www.worldgastroenterology.org                                                                   WORLD                     ...
Contents            Editorials                                    WDHD                                            WGO news...
Editorials           Message from the Editor                                                                           Joh...
Editorials             Message from the WGO President             Continued from cover                                    ...
Scientific news           Cancer of the exocrine pancreas                                                    René Lambert,...
Scientific news                   (1a) Incidence of Pancreas cancer: ASR (World) - Male (All ages)                        ...
Scientific news                                          Men         Women           Chile: Valdivia                4.2   ...
Scientific news             Several registries are collecting         occur predominantly in middle-aged            screen...
Scientific news           the presence of mucinous neoplasms,                 Screening and surveillance                  ...
Scientific news             Further Reading             Bae	JM,	Lee	EJ,	Guyatt	G.	Citrus	fruit	intake	   Kurtz	RC,	Ludwig	...
Scientific news           How much fluid should be given during the           initial management of acute pancreatitis?   ...
Scientific news                                                                                   Fig 2. Progression to pa...
Scientific news           Management of autoimmune pancreatitis:           a review of medical therapy                    ...
Scientific news                                                                                                      Table...
Scientific news           et al. described the course of 125            described by Kamisawa et al. [5]. Ten             ...
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
Message from the WGO President
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Message from the WGO President

  1. 1. www.worldgastroenterology.org WORLD GASTROENTEROLOGY NEWSOfficial newsletter of the World Gastroenterology Organisation Vol.14, Issue 1 Message from the WGO President Eamonn M.M. Quigley, MD President, World Gastroenterology Organisation; Professor of Medicine and Human Physiology, Alimentary Pharmabiotic Center, University College Cork, Cork, Ireland These are strange and difficult times— that the World Gastroenterology manner. World Digestive Health Day the entire world is facing considerable Organisation (WGO) has chosen irritable on 29 May 2009, and indeed the financial challenges, while the issues bowel syndrome (IBS) as its theme for entire year, will bring IBS into rightful of conflict, strife, starvation, and World Digestive Health Day 2009. This prominence and will hopefully promote climate change have also not departed. is truly a global disorder that affects a better understanding of the condition Regrettably, health care tends to get millions worldwide, many of whom and lead to progress in diagnosis and short shrift in straitened circumstances suffer in silence. The true importance management. and, within health care, priority is often of IBS in gastroenterology, medicine, The “global crisis” presents challenges given to the more acute and “dramatic” and public health is amply and vividly to WGO. As each nation strives to illnesses, while common, important, illustrated by the tremendous response address its own economic and social debilitating, but “unexciting” disorders from national societies around the problems, global issues fade into the (among which many of the most globe to this topic. IBS has proven to gloom, and funding becomes ever common digestive disorders rank), and be a galvanizing World Digestive Health more difficult to source for programs the many patients who suffer from them, Day theme for medical practitioners, such as those that WGO supports are ignored. This is not only unfortunate patients, and the public alike. WGO will around the world (Train the Trainers, but also short-sighted, given the impact be playing a truly global role on World Training Centers, Global Guidelines, of these conditions on the young and Digestive Health Day—energizing International Digestive Cancer Alliance, active during what should be the most member societies, supporting their Outreach). It is most appropriate that productive years of their lives. activities, and generating supportive It is most appropriate, therefore, materials in a most collaborative Continued on Page 4 ThIs Issue World Digestive Health Day Cancer of the exocrine pancreas Irritable bowel syndrome: René Lambert & Robert C. Kurtz a global perspective WGO and ACG Global World Digestive Health Day Collaboration Agreement Events Around the World Amy Foxx-Orenstein
  2. 2. Contents Editorials WDHD WGO news 01 24 19 Message from the What do we know TTT Trial Design WGO President about IBS in Mexico? Workshop Eamonn M.M. Quigley Jim Toouli Max Schmulson 03 26 20 Message from WGO and ACG Current the Editor Global Collaboration challenges in John Baillie diagnosing and Agreement Amy Foxx-Orenstein treating IBS: Per Olav Vandvik Scientific news 21 30 Endoscopy workshop Interview with in Ghana 05 Professor Mark Topazian Cancer of the Zaigham Abbas: exocrine pancreas IBS in Asia René Lambert, Robert C. Kurtz 32 Personality Corner Irritable bowel 11 syndrome: How much a global perspective fluid should be 40 given during the Interview with 35 David Carr-Locke initial management Gastroenterology of acute on the Internet: pancreatitis? 42 IBS and PubMed Great mentors Nison Badalov, Scott Tenner Justus Krabshuis Steven Silvis and Jack Vennes 13 John Baillie Management GASTRO 2009 of autoimmune 43 pancreatitis: Interview with a review of 38 Tom Finn, President medical therapy GASTRO 2009 of Global Health Ashraf Saleemuddin, — the place to be Care for Procter & William R. Brugge Michael Farthing Gamble Vol.14, Issue 1 ©2009 World Gastroenterology Organisation. No part Editor John Baillie Managing editor Molly Fassbender of this publication may be reproduced, stored in a Design Milkdesign Studio retrieval system or transmitted in any form without the Editorial office WGO Executive Secretariat, Medconnect GmbH, prior permission of the copyright owner. Bruennsteinstr. 10. 81541 Munich, Germany Email info@worldgastroenterology.org
