OC-002 Do steroids improve the long-term outcome of autoimmune pancreatitis

IntroductionImprovement in jaundice and pancreatic enlargement with prednisolone is a reliable and characteristic feature of autoimmune pancreatitis (AIP). The aim of this study was to investigate whether immunosuppression results in long-term preservation of pancreatic structure and function, as th...

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Veröffentlicht in:Gut 2010-04, Vol.59 (Suppl 1), p.A1-A1
Hauptverfasser: Sandanayake, N S, Chapman, M H, Kalaitzakis, E, Amin, Z, Novelli, M, Winstanley, A, Rodriguez-Justo, M, Hatfield, A R, Pereira, S P, Webster, G J
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container_end_page A1
container_issue Suppl 1
container_start_page A1
container_title Gut
container_volume 59
creator Sandanayake, N S
Chapman, M H
Kalaitzakis, E
Amin, Z
Novelli, M
Winstanley, A
Rodriguez-Justo, M
Hatfield, A R
Pereira, S P
Webster, G J
description IntroductionImprovement in jaundice and pancreatic enlargement with prednisolone is a reliable and characteristic feature of autoimmune pancreatitis (AIP). The aim of this study was to investigate whether immunosuppression results in long-term preservation of pancreatic structure and function, as this has not been well defined.MethodsWe report a single-centre cohort study of all patients diagnosed with AIP from 2004 to 2009. Diagnosis was based on the HISORt criteria, while assessment of pancreatic morphology was made pre and post-treatment, by CT and/or MRI, according to clinical need. Exocrine and endocrine function was measured by stool faecal elastase 1 (FE1) and plasma glucose/HbA1c.ResultsForty-one patients (33M/F8; median age 62 years, range 29–83) were included. Median follow-up to date is 35 months (range 1–71). At diagnosis, a pancreatic mass/diffuse swelling was noted in 36/41 (88%), serum IgG4 was elevated (>1.3 g/l) in 22/41 (54%), and 30/41 (73%) had an immunohistological diagnosis, with >10 IgG4-positive plasma cells/high power field (n=15 pancreas, n=8 liver, n=8 ampulla, n=2 salivary gland, n=2 renal). 30/41 (73%) had IgG4-associated cholangitis, 14/41 (34%) had other organ involvement (n=7 renal, n=3 salivary gland, n=2 retroperitoneal fibrosis, n=2 neurological). Seven (17%) of 41 patients had previously undergone pancreatobiliary surgery for presumed pancreatic cancer (n=3 Whipple resection, n=4 biliary bypass). 38/41 (93%) patients were treated with a tapering oral prednisolone regimen, which was ceased at a median of 6 months (range 2.5–70.9 months). An initial clinical, radiological, and biochemical response was seen in all patients. Relapse following disease remission or failure to wean steroids occurred in 19/41 (46%), and of these patients 6/19 were treated with steroids, and 13/19 were treated with prednisolone 30 mg and azathioprine 2 mg/kg daily. Post-steroid pancreatic atrophy was seen in 23/41 (56%). Low FE1 (
doi_str_mv 10.1136/gut.2009.208934b
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The aim of this study was to investigate whether immunosuppression results in long-term preservation of pancreatic structure and function, as this has not been well defined.MethodsWe report a single-centre cohort study of all patients diagnosed with AIP from 2004 to 2009. Diagnosis was based on the HISORt criteria, while assessment of pancreatic morphology was made pre and post-treatment, by CT and/or MRI, according to clinical need. Exocrine and endocrine function was measured by stool faecal elastase 1 (FE1) and plasma glucose/HbA1c.ResultsForty-one patients (33M/F8; median age 62 years, range 29–83) were included. Median follow-up to date is 35 months (range 1–71). At diagnosis, a pancreatic mass/diffuse swelling was noted in 36/41 (88%), serum IgG4 was elevated (&gt;1.3 g/l) in 22/41 (54%), and 30/41 (73%) had an immunohistological diagnosis, with &gt;10 IgG4-positive plasma cells/high power field (n=15 pancreas, n=8 liver, n=8 ampulla, n=2 salivary gland, n=2 renal). 30/41 (73%) had IgG4-associated cholangitis, 14/41 (34%) had other organ involvement (n=7 renal, n=3 salivary gland, n=2 retroperitoneal fibrosis, n=2 neurological). Seven (17%) of 41 patients had previously undergone pancreatobiliary surgery for presumed pancreatic cancer (n=3 Whipple resection, n=4 biliary bypass). 