Beyond the Sausage-Shaped Pancreas: Recognizing the Extended Spectrum of Autoimmune Pancreatitis (AIP)

Beyond the Sausage-Shaped Pancreas: Recognizing the Extended Spectrum of Autoimmune Pancreatitis (AIP)

Abstracts T1197 Beyond the Sausage-Shaped Pancreas: Recognizing the Extended Spectrum of Autoimmune Pancreatitis (AIP) Suresh T. Chari, Thomas C. Smy...

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Abstracts

T1197 Beyond the Sausage-Shaped Pancreas: Recognizing the Extended Spectrum of Autoimmune Pancreatitis (AIP) Suresh T. Chari, Thomas C. Smyrk, Jonathan E. Clain, Michael J. Levy, Randall K. Pearson, Bret T. Petersen, Mark D. Topazian, Michael B. Farnell Background: The most frequently described presentation of AIP is that of a pancreatic mass that mimics pancreatic cancer. However, its full clinical spectrum remains unknown. We have diagnosed 25 patients with AIP since 1999, of whom 14 were diagnosed after 1/2003. The aim of our study was to review the clinical presentation of AIP. Methods: AIP was diagnosed if one of the following criteria was met a) Characteristic histologic findings of lymphoplasmacytic sclerosing pancreatitis on pancreatic resection (n Z 11) or core biopsy (n Z 4), b) Elevated serum immunoglobulins/IgG4 subclass with either i) characteristic imaging findings (sausage-shaped pancreas on CT or ERCP showing diffusely irregular, attenuated pancreatic duct) (n Z 4) or ii) Steroid-responsive biliary strictures and pancreatic mass and benign follow-up (n Z 6). The demographic, clinical, and radiological features were noted. Results: The mean (GSD) age at diagnosis of AIP was 58 G 17 years (range 17-81) and 81% were males. The presentation was with obstructive jaundice in 19, and 2 patients each presented with a pancreatic mass without jaundice, abrupt onset of pancreatic steatorrhea without diabetes, and recurrent pancreatitis. Additionally, 4 had diabetes and 1 had pancreatic calcification, pancreatic atrophy and dilated pancreatic duct. Of the 20 patients with biliary strictures, 5 (25%) had multiple intra-hepatic strictures and 15 (75%) had distal CBD strictures. Obstructive jaundice was the presentation in 11/11 patients diagnosed before 2003 vs 8/14 diagnosed after 1/2003 (p Z 0.01). Surgery for patients presenting primarily to Mayo with obstructive jaundice or pancreatic mass was performed in 8/10 diagnosed before 2003 vs 1/7 diagnosed since 1/2003 (p Z 0.007). All 6 patients treated with steroids showed resolution of pancreatic mass and biliary strictures. In patients with spontaneous or steroid-induced remission, the pancreas on follow-up appeared ‘‘normal’’ in size and eventually looked atrophic. In the patients with steatorrhea, the pancreas on CT appeared normal or atrophic in size. Conclusion: AIP can present as pancreatic mass with or without obstructive jaundice, acute pancreatitis, chronic pancreatitis and pancreatic insufficiency. In AIP the pancreas on CT can show diffuse enlargement or localized mass, be normal-sized or be atrophic with calcification. With a high index of suspicion and awareness of its extended clinical spectrum, AIP can be diagnosed prospectively.

patients who had not undergone prior endoscopic treatments. There were only 6 episodes (3.7%) of PEP in 162 cases undergoing manometry during the first 6 months of 2004.

Conclusion: These data confirm that sphincter manometry can be performed with a low risk of pancreatitis, using temporary pancreatic stents.