  3. 3. Editorials Message from the Editor John Baillie, B. Sc (Hons), MB, Ch.B, FRCP, FASGE, FACG Wake Forest University Health Sciences Winston-Salem, North Carolina, USA Welcome to the second electronic interest, but a concerted effort and major about IBS in Mexico.” Finally, the WGO edition of World Gastroenterology research funding will be needed to librarian, Justus Krabshuis, continues his News! Our first issue was uniformly “crack” this particular killer. Inadequate outstanding series on gastroenterology well-received. Emboldened by this intravenous hydration is the silent killer on the Internet, with an overview of how positive feedback, we are exploring in patients with severe acute pancreatitis to search intelligently for IBS-related the possibilities for web-based learning (SAP), contributing to pancreatic publications. Last, but by no means opportunities. Keep a look-out for these necrosis, kidney failure, and other least, Irritable Bowel Syndrome: A Global new sections—such as embedded systemic manifestations of the disease. Perspective presents the highlights of videos demonstrating new procedures— Dr. Scott Tenner of the State University WGO’s Global Guideline for IBS. in the upcoming issues. of New York has been a leading The year 2009 seems to be whizzing It is no accident that the Scientific advocate of aggressive intravenous by, and in the blink of an eye it will be Section of this issue of WGN has hydration in these cases. Dr. Tenner the end of the year and time for the next a distinctly pancreatic “flavor.” As and his colleague Dr. Badalov outline World Congress of Gastroenterology, to a card-carrying pancreatologist, I for us the fluid regimen that should be be held in London. In this issue of WGN, make no apologies for three articles used in cases of predicted and actual Dr. Michael Farthing assures us that on my favorite subject! Professors SAP—information that (to the best this will be an outstanding international René Lambert and Robert Kurtz have of my knowledge) has not previously meeting, with something for everyone, contributed a detailed review of tumors been promulgated in journal form. including a live endoscopy course of the exocrine pancreas. Clearly, the Finally, pancreatologist extraordinaire featuring procedures beamed in from no most vexing is adenocarcinoma of the Dr. Bill Brugge and his colleague Dr. less than three countries! pancreas, which stands almost alone Saleemuddin from the Massachusetts Under the heading “Great Mentors,” I amongst gastrointestinal malignancies General Hospital in Boston review have kicked off a little series on the role as being a cancer with little hope of cure. the hot topic of medical therapy for of mentors in gastroenterology. Mentors When so many other once-fatal cancers autoimmune pancreatitis. for young researchers and for clinical are now eminently treatable, and often We intend to have future Scientific and procedural trainees in particular are curable, why has pancreatic cancer Sections dedicated to particular becoming an endangered species—let’s remained such a predictable killer? diseases and management themes. help preserve the species by honoring Until recently, pancreatic cancer has I would welcome readers’ suggestions them. I have already received invited not been a disease attracting charitable for subjects we should look at. contributions to this series that will funding (compare it, for example, to Irritable bowel syndrome (IBS) is “big” appear in future issues of WGN, but I breast cancer, childhood leukemia this year, and in recognition of that fact, would be very happy to have more. Had and, especially, AIDS). Deaths from we are pleased to bring you four papers a great mentor, or mentors? Let’s hear pancreatic cancer among celebrities on the subject. Dr. Per Olav Vandvik, about her or him! (such as “Bonanza” actor Michael Associate Professor of Medicine at And finally, I would greatly appreciate Landon and Italian tenor Luciano the University of Oslo, discusses feedback from our readers about the Pavarotti) and news of others living with current challenges in the diagnosis and current format and content of WGN. Now the disease (e.g., U.S. Supreme Court treatment of IBS. Dr. Max Schmulson that we are electronic, (almost) anything Judge Ruth Bader Ginsberg and actor of the Universidad Nacional Autónoma is possible! Let me know what you would Patrick Swayze) have spiked public de México tells us “what we know like to see in the journal.MAY 2009 WORLD GASTROENTEROLOGY NEWS 3
  4. 4. Editorials Message from the WGO President Continued from cover much vigor and rigor as ever. Join us in these efforts! WGO has therefore established the Of course, the pinnacle of our year WGO Foundation to raise funds and will be GASTRO 2009 in London secure the future of its programs. We in November, which will represent would ask all of you, as individuals, the culmination of a remarkable members of national societies, or as collaboration between WGO, UEGF, acquaintances of funding agencies or OMED, and the British Society of philanthropists, to support the WGO Gastroenterology to present a world Foundation. Our future is your future! congress with a European flavor in Despite these challenges, WGO a British atmosphere. The excitement has been even more active: a new around GASTRO 2009 worldwide TTT program on “Trial Design” was is really palpable and—having seen most successfully presented in the the program and knowing of all the beautiful city of Dubrovnik, Croatia; work that has gone into developing it, a full TTT will take place in Santiago, including the working parties, Young Chile in September; IDCA will present Clinicians’ Program, and the various or participate in several important named lectures (distinctive features programs; two new guidelines are of world congresses)—I know that about to appear; and major equipment “this excitement is fully justified. donations were delivered to our training Come to London in November. You centers in Suva (Fiji) and Ribeirão will be richly rewarded! Prêto (Brazil). WGO has not reneged and will not renege on its mission and Eamonn M.M. Quigley will continue to pursue its goals with as WGO President 2005-2009 4 WORLD GASTROENTEROLOGY NEWS MAY 2009