38/41 (93%) patients were treated with a tapering oral prednisolone regimen, which was ceased at a median of 6 months (range 2.5–70.9 months). An initial clinical, radiological, and biochemical response was seen in all patients. Relapse following disease remission or failure to wean steroids occurred in 19/41 (46%), and of these patients 6/19 were treated with steroids, and 13/19 were treated with prednisolone 30 mg and azathioprine 2 mg/kg daily. Post-steroid pancreatic atrophy was seen in 23/41 (56%). Low FE1 (&lt;200 μg/g of stool) was noted in 23/41 (56%). 15/41 (37%) patients were diabetic, while 9/41 (22%) have reduced bone mineral density (n=5 osteopaenia, n=4 osteoporosis). Three patients have died during follow-up (n=1 liver failure, n=1 encephalitis, n=1 cholangiocarcinoma).ConclusionWhile a favourable initial response to steroids is predictable in patients with a definitive diagnosis of AIP, loss of pancreatic structure and function is common, and the disease is associated with long-term morbidity and mortality. It will be difficult to clearly define the impact of treatment on the outcome of pancreatic and extra-pancreatic disease without a placebo controlled trial.</description><identifier>ISSN: 0017-5749</identifier><identifier>EISSN: 1468-3288</identifier><identifier>DOI: 10.1136/gut.2009.208934b</identifier><language>eng</language><publisher>London: BMJ Publishing Group LTD</publisher><subject>Atrophy ; Azathioprine ; Bone mineral density ; Cholangiocarcinoma ; Cholangitis ; Diabetes mellitus ; Diagnosis ; Elastase ; Encephalitis ; Fibrosis ; Immunoglobulin G ; Immunosuppression ; Jaundice ; Liver diseases ; Magnetic resonance imaging ; Morbidity ; Osteoporosis ; Pancreatic cancer ; Pancreatic diseases ; Pancreatitis ; Patients ; Plasma cells ; Salivary gland ; Steroid hormones ; Steroids ; Surgery</subject><ispartof>Gut, 2010-04, Vol.59 (Suppl 1), p.A1-A1</ispartof><rights>2010, Published by the BMJ Publishing Group Limited For permission to use, (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><rights>Copyright: 2010 © 2010, Published by the BMJ Publishing Group Limited For permission to use, (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttp://gut.bmj.com/content/59/Suppl_1/A1.2.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttp://gut.bmj.com/content/59/Suppl_1/A1.2.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,314,780,784,3196,23571,27924,27925,77600,77631</link.rule.ids></links><search><creatorcontrib>Sandanayake, N S</creatorcontrib><creatorcontrib>Chapman, M H</creatorcontrib><creatorcontrib>Kalaitzakis, E</creatorcontrib><creatorcontrib>Amin, Z</creatorcontrib><creatorcontrib>Novelli, M</creatorcontrib><creatorcontrib>Winstanley, A</creatorcontrib><creatorcontrib>Rodriguez-Justo, M</creatorcontrib><creatorcontrib>Hatfield, A R</creatorcontrib><creatorcontrib>Pereira, S P</creatorcontrib><creatorcontrib>Webster, G J</creatorcontrib><title>OC-002 Do steroids improve the long-term outcome of autoimmune pancreatitis</title><title>Gut</title><description>IntroductionImprovement in jaundice and pancreatic enlargement with prednisolone is a reliable and characteristic feature of autoimmune pancreatitis (AIP). The aim of this study was to investigate whether immunosuppression results in long-term preservation of pancreatic structure and function, as this has not been well defined.MethodsWe report a single-centre cohort study of all patients diagnosed with AIP from 2004 to 2009. Diagnosis was based on the HISORt criteria, while assessment of pancreatic morphology was made pre and post-treatment, by CT and/or MRI, according to clinical need. Exocrine and endocrine function was measured by stool faecal elastase 1 (FE1) and plasma glucose/HbA1c.ResultsForty-one patients (33M/F8; median age 62 years, range 29–83) were included. Median follow-up to date is 35 months (range 1–71). At diagnosis, a pancreatic mass/diffuse swelling was noted in 36/41 (88%), serum IgG4 was elevated (&gt;1.3 g/l) in 22/41 (54%), and 30/41 (73%) had an immunohistological diagnosis, with &gt;10 IgG4-positive plasma cells/high power field (n=15 pancreas, n=8 liver, n=8 ampulla, n=2 salivary gland, n=2 renal). 30/41 (73%) had IgG4-associated cholangitis, 14/41 (34%) had other organ involvement (n=7 renal, n=3 salivary gland, n=2 retroperitoneal fibrosis, n=2 neurological). Seven (17%) of 41 patients had previously undergone pancreatobiliary surgery for presumed pancreatic cancer (n=3 Whipple resection, n=4 biliary bypass). 