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'''For the WikiDoc page for this topic, click [[xyz|here]]''' | '''For the WikiDoc page for this topic, click [[xyz|here]]''' | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} {{IQ}} | ||
==Overview== | ==Overview== | ||
Autoimmune pancreatitis is a rare form of [[chronic pancreatitis]] and is also known as primary [[inflammatory]] [[pancreatitis]], lymphoplasmacytic sclerosing pancreatitis, pseudotumorous pancreatitis, [[chronic pancreatitis]] with irregular narrowing of the main [[pancreatic duct]], idiopathic chronic pancreatitis, and nonalcoholic duct destructive chronic pancreatitis. Autoimmune pancreatitis may be classified into two types; Type 1 AIP and Type 2 AIP or idiopathic duct-centric pancreatitis. | |||
==What are the Symptoms of | ==What are the Symptoms of Autoimmune pancreatitis?== | ||
Patients with autoimmune pancreatitis may do not have a positive history of [[alcohol]] abuse. Common symptoms of autoimmune pancreatitis include mild abdominal pain, abdominal mass/[[pancreatic]] mass, painless [[jaundice]], [[weight loss]], and [[diabetes mellitus]]. Less common symptoms may include symptoms related to other autoimmune diseases such as [[Sjögren's syndrome]], [[Primary sclerosing cholangitis|primary sclerosing cholangitis (PSC)]], [[inflammatory bowel disease]] (IBD), and [[retroperitoneal fibrosis]]. Autoimmune pancreatitis may have extra-pancreatic involvement such as [[bile duct]], [[salivary gland]], [[retroperitoneum]], [[kidneys]], and [[orbit]] depending upon the various stages of autoimmune pancreatitis. | |||
==What Causes | ==What Causes Autoimmune pancreatitis?== | ||
Autoimmune pancreatitis is [[idiopathic]] in origin and has no clear etiology. Autoimmune pancreatitis is thought to be due to some [[autoimmune]] reaction against [[pancreas]] and might be associated with other [[autoimmune diseases]]. | |||
== | == Diagnosis == | ||
* Laboratory findings consistent with the diagnosis of autoimmune pancreatitis may include increased [[serum]] IgG4 levels and [[hypergammaglobulinemia]] (>2 times the upper limit of normal in most patients), antilactoferrin antibody, anticarbonic anhydrase II antibody, other [[autoantibodies]] (ANA), [[rheumatoid factor]] (RF), IgG4-positive plasma cells, elevated [[serum]] [[alkaline phosphatase]] levels (ALP), elevated [[serum]] [[aminotransferases]], [[ESR]], and [[CA19-9]]. | |||
* [[Endoscopic ultrasound]] guided fine needle aspiration (EUS-FNA) may be used to obtain [[histologic]] specimens from [[pancreas]]. | |||
* [[CT scan]] findings suggestive of autoimmune pancreatitis may include diffusely enlarged [[Pancreas|pancrea]]<nowiki/>s with featureless borders, delayed enhancement with or without a capsule-like rim and rarely [[Calcification|calcifications]] may be seen. | |||
* [[Endoscopic retrograde cholangiopancreatography|ERCP]] or [[Magnetic resonance cholangiopancreatography|MRCP]] findings in autoimmune pancreatitis may include a narrowed main and dorsal [[pancreatic duct]], [[diffuse]] and irregular narrowing of the [[pancreatic duct]] (beaded appearance), focal stricture of the [[pancreatic duct]], [[proximal]] or [[distal]] [[common bile duct]] and irregular narrowing of the intrahepatic ducts. | |||
* Other diagnostic studies for autoimmune pancreatitis usually include [[pancreatic]] [[biopsy]], which may demonstrate extensive [[fibrosis]]. [[Endoscopic]] ultrasound-guided trucut biopsy (EUS-TCB) is usually avoided due to increased risk of complications and limited diagnostic ability. | |||
==Treatment Options== | ==Treatment Options== | ||
==Where to find Medical Care for | ===== Medical Therapy: ===== | ||
[[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. [[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]]. | |||
===== Surgical Management: ===== | |||
Surgery is usually considered when pain management fails with medical and [[endoscopic]] therapies. The goals of surgery include effective pain relief, and to preserve long-term pancreatic function. Surgery for chronic pancreatitis tends to be divided into two areas - resectional and drainage procedures. Dilated [[pancreatic duct]] may be managed with [[lateral]] pancreaticojejunostomy (LPJ) and [[lateral]]<nowiki/>pancreaticojejunostomy with localized pancreatic head resection. Nondilated [[pancreatic duct]] is usually managed with [[pancreaticoduodenectomy]], duodenal-preserving pancreatic head resection (DPPHR), distal [[pancreatectomy]] (DP) and total [[pancreatectomy]] (TP). | |||
==Where to find Medical Care for Autoimmune pancreatitis?== | |||
Medical care for (disease name) can be found [https://www.google.com/maps/search/hospitals/ here]. | Medical care for (disease name) can be found [https://www.google.com/maps/search/hospitals/ here]. | ||
==Prevention== | ==Prevention== | ||
Patients should be managed appropriately and timely with [[glucocorticoids]] and [[immunomodulatory]] drugs to prevent complications such as [[pancreatic insufficiency]], [[diabetes]], and pancreatic calcifications or stones. | |||
==What to Expect (Outlook/Prognosis)?== | ==What to Expect (Outlook/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.<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]]. | |||
**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" /><ref name="pmid19345283" /><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]]. | |||
==Possible Complications== | ==Possible Complications== | ||
Common complications of autoimmune pancreatitis may include: | |||
*[[Pancreatic insufficiency]] | |||
*[[Diabetes]] | |||
*Pancreatic [[Calcification|calcifications]] or stones | |||
==Sources== | ==Sources== | ||
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{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} | ||
<references /> | |||
Latest revision as of 21:46, 5 January 2018
Template:Autoimmune pancreatitis (patient information)
For the WikiDoc page for this topic, click here
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]
Overview
Autoimmune pancreatitis is a rare form of chronic pancreatitis and is also known as primary inflammatory pancreatitis, lymphoplasmacytic sclerosing pancreatitis, pseudotumorous pancreatitis, chronic pancreatitis with irregular narrowing of the main pancreatic duct, idiopathic chronic pancreatitis, and nonalcoholic duct destructive chronic pancreatitis. Autoimmune pancreatitis may be classified into two types; Type 1 AIP and Type 2 AIP or idiopathic duct-centric pancreatitis.