  5. 5. Scientific news Cancer of the exocrine pancreas René Lambert, MD and Robert C. Kurtz, MD René Lambert, MD, IARC, Lyons, France Robert C. Kurtz, MD, Memorial Sloan-Kettering Cancer Center, New York, New York, USA Introduction group derived from the islets of as an acinar cystadenocarcinoma), Pancreatic tumors are classified Langerhans. Misclassification may and some endocrine-secreting on the basis of cell differentiation occur in these cases in the presence tumors (e.g., insulinoma, gastrinoma, and immunostaining. Cancer of the of mixed exocrine–endocrine tumors, glucagonoma). exocrine pancreas develops from ductal with combinations such as acinar– epithelial cells or from acinar cells. endocrine or acinar–ductal–endocrine. The burden of Ductal adenocarcinomas represent Tumors of the pancreas are pancreatic cancer more than 90% of all pancreatic also classified on the basis of The epidemiology and burden carcinomas. Other neoplastic their morphology—solid or cystic. of pancreatic cancer relate to ductal lesions with a variable risk Solid tumors include ductal ductal adenocarcinoma—a dismal of malignancy include areas of flat adenocarcinoma, acinar carcinoma, disease with a poor prognosis and hyperplasia in the ductal epithelium, and borderline lesions: solid early lymphatic and hematogenic with successive mutations of KRAS, pseudopapillary tumor and endocrine dissemination. At the time of diagnosis, p53, p16, DPC4, and three categories neoplasia. Cystic pancreatic masses less than 10% of the lesions present of cystic neoplastic lesion: mucinous are increasingly being recognized, as localized tumors, and the patients’ cystic adenomas, intraductal papillary as a direct consequence of the 5-year survival is still not over 5%. In mucinous neoplasms (IPMNs), and more widespread use of imaging the International Agency for Research serous cystadenomas, which have a technology. Nonneoplastic and on Cancer (IARC) Globocan 2002 very low risk for malignancy. inflammatory cystic masses— database, the worldwide burden of Nonductal adenocarcinomas pseudocysts—are of course by far pancreatic cancer for the year 2005 is develop from the acinar cells and the most common pancreatic cystic estimated at 232,000 new cases and are relatively rare. In acinar cell lesions, associated with a history 227,000 deaths [1]. Of the new cases, carcinoma, acinar differentiation of pancreatitis. Cystic neoplasms, 125,000 affect men and 107,500 is confirmed by zymogen granules which represent 10–15% of cystic women. In the same database, cancer positive for periodic acid–Schiff masses of the pancreas, include of the pancreas ranks twelfth for the and immunostaining for trypsin; the serous cystadenomas, mucinous frequency of cancer and eighth for tumor is usually large at detection cystic neoplasms, and intrapancreatic mortality. (around 10 cm) and mutations of mucinous neoplasms (IPMNs). Incidence. The estimated numbers the β-catenin gene are frequent. Finally, some solid tumors may have of annual cases occurring in various Pancreatoblastoma is another highly a cystic morphology; this occurs in regions are shown in Table 1, which malignant nonductal carcinoma, which pseudopapillary neoplasm, acinar is derived from the IARC database occurs in children under the age of carcinoma (which is then classified [1]. Geographic variations in the 10 years. The genetic alterations of ductal carcinoma are not present, and alteration in the β-catenin pathway Region Cases (n) is frequent. Solid pseudopapillary North America 34900 Table 1. Estimated numbers of incident neoplasms, which are borderline South America 13400 cases of cancer of the pancreas (both lesions with low-grade malignancy, sexes) occurring in 2002 in various Central America 4200 regions of the world, from the International are also classified in this group. Agency for Research on Cancer (IARC) Africa (5 areas) 7100 Endocrine pancreatic tumors, Globocan 2002 database [1]. Europe (with Russia) 78000 including multiple endocrine neoplasia Asia (with Japan) 91000 type 1 (MEN-1), represent a thirdMAY 2009 WORLD GASTROENTEROLOGY NEWS 5
  6. 6. Scientific news (1a) Incidence of Pancreas cancer: ASR (World) - Male (All ages) relative survival have been analyzed < 1.3 for the period 1975–2000 in the SEER < 2.3 registries and are shown in Table 5; < 4.2 < 7.2 a slight improvement is evident, with < 12.1 figures increasing from 3.6% to 5.2% in men and 2.1% to 5.4% in women [3]. Causal factors in sporadic pancreatic cancer The identification of environmental factors in carcinogenesis is of major help for developing cancer prevention policies. Unfortunately, most case– control and cohort studies conducted (1b) Incidence of Pancreas cancer: ASR (World) - Female (All ages) for pancreatic cancer have proved inconclusive. < 1.1 Smoking. This is the only significant < 1.7 promotor factor. In a meta-analysis < 3.5 conducted recently by Iodice et al. [5], < 4.6 the risk of pancreatic cancer is increased < 11.4 at 1.74 for current smokers and 1.2 for former smokers. Overall, smoking causes a 75% increase in risk of pancreatic cancer. Taking into account the proportion of smokers in the population, the attributable risk of smoking for pancreatic cancer is around 20%. Alcohol, coffee, tea. The data from two very large cohort studies in the Fig 1. Regional variations in the age-adjusted incidence per 100,000 of cancer of the USA, the Health Professionals Follow- pancreas presented in a world map, for men (1a) and women (1b). (From the International Agency for Research on Cancer Globocan 2002 database) [1]. Up Study and the Nurses’ Health Study, with 1,907,222 person-years of follow- up, have been revised by Michaud et al. estimated age-adjusted incidence per Mortality. A comparison between the [6]. They do not support any association 100,000 (age-standardized rate, ASR) estimated incidence and mortality from between coffee or alcohol intake and for pancreatic cancer are shown for pancreatic cancer is also available in the risk of pancreatic cancer. A similar men and women in Fig. 1 from the the IARC database [1] and is shown negative conclusion emerged from the same database. The figures are higher for three countries in Table 4. The European Prospective Investigation in North America, European countries annual number of deaths is equivalent into Cancer and Nutrition (EPIC) and and Russia, Japan, and Argentina, and to the annual number of new cases; from the Collaborative Cohort Study for much lower in developing countries in this confirms that the average survival Evaluation of Cancer Risk (JACC study) Africa, as well as in India and China. is not more than 1 year from diagnosis. in Japan. The data in the population- The observed ASR for pancreatic The age-adjusted mortality rate of per based cohort study in Japan (JPHC cancer in cancer registries is shown 100,000 in men and in women is very study) do not support any impact of for some countries around the world in similar to that of ASR incidence, as green tea consumption on the risk of Table 2 and is slightly lower in women shown in Table 3; the difference is pancreatic cancer. than in men [2]. In the USA, slight minimal in men but more marked in Physical activity and obesity. differences in the ASR incidence are women. Occupational or leisure-time physical shown in the Surveillance Epidemiology Survival. The prognosis with activity has been associated with a and End Results (SEER) registries for pancreatic carcinoma is very poor, lower risk for pancreatic cancer in different ethnic groups (Table 3) [3]. and the relative 5-year survival in 83 several studies, but a systematic review The highest values occur in persons of cancer registries and 23 European of the literature has not provided strong African origin and the lowest in those countries in the EUROCARE-4 study evidence for an association. In a meta- with Native American and Asian ethnic was 5.5% for both sexes in the period analysis conducted by Larsson et al. on backgrounds. 1995–99 [4]. Time trends in the 5-year the role of obesity, a slight association 6 WORLD GASTROENTEROLOGY NEWS MAY 2009