38/41 (93%) patients were treated with a tapering oral prednisolone regimen, which was ceased at a median of 6 months (range 2.5–70.9 months). An initial clinical, radiological, and biochemical response was seen in all patients. Relapse following disease remission or failure to wean steroids occurred in 19/41 (46%), and of these patients 6/19 were treated with steroids, and 13/19 were treated with prednisolone 30 mg and azathioprine 2 mg/kg daily. Post-steroid pancreatic atrophy was seen in 23/41 (56%). Low FE1 (&lt;200 μg/g of stool) was noted in 23/41 (56%). 15/41 (37%) patients were diabetic, while 9/41 (22%) have reduced bone mineral density (n=5 osteopaenia, n=4 osteoporosis). Three patients have died during follow-up (n=1 liver failure, n=1 encephalitis, n=1 cholangiocarcinoma).ConclusionWhile a favourable initial response to steroids is predictable in patients with a definitive diagnosis of AIP, loss of pancreatic structure and function is common, and the disease is associated with long-term morbidity and mortality. It will be difficult to clearly define the impact of treatment on the outcome of pancreatic and extra-pancreatic disease without a placebo controlled trial.</description><subject>Atrophy</subject><subject>Azathioprine</subject><subject>Bone mineral density</subject><subject>Cholangiocarcinoma</subject><subject>Cholangitis</subject><subject>Diabetes mellitus</subject><subject>Diagnosis</subject><subject>Elastase</subject><subject>Encephalitis</subject><subject>Fibrosis</subject><subject>Immunoglobulin G</subject><subject>Immunosuppression</subject><subject>Jaundice</subject><subject>Liver diseases</subject><subject>Magnetic resonance imaging</subject><subject>Morbidity</subject><subject>Osteoporosis</subject><subject>Pancreatic cancer</subject><subject>Pancreatic diseases</subject><subject>Pancreatitis</subject><subject>Patients</subject><subject>Plasma cells</subject><subject>Salivary gland</subject><subject>Steroid hormones</subject><subject>Steroids</subject><subject>Surgery</subject><issn>0017-5749</issn><issn>1468-3288</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNqFkD9PwzAQxS0EEqWwM1piRC7n2GnsEZW_UlEXmC3HsUuqJg62g8TGwhflk-Cq3Vnuhnvv7t0PoUsKM0rZ_GY9plkBIHMRkvH6CE0onwvCCiGO0QSAVqSsuDxFZzFuAEAISSfoZbUgAMXv98-dxzHZ4Nsm4rYbgv-0OL1bvPX9muRBh_2YjO8s9g7rMfm268be4kH3Jlid2tTGc3Ti9Dbai0OforeH-9fFE1muHp8Xt0tSUygFMQ23tbScFcwUjaGydsY1hjcNVKWpqxzfFQacdhIqXUvgleCMl5AfdNxqNkVX-7055sdoY1IbP4Y-n1QFcMayWIqsgr3KBB9jsE4Noe10-FIU1A6aytDUDpo6QMuW672l7jb_q_8AiDtuvA</recordid><startdate>201004</startdate><enddate>201004</enddate><creator>Sandanayake, N S</creator><creator>Chapman, M H</creator><creator>Kalaitzakis, E</creator><creator>Amin, Z</creator><creator>Novelli, M</creator><creator>Winstanley, A</creator><creator>Rodriguez-Justo, M</creator><creator>Hatfield, A R</creator><creator>Pereira, S P</creator><creator>Webster, G J</creator><general>BMJ Publishing Group LTD</general><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88I</scope><scope>8AF</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M1P</scope><scope>M2P</scope><scope>M7P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>Q9U</scope></search><sort><creationdate>201004</creationdate><title>OC-002 Do steroids improve the long-term outcome of autoimmune pancreatitis</title><author>Sandanayake, N S ; Chapman, M H ; Kalaitzakis, E ; Amin, Z ; Novelli, M ; Winstanley, A ; Rodriguez-Justo, M ; Hatfield, A R ; Pereira, S P ; Webster, G J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b1058-cd4eb9e4323c2dc19bfcfdc4dd075cb734bf2c0faf907ab9047843450200f4ea3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2010</creationdate><topic>Atrophy</topic><topic>Azathioprine</topic><topic>Bone mineral density</topic><topic>Cholangiocarcinoma</topic><topic>Cholangitis</topic><topic>Diabetes mellitus</topic><topic>Diagnosis</topic><topic>Elastase</topic><topic>Encephalitis</topic><topic>Fibrosis</topic><topic>Immunoglobulin G</topic><topic>Immunosuppression</topic><topic>Jaundice</topic><topic>Liver diseases</topic><topic>Magnetic resonance imaging</topic><topic>Morbidity</topic><topic>Osteoporosis</topic><topic>Pancreatic cancer</topic><topic>Pancreatic diseases</topic><topic>Pancreatitis</topic><topic>Patients</topic><topic>Plasma cells</topic><topic>Salivary gland</topic><topic>Steroid hormones</topic><topic>Steroids</topic><topic>Surgery</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sandanayake, N S</creatorcontrib><creatorcontrib>Chapman, M H</creatorcontrib><creatorcontrib>Kalaitzakis, E</creatorcontrib><creatorcontrib>Amin, Z</creatorcontrib><creatorcontrib>Novelli, M</creatorcontrib><creatorcontrib>Winstanley, A</creatorcontrib><creatorcontrib>Rodriguez-Justo, M</creatorcontrib><creatorcontrib>Hatfield, A