Autoimmune pancreatitis natural history, complications and prognosis: Difference between revisions

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==Overview==
==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 [[Calcification|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, Complications, and Prognosis==


===Natural History===
===Natural History===
*The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.  
*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]].  
*The symptoms of (disease name) typically develop ___ years after exposure to ___.  
*If left untreated, patients with autoimmune pancreatitis may progress to develop [[pancreatic insufficiency]], [[diabetes]], and pancreatic [[Calcification|calcifications]] or stones.
*If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].


===Complications===
===Complications===
*Common complications of autoimmune pancreatitis may include:
*Common complications of autoimmune pancreatitis include:
**Pancreatic insufficiency
**[[Pancreatic insufficiency]]
**Diabetes
**[[Diabetes]]
**Pancreatic calcifications or stones
**Pancreatic [[Calcification|calcifications]] or stones


===Prognosis===
===Prognosis===
* The prognosis is usually good and following responses have been observed in response to corticosteroid therapy:
* 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.<ref name="pmid18222442">{{cite journal |vauthors=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 |title=Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy |journal=Gastroenterology |volume=134 |issue=3 |pages=706–15 |year=2008 |pmid=18222442 |doi=10.1053/j.gastro.2007.12.009 |url=}}</ref><ref name="pmid19532132">{{cite journal |vauthors=Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Whitcomb DC, Slivka A |title=Evaluation and management of autoimmune pancreatitis: experience at a large US center |journal=Am. J. Gastroenterol. |volume=104 |issue=9 |pages=2295–306 |year=2009 |pmid=19532132 |doi=10.1038/ajg.2009.325 |url=}}</ref><ref name="pmid19345283">{{cite journal |vauthors=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 |title=Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis |journal=Clin. Gastroenterol. Hepatol. |volume=7 |issue=10 |pages=1089–96 |year=2009 |pmid=19345283 |doi=10.1016/j.cgh.2009.03.021 |url=}}</ref>
**About 2/3rd of patients show good response to [[glucocorticoids]] with complete recovery.<ref name="pmid18222442">{{cite journal |vauthors=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 |title=Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy |journal=Gastroenterology |volume=134 |issue=3 |pages=706–15 |year=2008 |pmid=18222442 |doi=10.1053/j.gastro.2007.12.009 |url=}}</ref><ref name="pmid19532132">{{cite journal |vauthors=Raina A, Yadav D, Krasinskas AM, McGrath KM, Khalid A, Sanders M, Whitcomb DC, Slivka A |title=Evaluation and management of autoimmune pancreatitis: experience at a large US center |journal=Am. J. Gastroenterol. |volume=104 |issue=9 |pages=2295–306 |year=2009 |pmid=19532132 |doi=10.1038/ajg.2009.325 |url=}}</ref><ref name="pmid19345283">{{cite journal |vauthors=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 |title=Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis |journal=Clin. Gastroenterol. Hepatol. |volume=7 |issue=10 |pages=1089–96 |year=2009 |pmid=19345283 |doi=10.1016/j.cgh.2009.03.021 |url=}}</ref>
***Approximately 25% may require a second course of [[glucocorticoids]].
**Approximately 25% may require a second course of [[glucocorticoids]].
***A few patients with autoimmune pancreatitis may require continuous treatment
**A few patients with autoimmune pancreatitis may require continuous treatment.
* Patients with biliary strictures have a variable response to [[glucocorticoids]] such as:<ref name="pmid18222442">{{cite journal |vauthors=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 |title=Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy |journal=Gastroenterology |volume=134 |issue=3 |pages=706–15 |year=2008 |pmid=18222442 |doi=10.1053/j.gastro.2007.12.009 |url=}}</ref><ref name="pmid19345283">{{cite journal |vauthors=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 |title=Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis |journal=Clin. Gastroenterol. Hepatol. |volume=7 |issue=10 |pages=1089–96 |year=2009 |pmid=19345283 |doi=10.1016/j.cgh.2009.03.021 |url=}}</ref><ref name="pmid16843735">{{cite journal |vauthors=Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, Clain JE, Pearson RK, Petersen BT, Vege SS, Farnell MB |title=Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience |journal=Clin. Gastroenterol. Hepatol. |volume=4 |issue=8 |pages=1010–6; quiz 934 |year=2006 |pmid=16843735 |doi=10.1016/j.cgh.2006.05.017 |url=}}</ref><ref name="pmid15545176">{{cite journal |vauthors=Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A |title=Morphological changes after steroid therapy in autoimmune pancreatitis |journal=Scand. J. Gastroenterol. |volume=39 |issue=11 |pages=1154–8 |year=2004 |pmid=15545176 |doi=10.1080/00365520410008033 |url=}}</ref><ref name="pmid15632697">{{cite journal |vauthors=Wakabayashi T, Kawaura Y, Satomura Y, Watanabe H, Motoo Y, Sawabu N |title=Long-term prognosis of duct-narrowing chronic pancreatitis: strategy for steroid treatment |journal=Pancreas |volume=30 |issue=1 |pages=31–9 |year=2005 |pmid=15632697 |doi= |url=}}</ref>
** 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==
==References==

Latest revision as of 15:43, 18 January 2018

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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

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

Complications

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]

References

  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.
  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.
  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.
  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.
  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.
  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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