  7. 7. Scientific news Men Women Chile: Valdivia 4.2 3.8 occur in chronic pancreatitis (type 1 Table 2. Observed age adjusted diabetes). It has been suggested that USA: SEER, 14 registries 7.9 5.9 incidence rate per 100,000 of pancreatic cancer causes diabetes. China: Hong Kong 4.5 3.1 cancer of the pancreas in cancer registries from various countries Recognition of new-onset diabetes as China: Shanghai 7.5 5.3 during the period 1998–2002 an early manifestation of pancreatic Japan: Osaka 9.3 5.4 (from Cancer Incidence in Five cancer could lead to the diagnosis of Korea: Seoul 8.7 5.0 Continents, vol. 9, Lyons: IARC, early-stage pancreatic cancer. New- 2007; IARC Scientific Publications Denmark 7.8 6.6 onset hyperglycemia could be used no. 160) [2]. France: Calvados 7.6 5.2 as a screening tool to identify people Italy: Veneto 9.9 7.0 with asymptomatic pancreatic cancer; however, the success of this method depends on our ability to differentiate was observed, with an estimated insulin resistance are involved in the pancreatic cancer-associated diabetes relative risk of pancreatic cancer (per development of pancreatic cancer, but from the more common type 2 diabetes. 5 kg/m2) of 1.12 [7]. epidemiologic studies on the role of Fruit and vegetables. Case–control added sugar or sugar-sweetened foods Hereditary factors studies have suggested that higher and beverages are inconclusive. In a The occurrence of at least two cases consumption of fruit and vegetables, large study with a 7.2-year follow-up of pancreatic cancer in first-degree including citrus fruits, is associated conducted in the USA, consumption relatives of a family suggests a with a lower risk of pancreatic cancer, of sugar was not associated with familial aggregation of cases. This but cohort studies do not support pancreatic cancer. situation is present in almost 10% of this association. No protection Diabetes. Two types of diabetes are cases and has analyzed by Geenen against pancreatic cancer from the associated with pancreatic diseases. and Kaul in another issue of WGN. consumption of fruit and vegetables was Hyperinsulinemia and peripheral insulin Pancreatic cancer can occur in demonstrated in the very large European resistance (type 2 diabetes) are the genetic hereditary syndromes for other prospective study cohort (EPIC). prevailing diabetic traits in pancreatic categories of tumor, with an identified Sugar. It has been hypothesized that cancer, whereas reduced islet cell germline mutation: BRCA1/BRCA2 hyperglycemia, hyperinsulinemia, and mass and impaired insulin secretion gene mutations and breast cancer, mismatch repair gene (MLH1, MSH2) mutations and hereditary nonpolyposis Men Women colorectal cancer (HNPCC) syndrome, Incidence Mortality Incidence Mortality STK-11/LKB1 gene mutation, and Peutz–Jeghers syndrome, CDKN2A All ethnic groups 13.0 12.2 10.3 9.3 gene mutation and the familial atypical White 13.0 12.1 10.0 9.0 multiple mole-melanoma (FAMMM) Black 16.2 15.4 14.3 12.4 syndrome, in which melanomas Asian 10.1 8.0 8.2 6.9 are associated with benign moles. Native Americans 10.9 8.6 8.2 7.2 Pancreatic cancer also occurs in the Hispanic 10.9 9.1 10.3 7.6 hereditary pancreatitis syndrome, in which mutations in SPINK1/PRSS1 Table 3. Observed age-adjusted incidence and mortality per 100,000 of cancer of the genes play a role. This syndrome is pancreas by sex and ethnic group in cancer registries in the USA (2001–2005 period, from 17 Surveillance Epidemiology and End Results registries) [3]. characterized by recurrent attacks of acute pancreatitis. Finally, aggregation of pancreatic cancer occurs in the Country Cases (n) Deaths (n) Men Women familial pancreatic cancer syndrome, France 5300 7250 1975 3.6 2.1 where the mutation is unknown. USA 31650 30300 1985 2.6 3.9 Familial pancreatic cancer can be Japan 19900 20100 1995 3.1 4.5 defined as an inherited predisposition 2000 5.2 5.4 based on family clustering, with two or Table 4. Comparison of the estimated more blood relatives with pancreatic numbers of incident cases and of Table 5. Time trends in the 5-year adenocarcinoma of ductal origin. As deaths from cancer of the pancreas relative survival by sex in cancer shown by Hruban et al. [8], a family (both sexes) occurring in 2002 in registries in the USA (1975–2000 three countries, from the International period, from nine Surveillance history of pancreatic cancer is present Agency for Research on Cancer (IARC) Epidemiology and End Results in about 10% of patients with this Globocan 2002 database [1]. registries) [3]. disorder.MAY 2009 WORLD GASTROENTEROLOGY NEWS 7
  8. 8. Scientific news Several registries are collecting occur predominantly in middle-aged screening procedure, may detect data on hereditary pancreatic cancer. women and account for 45% of primary dilation of the biliary duct and even of The EUROPAC registry is based in cystic neoplasms. Their appearance is the main pancreatic duct (of Wirsung), Liverpool, United Kingdom, and there often that of a cyst within a cyst, without but the pancreas itself is often poorly are about a dozen hereditary pancreatic communication with the pancreatic visible due to gas in overlying bowel cancer registries established in the duct. The presence of ovarian stroma segments. The procedure is very USA, including those at the Johns in the tumor is strongly suggestive of operator-dependent. High-resolution Hopkins Medical Center and the Mayo the diagnosis. They are classified as helical CT scanning of the abdomen Clinic. A Familial Pancreatic Tumor borderline neoplastic lesions, which with intravenous contrast enhancement Registry has been established at the progress in 5–35% of cases to mucinous is now widely available. The size and Memorial Sloan-Kettering Cancer cystadenocarcinomas. Intraductal the location of cystic neoplasms in the Center in New York. Over 1500 