, Camps MT, Gil A, Del Pino-Montes J, et al. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine (Baltimore). 2008;87(4):210–9.

    Article  CAS  Google Scholar 

  16. Sheikh SH, Shaw-Stiffel TA. The gastrointestinal manifestations of Sjogren’s syndrome. Am J Gastroenterol. 1995;90(1):9–14.

    PubMed  CAS  Google Scholar 

  17. Ostuni PA, Gazzetto G, Chieco-Bianchi F, Riga B, Plebani M, Betterle C, et al. Pancreatic exocrine involvement in primary Sjogren’s syndrome. Scand J Rheumatol. 1996;25(1):47–51.

    Article  PubMed  CAS  Google Scholar 

  18. Coll J, Navarro S, Tomas R, Elena M, Martinez E. Exocrine pancreatic function in Sjogren’s syndrome. Arch Intern Med. 1989;149(4):848–52.

    Article  PubMed  CAS  Google Scholar 

  19. Nishimori I, Morita M, Kino J, Onodera M, Nakazawa Y, Okazaki K, et al. Pancreatic involvement in patients with Sjogren’s syndrome and primary biliary cirrhosis. Int J Pancreatol. 1995;17(1):47–54.

    PubMed  CAS  Google Scholar 

  20. Hradsky M, Bartos V, Keller O. Pancreatic function in Sjogren’s syndrome. Gastroenterologia. 1967;108(5):252–60.

    Article  PubMed  CAS  Google Scholar 

  21. Lankishc PG, Arglebe C, Chilla R. Pancreatic function in Sjogren’s syndrome. Dig Dis Sci. 1988;33:11.

    Google Scholar 

  22. Gobelet C, Gerster JC, Rappoport G, Hiroz CA, Maeder E. A controlled study of the exocrine pancreatic function in Sjogren’s syndrome and rheumatoid arthritis. Clin Rheumatol. 1983;2(2):139–43.

    Article  PubMed  CAS  Google Scholar 

  23. Kelly CA, Katrak A, Griffiths ID. Pancreatic function in patients with primary Sjogren’s syndrome. Br J Rheumatol. 1993;32(2):169.

    Article  PubMed  CAS  Google Scholar 

  24. Afzelius P, Fallentin EM, Larsen S, Moller S, Schiodt M. Pancreatic function and morphology in Sjogren’s syndrome. Scand J Gastroenterol. 2010;45(6):752–8.

    Article  PubMed  Google Scholar 

  25. Ramos-Casals M, Brito-Zeron P, Siso A, Vargas A, Ros E, Bove A, et al. High prevalence of serum metabolic alterations in primary Sjogren’s syndrome: influence on clinical and immunological expression. J Rheumatol. 2007;34(4):754–61.

    PubMed  CAS  Google Scholar 

  26. Onodera M, Okazaki K, Morita M, Nishimori I, Yamamoto Y. Immune complex specific for the pancreatic duct antigen in patients with idiopathic chronic pancreatitis and Sjögren’s syndrome. Autoimmunity. 1994;19(1):23–9.

    Article  PubMed  CAS  Google Scholar 

  27. Sundkvist G, Lindahlg G, Koskinene P, Bolinder J. Pancreatic autoantibodies and pancreatic function in Sjögreńs syndrome. Int J Pancreatol. 1991;8:141–9.

    Google Scholar 

  28. Ludwig H, Schernthaner G, Scherak O, Kolarz G. Antibodies to pancreatic duct cells in Sjögren’s syndrome and rheumatoid arthritis. Gut. 1977;18:311–5.

    Article  PubMed  CAS  Google Scholar 

  29. Tsianos EB, Tzioufas AG, Kita MD, Tsolas O, Moutsopoulos HM. Serum isoamylases in patients with autoimmune rheumatic diseases. Clin Exp Rheumatol. 1984;2(3):235–8.

    PubMed  CAS  Google Scholar 

  30. Szanto L, Farkas K, Gyulai E. On Sjogren’s disease. Rheumatism. 1957;13(3):60–3.

    PubMed  CAS  Google Scholar 

  31. Bucher UG, Reid L. Sjogren’s syndrome: report of a fatal case with pulmonary and renal lesions. Br J Dis Chest. 1959;53:237–52.

