Health Dictionary

Overview

The symptoms of autoimmune pancreatitis usually develop in the fifth decade of life, and start with symptoms such as painless jaundice, dark urine, and mild abdominal pain. During the later course of disease, patients may present with abdominal mass mimicking pancreatic cancer. If left untreated, patients with autoimmune pancreatitis may progress to develop pancreatic insufficiency, diabetes, and pancreatic calcifications or stones. The prognosis is usually good; 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.

Natural History, Complications, and Prognosis

Natural History

The symptoms of autoimmune pancreatitis usually develop in the fifth decade of life, and start with symptoms such as painless jaundice, dark urine, and mild abdominal pain. During the later course of disease, patients may present with abdominal mass mimicking pancreatic cancer.
If left untreated, patients with autoimmune pancreatitis may progress to develop pancreatic insufficiency, diabetes, and pancreatic calcifications or stones.

Complications

Common complications of autoimmune pancreatitis include:
- Pancreatic insufficiency
- Diabetes
- Pancreatic calcifications or stones

Prognosis

The prognosis is usually good and following responses have been observed in response to corticosteroid therapy:
- About 2/3rd of patients show good response to glucocorticoids with complete recovery.^[1]^[2]^[3]
- Approximately 25% may require a second course of glucocorticoids.
- A few patients with autoimmune pancreatitis may require continuous treatment.
Patients with biliary strictures have a variable response to glucocorticoids such as:^[1]^[3]^[4]^[5]^[6]
- Patients with biliary strictures may respond to glucocorticoids.
- Patients with biliary strictures may not respond to glucocorticoids.
- Patients with biliary strictures may have an incomplete response to glucocorticoids.
- Patients with biliary strictures may require maintenance therapy with glucocorticoids to prevent relapse.
- Patients with biliary strictures may require maintenance therapy with glucocorticoids and/or immunomodulatory drugs to prevent relapse.

References

↑ ^1.0 ^1.1 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.
↑ 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.
↑ ^3.0 ^3.1 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.
↑ 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.
↑ 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.
↑ 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.

[pmid18222442-1] 1.0 ^1.1 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.

[pmid19532132-2] 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.

[pmid19345283-3] 3.0 ^3.1 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.

[pmid16843735-4] 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.

[pmid15545176-5] 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.

[pmid15632697-6] 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.

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