papillary mucinous neoplasms (IPMNs) head, body, or tail of the pancreas can individuals have been recruited to the occur more often in men and account be determined, as well as the presence registry, including pancreatic cancer for 25% of primary cystic neoplasms. of intracystic masses or mural nodules patients with multiple affected relatives, Their appearance is that of a grape and communication with the pancreatic healthy individuals with multiple affected with cysts. IPMNs arise from the main duct. Serous cystic adenomas often relatives, healthy controls, patients pancreatic duct or from its branches, show a honeycomb pattern, with with sporadic pancreatic cancer, and and communicate with the duct. IPMNs multiple, small cysts within a large patients with IPMNs. Because of the arising from the main pancreatic duct cyst and a central stellar scar that may association of BRCA mutations and carry a higher risk of malignancy (60– calcify. Mucinous cystic neoplasms are pancreatic cancer, individuals with 90%) than those arising from collateral often large and multilocular; IPMNs a BRCA1 or BRCA2 mutation and a branches (around 5%). produce the characteristic image of a family history of pancreatic cancer are Solid pseudopapillary neoplasms are bunch of grapes containing numerous also recruited. The registry has served tumors with nonductal differentiation, small cysts, communicating with a as a resource for a number of ongoing composed of partially encapsulated dilated pancreatic duct or its branches; studies, including epidemiology of sheets of polygonal cells with a cystic mural nodules or intracystic masses are pancreatic cancer, screening at-risk morphology. They stain positive for frequent. Another recently introduced relatives for pancreatic neoplasia, and the markers vimentin and CD10, and imaging test is magnetic resonance genome-wide association studies. are negative for ductal and endocrine cholangiopancreatography (MRCP). markers. Solid pseudopapillary Single-shot, fast spin-echo MR images Premalignant neoplastic neoplasms are more frequent in can detect small pancreatic cysts lesions women and are classified as borderline with more precision than abdominal Pancreatic intraepithelial neoplasia malignant lesions. ultrasound or CT scanning. is a precursor of ductal carcinoma. In surgical specimens, areas of flat Diagnosis Endoscopy hyperplasia with columnarization of The dramatic progress in radiographic Endoscopic retrograde ductal epithelium and ductal papillary imaging and endoscopic exploration cholangiopancreatography (ERCP) hyperplasia are found adjacent to of the pancreas has led to a much is the most sensitive technique for invasive carcinoma. The progression more reliable classification of cystic detecting a communication between to malignancy is accompanied at an lesions of the pancreas and made a cyst and the pancreatic ducts early stage by mutations in the KRAS it possible to distinguish malignant occurring in IPMN. The diagnosis oncogene, followed by mutations of or benign neoplastic lesions from is also suggested when mucus is p53, p16, and DPC4/ SMADD4 tumor pancreatitis. Percutaneous biopsies of seen leaking from a patulous papilla. suppressor genes. a solid pancreatic mass performed with Pancreatoscopy for direct observation Benign cystic neoplasms are tumors ultrasound or computed tomography of the tumoral lesions in the pancreatic with ductal differentiation that have a (CT) guidance provide tissue duct is now possible using small-caliber variable potential for malignancy. Serous specimens for histology. Overall, there flexible endoscopes (miniscopes). cystadenomas (SCAs) occur mainly has been some progress in the early Endoscopic ultrasonography (EUS) is in middle-aged women and account detection of pancreatic cancer, but increasingly available for the diagnosis for 30% of primary cystic neoplasms; survival after treatment for pancreatic and management of pancreatic depending on their size, they are cancer remains depressingly poor. neoplastic lesions. As an alternative to classified as microcystic or macrocystic. conventional echo endoscopes, flexible They are usually benign; progression to Imaging high-frequency (20-MHz) catheter serous cystadenocarcinoma is very rare. Transabdominal ultrasound, which probes can be introduced through the Mucinous cystic neoplasms (MCNs) outside the U.S. is still the basic working channel of an endoscope. In 8 WORLD GASTROENTEROLOGY NEWS MAY 2009
  9. 9. Scientific news the presence of mucinous neoplasms, Screening and surveillance and beneficial. A screening program malignancy can be predicted with an Guidelines recommend surveillance was started at the Memorial Sloan- accuracy of 50% with EUS, based on using repeated imaging procedures Kettering Cancer Center in 2002, the following criteria: size greater than (CT, MRI, EUS) in individuals diagnosed using cross-sectional imaging as the 2 cm, pancreatic duct dilation, wall as having an IPMN, which is associated primary screening tool, followed by calcifications, and masses or mural with a significant risk of malignant endosonography if any pancreatic nodules. In specialized centers, EUS development. abnormalities were found. IPMN is used to aspirate cyst fluid using fine- No screening strategy for early was the most common pancreatic needle aspiration (FNA).The aspirated detection of sporadic pancreatic cancer lesion found in the FPC population fluid is evaluated for cytology, with is recommended in asymptomatic and may very well represent the cuboidal cells staining for glycogen, persons. On the other hand, screening pancreatic cancer precursor lesion in in serous cysts and columnar cells and surveillance at regular intervals this population. This finding is similar staining for mucin in mucinous cysts. are recommended for those with an to what was reported by Canto et al. Two biological markers are helpful increased risk for pancreatic ductal [9] from Johns Hopkins in their FPC for differentiating between mucinous adenocarcinoma. This applies to screening study. Clearly, it is too early and serous neoplastic lesions: inherited genetic syndromes, hereditary to determine the long-term benefit from amylase activity is low in serous pancreatitis, and familial pancreatic screening members of FPC families. neoplasms and high in mucinous cancer. In the USA, pancreatic cancer ones; the carcinoembryonic antigen has a low prevalence; however, (CEA) concentration is below the members of families with familial threshold value of 192 ng/mL in serous pancreatic cancer (FPC) are an neoplasms and above that value in identifiable at-risk group for whom mucinous