    Article  PubMed  CAS  Google Scholar 

  32. Kamisawa T, Tu Y, Egawa N, Tsuruta K, Okamoto A, Kodama M, et al. Can MRCP replace ERCP for the diagnosis of autoimmune pancreatitis? Abdom Imaging. 2009;34(3):381–4.

    Article  PubMed  CAS  Google Scholar 

  33. Camatte R, Sarles H. Treatment of digestive disorders of allergic origin with prothipendyl hydrochloride (Dominal). Sem Hop. 1961;37:2989–94.

    PubMed  CAS  Google Scholar 

  34. 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):732–8.

    Article  PubMed  CAS  Google Scholar 

  35. Deshpande V, Chicano S, Finkelberg D, Selig MK, Mino-Kenudson M, Brugge WR, et al. Autoimmune pancreatitis: a systemic immune complex mediated disease. Am J Surg Pathol. 2006;30(12):1537–45.

    Article  PubMed  Google Scholar 

  36. Deshpande V, Gupta R, Sainani NI, Sahani DV, Virk R, Ferrone CR, et al. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011;35:26–35.

    Google Scholar 

  37. Zhang L, Notohara K, Levy MJ, Chari ST, Smyrk TC. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol. 2007;20(1):23–8.

    Article  PubMed  CAS  Google Scholar 

  38. Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, et al. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology. 2010;139(1):140–8; quiz e12–3.

    Article  PubMed  Google Scholar 

  39. Okazaki K, Kawa S, Kamisawa T, Shimosegawa T, Tanaka M. Japanese consensus guidelines for management of autoimmune pancreatitis: I. Concept and diagnosis of autoimmune pancreatitis. J Gastroenterol. 2010;45(3):249–65.

    Article  PubMed  Google Scholar 

  40. Frulloni L, Scattolini C, Falconi M, Zamboni G, Capelli P, Manfredi R, et al. Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients. Am J Gastroenterol. 2009;104(9):2288–94.

    Article  PubMed  Google Scholar 

  41. Sahani DV, Kalva SP, Farrell J, Maher MM, Saini S, Mueller PR, et al. Autoimmune pancreatitis: imaging features. Radiology. 2004;233(2):345–52.

    Article  PubMed  Google Scholar 

  42. Irie H, Honda H, Baba S, Kuroiwa T, Yoshimitsu K, Tajima T, et al. Autoimmune pancreatitis: CT and MR characteristics. AJR Am J Roentgenol. 1998;170(5):1323–7.

    PubMed  CAS  Google Scholar 

  43. Sugumar A, Levy MJ, Kamisawa T, JM Webster G, Kim MH, Enders F, et al. Endoscopic retrograde pancreatography criteria to diagnose autoimmune pancreatitis: an international multicentre study. Gut. 2011;60(5):666–70.

    Article  PubMed  Google Scholar 

  44. Sah RP, Chari ST. Serologic issues in IgG4-related systemic disease and autoimmune pancreatitis. Curr Opin Rheumatol. 2011;23(1):108–13.

    Article  PubMed  CAS  Google Scholar 

  45. Ghazale A, Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol. 2007;102(8):1646–53.

    Article  PubMed  CAS  Google Scholar 

  46. Raina A, Greer JB, Whitcomb DC. Serology in autoimmune pancreatitis. Minerva Gastroenterol Dietol. 2008;54(4):375–87.

    PubMed  CAS  Google Scholar 

  47. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006;355(25):2670–6.

    Article  PubMed  CAS  Google Scholar 

  48. 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):2135–42.

    Article  PubMed  CAS  Google Scholar 

  49. Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41(7):626–31.

    Article  PubMed  Google Scholar 

  50. Kasai T, Miyauchi K, Kurata T, Satoh H, Ohta H, Tanimoto K, et al. Long-term (11-year) statin therapy following percutaneous coronary intervention improves clinical outcome and is not associated with increased malignancy. Int J Cardiol. 2007;114(2):210–7.

    Article  PubMed  Google Scholar 

  51. Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic’s HISORt criteria. J Gastroenterol. 2007;42 Suppl 18:39–41.

    Article  PubMed  Google Scholar 

  52. Otsuki M, Chung JB, Okazaki K, Kim MH, Kamisawa T, Kawa S, et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis. J Gastroenterol. 2008;43(6):403–8.

    Article  PubMed  CAS  Google Scholar 

  53. Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98(12):2811–2.