What are the Symptoms of Autoimmune pancreatitis?
Patients with autoimmune pancreatitis may do not have a positive history of alcohol abuse. Common symptoms of autoimmune pancreatitis include mild abdominal pain, abdominal mass/pancreatic mass, painless jaundice, weight loss, and diabetes mellitus. Less common symptoms may include symptoms related to other autoimmune diseases such as Sjögren's syndrome, primary sclerosing cholangitis (PSC), inflammatory bowel disease (IBD), and retroperitoneal fibrosis. Autoimmune pancreatitis may have extra-pancreatic involvement such as bile duct, salivary gland, retroperitoneum, kidneys, and orbit depending upon the various stages of autoimmune pancreatitis.
What Causes Autoimmune pancreatitis?
Autoimmune pancreatitis is idiopathic in origin and has no clear etiology. Autoimmune pancreatitis is thought to be due to some autoimmune reaction against pancreas and might be associated with other autoimmune diseases.
Diagnosis
- Laboratory findings consistent with the diagnosis of autoimmune pancreatitis may include increased serum IgG4 levels and hypergammaglobulinemia (>2 times the upper limit of normal in most patients), antilactoferrin antibody, anticarbonic anhydrase II antibody, other autoantibodies (ANA), rheumatoid factor (RF), IgG4-positive plasma cells, elevated serum alkaline phosphatase levels (ALP), elevated serum aminotransferases, ESR, and CA19-9.
- Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) may be used to obtain histologic specimens from pancreas.
- CT scan findings suggestive of autoimmune pancreatitis may include diffusely enlarged pancreas with featureless borders, delayed enhancement with or without a capsule-like rim and rarely calcifications may be seen.
- ERCP or MRCP findings in autoimmune pancreatitis may include a narrowed main and dorsal pancreatic duct, diffuse and irregular narrowing of the pancreatic duct (beaded appearance), focal stricture of the pancreatic duct, proximal or distal common bile duct and irregular narrowing of the intrahepatic ducts.
- Other diagnostic studies for autoimmune pancreatitis usually include pancreatic biopsy, which may demonstrate extensive fibrosis. Endoscopic ultrasound-guided trucut biopsy (EUS-TCB) is usually avoided due to increased risk of complications and limited diagnostic ability.
Treatment Options
Medical Therapy:
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. 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.
Surgical Management:
Surgery is usually considered when pain management fails with medical and endoscopic therapies. The goals of surgery include effective pain relief, and to preserve long-term pancreatic function. Surgery for chronic pancreatitis tends to be divided into two areas - resectional and drainage procedures. Dilated pancreatic duct may be managed with lateral pancreaticojejunostomy (LPJ) and lateralpancreaticojejunostomy with localized pancreatic head resection. Nondilated pancreatic duct is usually managed with pancreaticoduodenectomy, duodenal-preserving pancreatic head resection (DPPHR), distal pancreatectomy (DP) and total pancreatectomy (TP).
Where to find Medical Care for Autoimmune pancreatitis?
Medical care for (disease name) can be found here.
Prevention
Patients should be managed appropriately and timely with glucocorticoids and immunomodulatory drugs to prevent complications such as pancreatic insufficiency, diabetes, and pancreatic calcifications or stones.
What to Expect (Outlook/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.
Possible Complications
Common complications of autoimmune pancreatitis may include:
- Pancreatic insufficiency
- Diabetes
- Pancreatic calcifications or stones
Sources
http://www.nlm.nih.gov/medlineplus/ency/article/000434.htm
- ↑ 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.