ones. screening could be both feasible References 1. Ferlay J, Bray F, Pisani P, Parkin DM. 4. Berrino F, De Angelis R, Sant M, and 7. Larsson SC, Orsini N, Wolk A. Body mass Globocan 2002: cancer incidence, mortality EUROCARE Working group. Survival for index and pancreatic cancer risk: a meta- and prevalence worldwide. IARC CancerBase eight major cancers and all cancers combined analysis of prospective studies. Int J Cancer Int J Cancer No. 5, version 2.0. Lyons: IARC Press, 2004. for European adults diagnosed in 1995–99: 2007;120:1993–8. results of the EUROCARE-4 study. Lancet 2. Curado MP, Edwards B, Shin HR, et al., 8. Hruban RH, Petersen GM, Goggins M, Oncol 2007;8:773–83. editors. Cancer incidence in five continents, et al. Familial pancreatic cancer. Ann Oncol Familial pancreatic cancer. Ann Oncol vol. 9. Lyons: IARC, 2007 (IARC Scientific 5. Iodice S, Gandini S, Maisonneuve P, et al. 1999;10(Suppl 4):69–73. Publications, no. 160). Tobacco and the risk of pancreatic cancer: a 9. Canto MI, Goggins M, Hruban RH, et al. review and meta-analysis. Langenbecks Arch 3. Surveillance, Epidemiology, and End Screening for early pancreatic neoplasia Surg 2008;393:535–45. Results (SEER) program (public use). http:// in high-risk individuals: a prospective seer.cancer.gov/index.html. 6. Michaud DS, Giovannucci E, Willett WC, controlled study. Clin Gastroenterol Hepatol et al. Coffee and alcohol consumption and the 2006;4:766–81. risk of pancreatic cancer in two prospective United States cohorts. Cancer Epidemiol Biomarkers Prev 2001;10:429–37. Continued on Page 10MAY 2009 WORLD GASTROENTEROLOGY NEWS 9
  10. 10. Scientific news Further Reading Bae JM, Lee EJ, Guyatt G. Citrus fruit intake Kurtz RC, Ludwig E, Simon J, et al. Sahni VA, Mortele KJ. Magnetic resonance and pancreatic cancer risk: a quantitative Pancreatic screening program for familial high cholangiopancreatography: current use systematic review. Gastric Cancer risk individuals [abstract]. Gastroenterology and future applications. Clin Gastroenterol 2008;11:23–32. 2007;132(Suppl 2):A-119. Hepatol 2008;6:967–77. Fitzgerald TL, Hickner ZJ, Schmitz M, et al Lachter J, Cooperman JJ, Shiller M, et al. Seo S, Doi R, Machimoto T, et al. Contribution Changing incidence of pancreatic neoplasms: The impact of endoscopic ultrasonography of 18F-fluorodeoxyglucose positron a 16-year review of statewide tumor registry. on the management of suspected emission tomography to the diagnosis of Pancreas 2008;37:134–8. pancreatic cancer—a comprehensive early pancreatic carcinoma. J Hepatobiliary longitudinal continuous evaluation. Pancreas Pancreat Surg 2008;15:634–9. Geenen JE, Kaul V. Familial pancreatic 2007;35:130–4. cancer: an update. World Gastroenterology Shi C, Daniels JA, Hruban RH. Molecular News [in press]. Parkin DM, Whelan SL, Ferlay J, et al., characterization of pancreatic neoplasms. editors. Cancer incidence in five continents, Adv Anat Pathol 2008;15:185–95. Hart AR, Kennedy H, Harvey I. Pancreatic vol. 8. Lyons: IARC, 2002 (IARC Scientific cancer: a review of the evidence on Singh M, Maitra A. Precursor lesions of Publications, no. 155). causation. Clin Gastroenterol Hepatol pancreatic cancer: molecular pathology 2008;6:275–82. Rubenstein JH, Scheiman JM, Anderson and clinical implications. Pancreatology MA. A clinical and economic evaluation of 2007;7:9–19. Ho JM, Darcy SJ, Eysselein VE, et al. endoscopic ultrasound for patients at risk Evolution of fine needle aspiration cytology Zhang XM, Mitchell DG, Dohke M, et al. for familial pancreatic adenocarcinoma. in the accurate diagnosis of pancreatic Pancreatic cysts: depiction on single-shot Pancreatology 2007;7:514–25. neoplasms. Am Surg 2007;73:941–4. fast spin-echo MR images. Radiology Sahani DV, Lin DJ, Venkatesan AM, et al. 2002;22:547–53.a Hutchins GF, Draganov PV. Cystic neoplasms Multidisciplinary approach to diagnosis of the pancreas: a diagnostic challenge. and management of intraductal papillary World J Gastroenterol 2009;15:48–54. mucinous neoplasms of the pancreas. Clin Klimstra DS. Nonductal neoplasms of the Gastroenterol Hepatol 2009;7:259–69. pancreas. Mod Pathol 2007;20(Suppl 1): S94–112. 10 WORLD GASTROENTEROLOGY NEWS MAY 2009
  11. 11. Scientific news How much fluid should be given during the initial management of acute pancreatitis? Nison Badalov, MD and Scott Tenner*, MD (*photo) Division of Gastroenterology, Maimonides Medical Center, State University of New York Health Sciences Center, 2211 Emmons Avenue, Brooklyn, NY 11235, USA Regardless of etiology, the illustrates this process (Fig. 1). pancreatic perfusion, pancreatic pathogenesis of acute pancreatitis Vigorous intravenous hydration necrosis and the complications of results in the extravasation of leads to hemodilution and relief of pancreatitis leading to severe disease liters of intravascular fluid into the hemoconcentration. This translates into are prevented (Fig. 2). peritoneum. These losses manifest in direct benefits for the patient with acute Too often, patients with acute the development of pancreatic ascites, pancreatitis. A decreased hematocrit is pancreatitis are given suboptimal hypotension, tachycardia, and further associated with mild disease. Also, a intravenous hydration, resulting in destruction of the pancreas, also falling hematocrit during the first 24 h of pancreatic necrosis and organ failure. referred to as “pancreatic necrosis.” care leads to a decrease in morbidity. How much fluid should be given? Impairment of the microcirculation of the Clinical studies with aggressive plasma Part of the answer is related to the pancreas appears to lead to pancreatic volume expansion using intravenous amount of losses the patient presents necrosis. A vicious cycle develops dextran to promote hemodilution have with, and the other part is related to in which pancreatic inflammation suggested efficacy in preventing severe continuing losses due to the ongoing leads to extravasation of protein-rich disease. Although dextran is not used pancreatic inflammatory process. A intravascular fluid into the peritoneum. clinically at present, isotonic saline is patient who presents with hypotension The intravascular hypovolemia that our practical alternative. It appears that and tachycardia clearly needs more accompanies acute pancreatitis vigorous intravenous hydration early aggressive hydration than one who is subsequently leads to a decrease in the course of acute pancreatitis can normotensive with a normal baseline in pancreatic blood flow. Pancreatic prevent the development of necrosis. pulse. Regardless of this, clinicians ischemia leads to the activation of The goal in managing patients