T1199 ERCP/Manometry Findings in 1,108 Idiopathic Pancreatitis Patients Monika Fischer, Evan L. Fogel, Lee McHenry, Stuart Sherman, James L. Watkins, Suzette Schmidt, Glen A. Lehman

T1198 The Incidence of Pancreatitis After ERCP/Manometry Has Fallen with the Increasing Use of Temporary Pancreatic Stents Experience of 2861 Patients Over 10 Years Peter Cotton, Patrick D. Mauldin, Joseph Romagnuolo, Robert H. Hawes There is increasing evidence that temporary pancreatic stenting reduces the incidence of post-ERCP pancreatitis (PEP) in patients at increased risk, such as those undergoing sphincter manometry. We have analysed the use of pancreatic stents and the pancreatitis rates for patients having manometry in our unit. Data were derived from the GITrac database, into which all procedures and their outcomes have been entered prospectively. Pancreatitis (and severity levels) was defined by established consensus criteria. Over a period of 10 years, 2861 patients underwent biliary and/or pancreatic manometry. Most underwent both, as well as sphincteromy based on the results. The overall rate of PEP in 1481 patients without stents was 8.1%, which was significantly higher (p Z 0.002) than the incidence (5.3%; 95% CI: 4.2-6.6%) in 1380 patients with stents. The odds ratio for PEP with stents was 0.63 (95% CI: 0.5-0.9). There was a trend to more episodes of moderate and severe pancreatitis in patients without stents (2.0%), compared to 1.3% in those with stents (p Z NS). The progressive increase in the proportion of patients receiving stents correlated (Pearson coefficient 0.840) with a progressive decrease in the rate of pancreatitis (figure). The data were comparable in the 1790

AB190 GASTROINTESTINAL ENDOSCOPY Volume 61, No. 5 : 2005

Background: ERCP with sphincter of Oddi manometry can detect abnormalities in patients otherwise classified as idiopathic pancreatitis. Methods: All patients undergoing ERCP at Indiana University Medical Center have data prospectively entered into a database. 1,301 patients were classified initially as idiopathic pancreatitis between 1994-2004. After H&P, review of imaging results, and prior endoscopies, 194 were eliminated for known causes of pancreatitis. This study reviews the findings of ERCP with or without manometry of the remaining 1108 patients. Results: Pre-ERCP diagnosis were acute pancreatitis 93 (8.4%), acute recurrent pancreatitis 524 (47.3%), chronic pancreatitis 178 (16.1%), unresolving pancreatitis 4 (0.4%), pancreatitis not otherwise specified 309 (27.9%). Post ERCP diagnoses were: sphincter of Oddi dysfunction (basal sphincter pressure R40 mmHg) in 445 (40.2%), pancreas divisum in 208 (19%), mucinous tumor in 45 (4.1%), choledocholithiasis in 32 (2.9%), cholelithiasis in 26 (2.3%), periampullary diverticulum in 68 (6.2%), PSC in 5 (0.5%), choledochal cyst 4 (0.4%), anomalous pancreatobiliary junction 7 (0.6%). Chronic pancreatitis (CP) was diagnosed in 405 (36.5%). Of these, mild CP 105 (9.5%), moderate CP 53 (4.8%), severe 81 (7.3%), CP not otherwise specified 122 (11%). Manometry was performed in 787 (71.0%) patients. Biliary sphincter basal pressures were elevated in 212 (19.1%). Pancreatic basal pressures were elevated in the major papilla in 362 (32.7%). 803 (72.7%) patients underwent a therapeutic intervention at ERCP. All findings (ERCP C manometry) were normal in 121(11%) of patients. 97 patients had elevated liver serum chemistries!2x the upper limit of normal; of these, 29 (30%) had SOD and 31(32%) had cholelithiasis or choledocholithiasis. Mucinous tumors were identified in 52 patients. Frequency of mucinous tumors by age is25-40: 4/305 (1.3%), 41-50: 11/282 (3.9%), 51-60: 9/250 (3.6%), 61-70: 11/154 (7.1%), O70: 17/149 (11.4%). Summary: In our large series of idiopathic pancreatitis patients, ERCP and manometry frequently detects abnormalities that are directly treatable. Manometry abnormalities are the most common treatable cause of idiopathic pancreatitis. Conclusion: ERCP with sphincter of Oddi of manometry is indicated for most patients with idiopathic pancreatitis.

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