R</creatorcontrib><creatorcontrib>Pereira, S P</creatorcontrib><creatorcontrib>Webster, G J</creatorcontrib><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health &amp; 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Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health &amp; Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Science Database</collection><collection>Biological Science Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central Basic</collection><jtitle>Gut</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sandanayake, N S</au><au>Chapman, M H</au><au>Kalaitzakis, E</au><au>Amin, Z</au><au>Novelli, M</au><au>Winstanley, A</au><au>Rodriguez-Justo, M</au><au>Hatfield, A R</au><au>Pereira, S P</au><au>Webster, G J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>OC-002 Do steroids improve the long-term outcome of autoimmune pancreatitis</atitle><jtitle>Gut</jtitle><date>2010-04</date><risdate>2010</risdate><volume>59</volume><issue>Suppl 1</issue><spage>A1</spage><epage>A1</epage><pages>A1-A1</pages><issn>0017-5749</issn><eissn>1468-3288</eissn><abstract>IntroductionImprovement in jaundice and pancreatic enlargement with prednisolone is a reliable and characteristic feature of autoimmune pancreatitis (AIP). The aim of this study was to investigate whether immunosuppression results in long-term preservation of pancreatic structure and function, as this has not been well defined.MethodsWe report a single-centre cohort study of all patients diagnosed with AIP from 2004 to 2009. Diagnosis was based on the HISORt criteria, while assessment of pancreatic morphology was made pre and post-treatment, by CT and/or MRI, according to clinical need. Exocrine and endocrine function was measured by stool faecal elastase 1 (FE1) and plasma glucose/HbA1c.ResultsForty-one patients (33M/F8; median age 62 years, range 29–83) were included. Median follow-up to date is 35 months (range 1–71). At diagnosis, a pancreatic mass/diffuse swelling was noted in 36/41 (88%), serum IgG4 was elevated (&gt;1.3 g/l) in 22/41 (54%), and 30/41 (73%) had an immunohistological diagnosis, with &gt;10 IgG4-positive plasma cells/high power field (n=15 pancreas, n=8 liver, n=8 ampulla, n=2 salivary gland, n=2 renal). 30/41 (73%) had IgG4-associated cholangitis, 14/41 (34%) had other organ involvement (n=7 renal, n=3 salivary gland, n=2 retroperitoneal fibrosis, n=2 neurological). Seven (17%) of 41 patients had previously undergone pancreatobiliary surgery for presumed pancreatic cancer (n=3 Whipple resection, n=4 biliary bypass). 38/41 (93%) patients were treated with a tapering oral prednisolone regimen, which was ceased at a median of 6 months (range 2.5–70.9 months). An initial clinical, radiological, and biochemical response was seen in all patients. Relapse following disease remission or failure to wean steroids occurred in 19/41 (46%), and of these patients 6/19 were treated with steroids, and 13/19 were treated with prednisolone 30 mg and azathioprine 2 mg/kg daily. Post-steroid pancreatic atrophy was seen in 23/41 (56%). Low FE1 (&lt;200 μg/g of stool) was noted in 23/41 (56%). 15/41 (37%) patients were diabetic, while 9/41 (22%) have reduced bone mineral density (n=5 osteopaenia, n=4 osteoporosis). Three patients have died during follow-up (n=1 liver failure, n=1 encephalitis, n=1 cholangiocarcinoma).ConclusionWhile a favourable initial response to steroids is predictable in patients with a definitive diagnosis of AIP, loss of pancreatic structure and function is common, and the disease is associated with long-term morbidity and mortality. It will be difficult to clearly define the impact of treatment on the outcome of pancreatic and extra-pancreatic disease without a placebo controlled trial.</abstract><cop>London</cop><pub>BMJ Publishing Group LTD</pub><doi>10.1136/gut.2009.208934b</doi></addata></record>
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subjects Atrophy
Azathioprine
Bone mineral density
Cholangiocarcinoma
Cholangitis
Diabetes mellitus
Diagnosis
Elastase
Encephalitis
Fibrosis
Immunoglobulin G
Immunosuppression
Jaundice
Liver diseases
Magnetic resonance imaging
Morbidity
Osteoporosis
Pancreatic cancer
Pancreatic diseases
Pancreatitis
Patients
Plasma cells
Salivary gland
Steroid hormones
Steroids
Surgery
title OC-002 Do steroids improve the long-term outcome of autoimmune pancreatitis
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