    Article  PubMed  Google Scholar 

  54. Deshpande V, Sainani NI, Chung RT, Pratt DS, Mentha G, Rubbia-Brandt L, et al. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Mod Pathol. 2009;22(10):1287–95.

    Article  PubMed  CAS  Google Scholar 

  55. Deshpande V, Mino-Kenudson M, Brugge W, Lauwers GY. Autoimmune pancreatitis: more than just a pancreatic disease? A contemporary review of its pathology. Arch Pathol Lab Med. 2005;129(9):1148–54.

    PubMed  Google Scholar 

  56. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17(5):303–32.

    Article  PubMed  CAS  Google Scholar 

  57. Okazaki K, Uchida K, Miyoshi H, Ikeura T, Takaoka M, Nishio A. Recent concepts of autoimmune pancreatitis and IgG4-related disease. Clin Rev Allergy Immunol. 2011;41:126–38.

    Google Scholar 

  58. Geyer JT, Deshpande V. IgG4-associated sialadenitis. Curr Opin Rheumatol. 2011;23(1):95–101.

    Article  PubMed  CAS  Google Scholar 

  59. Geyer JT, Ferry JA, Harris NL, Stone JH, Zukerberg LR, Lauwers GY, et al. Chronic sclerosing sialadenitis (Kuttner tumor) is an IgG4-associated disease. Am J Surg Pathol. 2010;34(2):202–10.

    Article  PubMed  Google Scholar 

  60. Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36(7):710–7.

    Article  PubMed  CAS  Google Scholar 

  61. Cornell LD, Chicano SL, Deshpande V, Collins AB, Selig MK, Lauwers GY, et al. Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease. Am J Surg Pathol. 2007;31(10):1586–97.

    Article  PubMed  Google Scholar 

  62. Lindstrom KM, Cousar JB, Lopes MB. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol. 2010;120(6):765–76.

    Article  PubMed  CAS  Google Scholar 

  63. Zen Y, Onodera M, Inoue D, Kitao A, Matsui O, Nohara T, et al. Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol. 2009;33(12):1833–9.

    Article  PubMed  Google Scholar 

  64. Movitz C, Brive L, Hellstrand K, Rabiet MJ, Dahlgren C. The annexin I sequence gln(9)-ala(10)-trp(11)-phe(12) is a core structure for interaction with the formyl peptide receptor 1. J Biol Chem. 2010;285(19):14338–45.

    Article  PubMed  CAS  Google Scholar 

  65. Bartholomew LG, Cain JC, Woolner LB, Utz DC, Ferris DO. Sclerosing cholangitis: its possible association with Riedel’s struma and fibrous retroperitonitis. Report of two cases. N Engl J Med. 1963;269:8–12.

    Article  PubMed  CAS  Google Scholar 

  66. Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134(3):706–15.

    Article  PubMed  Google Scholar 

  67. Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23(1):88–94.

    Article  PubMed  CAS  Google Scholar 

  68. Khosroshahi A, Stone JR, Pratt DS, Deshpande V, Stone JH. Painless jaundice with serial multi-organ dysfunction. Lancet. 2009;373(9673):1494.

    Article  PubMed  Google Scholar 

  69. Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34(12):1812–9.

    Article  PubMed  Google Scholar 

  70. 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):1264–9.

    Article  PubMed  Google Scholar 

  71. Zen Y, Nakanuma Y. Pathogenesis of IgG4-related disease. Curr Opin Rheumatol. 2011;23(1):114–8.

    Article  PubMed  CAS  Google Scholar 

  72. Zen Y, Fujii T, Harada K, Kawano M, Yamada K, Takahira M, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology. 2007;45(6):1538–46.

    Article  PubMed  CAS  Google Scholar 

  73. Okazaki K, Uchida K, Ohana M, Nakase H, Uose S, Inai M, et al. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology. 2000;118(3):573–81.

    Article  PubMed  CAS  Google Scholar 

  74. Aalberse RC, Schuurman J. IgG4 breaking the rules. Immunology. 2002;105(1):9–19.

    Article  PubMed  CAS  Google Scholar 

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Khosroshahi, A., Stone, J.H., Deshpande, V. (2011). Pancreatic Disease in Sjögren’s Syndrome and IgG4-Related Disease. In: Ramos-Casals, M., Stone, J., Moutsopoulos, H. (eds) Sjögren’s Syndrome. Springer, London. https://doi.org/10.1007/978-0-85729-947-5_18

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