with must suspect that a patient with acute inflammatory mediators. The decreased acute pancreatitis is to decrease the pancreatitis will subsequently develop blood flow also causes stasis and hematocrit, achieve hemodilution, serious intravascular fluid losses. One the development of thrombi, leading decrease the blood urea nitrogen (BUN) of the markers of severity previously to subsequent necrosis, which then and creatinine, and promote renal defined by Ranson and colleagues is exacerbates the inflammatory process. blood flow. By preventing intravascular related to intravascular losses. Ranson The association of hemoconcentration, depletion of fluid and promoting et al. (1976) found that sequestration in which the hematocrit (HCT) level pancreatic blood flow, pancreatic (i.e., peritoneal pancreatic ascites) of rises, with pancreatic necrosis perfusion is maintained. By maintaining over 6 L of fluid during the first 48 h was Fig 1. In acute pancreatitis, hypoperfusion to the pancreas results in increased pancreatic necrosis, which leads to the release of proinflammatory mediators, which in turn exacerbates hypoperfusion, leading to greater necrosis and a rise in hematocrit (HCT).MAY 2009 WORLD GASTROENTEROLOGY NEWS 11
  12. 12. Scientific news Fig 2. Progression to pancreatic necrosis. These images show the progression of pancreatic necrosis in a patient with acute pancreatitis. The dynamic CT scan performed on day 1 shows opacification of the kidneys, spleen, and pancreas. In contrast, a repeat CT scan on day 3 shows opacification of the kidneys and spleen, but no pancreatic opacification. These images demonstrate a loss of pancreatic perfusion between days 1 and 3 of the disease. This patient was poorly hydrated (150 mL/h) and developed adult respiratory distress syndrome (ARDS), renal failure, and had a rise in HCT from 42% to 46%. an independent predictor of severity. h of hydration, it becomes apparent hematocrit by at least three points A patient with mild disease routinely that if the same patient develops from baseline (below 44%). On the would lose 3–5 L into the peritoneum. acute pancreatitis, he is far more likely basis of animal studies, the goal may Not including baseline losses, to receive inadequate hydration in be to decrease the hematocrit to the how much intravenous hydration is comparison with a normal person. mid-30% range. If the patient presents needed? If we use the Ranson upper Which type of fluid should be used? as hypotensive and tachycardic, limit of severity of an amount of 6 L In order to promote perfusion and intravenous hydration should be much (expected losses), added to the minimal maintain intravascular pressure, the more aggressive—e.g., 500 mL/ intravenous fluid requirements of a 70- fluid should be isotonic. Hypertonic h minimum. Currently, there is no kg person during the first 48 h (8 L), solutions, which would maintain or specific therapy available to attenuate intravenous hydration should be at least even increase intravascular pressure, the inflammatory response in acute 300–350 mL per hour initially. The rate of are being studied but are considered pancreatitis. Instead, practice guidelines hydration is likely to be more important experimental. The two widely available universally recommend supportive care during the first 24 h, when a rising HCT isotonic solutions are “normal” (0.9%) with intravenous hydration. has been shown to correlate closely with saline and lactated Ringer’s solution. severe disease. The rate of hydration There are several theoretical benefits should be titrated to the hematocrit. The to using the more pH-balanced lactated Further Reading goal is to decrease the hematocrit 5–10 Ringer’s solution for fluid resuscitation points during the first 24 h. in comparison with normal saline. Baillargeon JD, Orav J, Ramagopal There are multiple caveats to the Although both are crystalloid solutions, V, Tenner SM, Banks PA. basic assumption of the initial rate the development of non-anion gap, Hemoconcentration as an early risk of hydration. In a patient with acute hyperchloremic metabolic acidosis factor for necrotizing pancreatitis. Am J pancreatitis, in order to guide hydration, associated with the infusion of large Gastroenterol 1998;93:2130–4. the clinician must take into account volumes of normal saline has been Gardner TB, Vege SS, Pearson RK, the patient’s age, underlying cardiac, well described. On the basis of the Chari ST. Fluid resuscitation in acute renal and pulmonary disease, and body metabolic acidosis associated with large- pancreatitis. Clin Gastroenterol Hepatol mass index (BMI). Whereas all elderly volume infusion of normal saline and 2008;6:1070––6. patients need to be followed closely, the available evidence suggesting that those with renal and cardiovascular the inflammation associated with acute Klar E, Herfarth C, Messmer K. disease may need intracardiac pancreatitis is a pH-dependent process, Therapeutic effect of isovolemic monitoring to guide hydration and resuscitation with lactated Ringer’s hemodilution with dextran 60 on the prevent congestive heart failure. Over solution may have significant benefits impairment of pancreatic microcirculation the last several years, we have learned over normal saline in the early treatment in acute biliary pancreatitis. Ann Surg that patients who are obese, with an of patients with acute pancreatitis. 1990;211:346–53. elevated BMI, are at increased risk Ranson JH, Rifkind KM, Turner JW. of pancreatic necrosis, organ failure, Summary Prognostic signs and nonoperative and death. It is likely that the reason In a patient who is otherwise healthy, peritoneal lavage in acute pancreatitis. why obese patients (those with an presenting as normotensive with Surg Gynecol Obstet 1976;143:209–19. elevated BMI) are more likely to have minimal tachycardia, intravenous complicated disease is directly related hydration with lactated Ringer’s solution Tenner S. Initial management of acute to inadequate intravenous hydration. at 250–350 mL/h, depending on BMI, pancreatitis: critical decisions during When one considers that a 100-kg should be initiated and maintained the first 72 hours. Am J Gastroenterol male who is 1.93 m (6 feet 4 inches) until the acute inflammatory process 2004;99:2489–94. tall needs a baseline of almost 400 mL/ resolves. The goal is to decrease the12 WORLD GASTROENTEROLOGY NEWS MAY 2009
