Autoimmune pancreatitis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]

Overview

Glucocorticoids are found to play an important role in the management of autoimmune pancreatitis via several ways such as efficacy in alleviating symptoms, decreasing the size of the pancreas, reversing histopathologic features in patients with AIP, and mprovement of lab findings. About 2/3rd of patients show good response to glucocorticoids with complete recovery, 25% may require a second course of glucocorticoids, and a few patients with autoimmune pancreatitis may require continuous treatment. Immunomodulatory drugs such as azathioprine are usually used when, AIP patients have no response to steroid management, relapse occurs and patients cannot be weaned off steroids.

Medical Therapy

Role of Glucocorticoids:

  • Preferred regimen: Prednisone is usually administered at an initial dose of 40 mg/d for 4 weeks followed by a taper of the daily dosage by 5 mg/wk depending upon the clinical parameters.
  • Glucocorticoids are found to play an important role in the management of autoimmune pancreatitis via several ways such as:[1][2][3][4]
    • Efficacy in alleviating symptoms.
    • Decreasing the size of the pancreas.
    • Reversing histopathologic features in patients with AIP.
    • Improvement of lab findings such as:[5]
Response to Glucocorticoids:
  • About 2/3rd of patients show good response to glucocorticoids with complete recovery.[6][7][8]
  • Approximately 25% may require a second course of glucocorticoids.
  • A few patients with autoimmune pancreatitis may require continuous treatment.

Response to Glucocorticoids in patients with Biliary strictures:

Patients with biliary strictures have a variable response to glucocorticoids such as:[6][8][9][10][11]

Monitoring of clinical parameters in patients on glucocorticoid therapy:

Glucocorticoids are tapered depending upon following clinical parameters:[2][3][12][13]

  • Relief of symptoms.
  • Serial changes in abdominal imaging of the pancreas and bile ducts.
  • Decreased serum γ-globulin and IgG4 levels.
  • Improvements in liver function tests.

Role of Immunomodulatory drugs:

Immunomodulatory drugs such as azathioprine are usually used when:

  • AIP patients have no response to steroid management.
  • AIP patients relapse, if steroids are discontinued.
  • AIP patients cannot be weaned off steroids.

The monoclonal antibody, Rituximab, is found to be effective in the management of AIP but further studies are required for the recommendation of routine use.[14][15][16]

References

  1. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A, Kamata N (2003). "Clinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma". Am. J. Gastroenterol. 98 (12): 2694–9. doi:10.1111/j.1572-0241.2003.08775.x. PMID 14687819.
  2. 2.0 2.1 Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, Nakai Y, Sasahira N, Tsujino T, Yoshida H, Kawabe T, Omata M (2007). "Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment". Gut. 56 (12): 1719–24. doi:10.1136/gut.2006.115246. PMC 2095691. PMID 17525092.
  3. 3.0 3.1 Moon SH, Kim MH, Park DH, Hwang CY, Park SJ, Lee SS, Seo DW, Lee SK (2008). "Is a 2-week steroid trial after initial negative investigation for malignancy useful in differentiating autoimmune pancreatitis from pancreatic cancer? A prospective outcome study". Gut. 57 (12): 1704–12. doi:10.1136/gut.2008.150979. PMID 18583399.
  4. Ito T, Nakano I, Koyanagi S, Miyahara T, Migita Y, Ogoshi K, Sakai H, Matsunaga S, Yasuda O, Sumii T, Nawata H (1997). "Autoimmune pancreatitis as a new clinical entity. Three cases of autoimmune pancreatitis with effective steroid therapy". Dig. Dis. Sci. 42 (7): 1458–68. PMID 9246047.
  5. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K (2001). "High serum IgG4 concentrations in patients with sclerosing pancreatitis". N. Engl. J. Med. 344 (10): 732–8. doi:10.1056/NEJM200103083441005. PMID 11236777.
  6. 6.0 6.1 Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, Topazian MD, Clain JE, Pearson RK, Petersen BT, Vege SS, Lindor K, Farnell MB (2008). "Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy". Gastroenterology. 134 (3): 706–15. doi:10.1053/j.gastro.2007.12.009. PMID 18222442.
  7. Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Whitcomb DC, Slivka A (2009). "Evaluation and management of autoimmune pancreatitis: experience at a large US center". Am. J. Gastroenterol. 104 (9): 2295–306. doi:10.1038/ajg.2009.325. PMID 19532132.
  8. 8.0 8.1 Sandanayake NS, Church NI, Chapman MH, Johnson GJ, Dhar DK, Amin Z, Deheragoda MG, Novelli M, Winstanley A, Rodriguez-Justo M, Hatfield AR, Pereira SP, Webster GJ (2009). "Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis". Clin. Gastroenterol. Hepatol. 7 (10): 1089–96. doi:10.1016/j.cgh.2009.03.021. PMID 19345283.
  9. Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB (2006). "Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience". Clin. Gastroenterol. Hepatol. 4 (8): 1010–6, quiz 934. doi:10.1016/j.cgh.2006.05.017. PMID 16843735.
  10. Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A (2004). "Morphological changes after steroid therapy in autoimmune pancreatitis". Scand. J. Gastroenterol. 39 (11): 1154–8. doi:10.1080/00365520410008033. PMID 15545176.
  11. Wakabayashi T, Kawaura Y, Satomura Y, Watanabe H, Motoo Y, Sawabu N (2005). "Long-term prognosis of duct-narrowing chronic pancreatitis: strategy for steroid treatment". Pancreas. 30 (1): 31–9. PMID 15632697.
  12. Maire F, Le Baleur Y, Rebours V, Vullierme MP, Couvelard A, Voitot H, Sauvanet A, Hentic O, Lévy P, Ruszniewski P, Hammel P (2011). "Outcome of patients with type 1 or 2 autoimmune pancreatitis". Am. J. Gastroenterol. 106 (1): 151–6. doi:10.1038/ajg.2010.314. PMID 20736934.
  13. Sahani DV, Sainani NI, Deshpande V, Shaikh MS, Frinkelberg DL, Fernandez-del Castillo C (2009). "Autoimmune pancreatitis: disease evolution, staging, response assessment, and CT features that predict response to corticosteroid therapy". Radiology. 250 (1): 118–29. doi:10.1148/radiol.2493080279. PMID 19017924.
  14. Hart PA, Topazian MD, Witzig TE, Clain JE, Gleeson FC, Klebig RR, Levy MJ, Pearson RK, Petersen BT, Smyrk TC, Sugumar A, Takahashi N, Vege SS, Chari ST (2013). "Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience". Gut. 62 (11): 1607–15. doi:10.1136/gutjnl-2012-302886. PMID 22936672.
  15. Topazian M, Witzig TE, Smyrk TC, Pulido JS, Levy MJ, Kamath PS, Chari ST (2008). "Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis". Clin. Gastroenterol. Hepatol. 6 (3): 364–6. doi:10.1016/j.cgh.2007.12.020. PMID 18328441.
  16. Rueda JC, Duarte-Rey C, Casas N (2009). "Successful treatment of relapsing autoimmune pancreatitis in primary Sjögren's syndrome with rituximab: report of a case and review of the literature". Rheumatol. Int. 29 (12): 1481–5. doi:10.1007/s00296-009-0843-5. PMID 19137434.

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