  13. 13. Scientific news Management of autoimmune pancreatitis: a review of medical therapy Ashraf Saleemuddin, MD and William R. Brugge*, MD (*photo) Tufts University School of Medicine and Massachusetts General Hospital, Boston, during the course of the corticosteroid Massachusetts, USA therapy. The patients were followed for a period of between 12 and 142 months and there were no indications for progression of pancreatic malignancy Introduction alternatives to corticosteroid therapy. and no recurrence of symptoms of The mainstay of medical therapy The surgical therapy was also reviewed jaundice or abdominal pain. A poor for autoimmune pancreatitis (AIP) is and used as a comparison. response to 1–2 weeks of corticosteroid administration of oral corticosteroids. therapy should raise concern for the A variety of regimens have been Critical literature review possibility of pancreatic cancer or forms described and used, but there is Autoimmune pancreatitis (AIP) of non-AIP chronic pancreatitis. a lack of consensus regarding is a form of chronic pancreatitis duration, dosing, and alternatives. characterized by lymphoplasmacytic Short-course Although corticosteroid therapy cellular infiltration of the pancreas, as corticosteroids is highly effective, supplementary well as other organs. The infiltration Hirano et al. have described the therapy is important in patients who may involve the ducts, parenchyma, response to a short course of steroids have suffered from side effects or or both. Although AIP is characterized [2]. Nineteen patients diagnosed with complications of corticosteroids. The by clear clinical, histologic, radiologic, AIP using the Mayo Clinic criteria role of immunosuppressive agents and serologic findings, differentiating were treated with a prednisone dose has recently been explored in a small the inflammatory process from of 25–50 mg per day for 2–4 weeks. number of patients. We have reviewed pancreaticobiliary malignancies The dose was tapered by 5 mg every the available literature on the medical remains a challenge. Medical therapy 2–4 weeks until the daily dose reached therapy of autoimmune pancreatitis and for AIP consists primarily of short 5 mg, followed by maintenance compared it to surgical therapy. (1–2 months) or medium (2–6 months) therapy at a dose of 2.5–5.0 mg/day. courses of corticosteroids. The use of In all 19 patients, imaging findings Methods corticosteroids provides an opportunity and laboratory abnormalities improved A text-word literature review was for therapy as well as a diagnostic 4 weeks after initiation of steroid performed using the PubMed and assessment in terms of resolution of therapy. In comparing AIP patients Medline databases. Terms including pancreatic lesions. treated with corticosteroids and to those “autoimmune pancreatitis,” “treatment In a recent series, the success of patients not treated, it was found that of autoimmune pancreatitis,” and treatment with corticosteroids was AIP-related unfavorable events such “management of autoimmune defined as resolution of the histologic, as jaundice, abnormal liver function pancreatitis” were used for a radiologic, and serologic parameters tests, and pancreatic pseudocyst were comprehensive search over the past [1]. In this series, a week’s course reduced in the corticosteroid-treated 20 years. Published manuscripts of prednisone 30–40 mg/day was group. During follow-up of the patients providing original reports of medical and followed by daily dose tapering of 2.5– not treated with corticosteroids, it was surgical therapy of AIP in peer-reviewed 5.0 mg/week. All patients treated with found that unfavorable events were journals were critically reviewed. steroid therapy for 1 month showed a observed in 16 of 23 patients (70%) Responses (clinical, radiologic, and significant reduction in the pancreatic after 25 months on average from onset. serologic) to the regimens described abnormality as defined by the results The effectiveness of corticosteroid were compiled in a table format. Nine of computed tomography (CT) and therapy may be due to its role in manuscripts describing therapy for AIP magnetic resonance imaging (MRI). In improving bile duct stenosis in patients in 101 patients were critically reviewed addition, serum gamma-globulin, IgG, with obstructive jaundice. Using a for the dose, duration of therapy, and and CA19-9 levels returned to normal longer course of corticosteroids, ItoMAY 2009 WORLD GASTROENTEROLOGY NEWS 13
  14. 14. Scientific news Table 1. Response of patients with autoimmune pancreatitis (AIP) to steroid therapy. All studies included in the table are sources of primary data. First author, ref. Patients Diagnostic criteria Dose Steroid therapy duration Responses to (n) therapy (n) Song [1] 4 1, diagnostic histopathologic features and bound IgG4+ 30–40 mg/d × Taper daily dose by 2.5–5 mg/wk 4 plasma cells on pancreatic tissues; 2, characteristic imaging 1 wk on CT and pancreatography, together with increased serum IgG, gamma-globulin levels or presence of autoantibodies; 3, response to steroid therapy Hirano [2], 19 Mayo * 25–50 mg/d × Taper 5 mg every 2–4 wk until 19 Nishimori [16] 2–4 wk 5 mg/d, then 2.5–5.0 mg/d for maintenance Ito [8] 14 JPS † 30, 40 mg/d × Taper over 2–3 months 98.4% remis- 2 wk sion rate Kamisawa [7] 10 Enlargement of the pancreas, irregular narrowing of the 30–40 mg/d Taper by 5 mg every 1–2 wk 10 MPD,‡ hypergammaglobulinemia (over 2.0 g/dL), elevated serum (IgG4, over 135 mg/dL), presence of autoantibod- ies, and lymphoplasmacytic infiltration with fibrosis of the pancreas Okazaki [17] 21 JPS NI § NI NI Kubota [4] 12 JPS 30 mg/d × 2 wk Taper by 5 mg/wk until daily 12 does of 5 mg reached Church [6] 11 1, increased levels of serum gammaglobulin or IgG4; 2, 30 mg/d Tapered over 2–3 months 11 presence of autoantibodies; 3, diffuse enlargement of the pancreas or a pancreatic mass; 4, diffuse irregular narrowing of the MPD; 5, fibrotic changes with lymphocyte infiltration; 6, no symptoms or only mild symptoms, usually without attacks of acute pancreatitis; 7, rare pancreatic calcification or cysts; 8, occasional association with other autoimmune diseases; 9, effective steroid therapy Kamisawa [18] 10 Swelling of the pancreas, irregular narrowing of the MPD, 5, 30, 40, 60 mg/d Tapered by 2.5–5 mg every 10 hypergammaglobulinemia, elevation of serum IgG4, pres- 1–2 wk ence of autoantibodies, association with other autoimmune diseases, and lymphoplasmacytic infiltration with fibrosis Ito [3] 3 Hypergammaglobulinemia, eosinophilia, US showing hy- 30 mg/d 1 month 3 poechoic diffuse swelling of pancreas, ERCP showing diffuse narrowing of the MPD with irregular thumbprint-like marks, reversible exocrine insufficiency, and positive anticarbonic anhydrase II antibody Chari [9] 17 Mayo 40 mg/d × 4 wk Tapered by 5 mg /wk over 8 wk 17 Moon [10] 22 Previously reported “cardinal features of AIP” and one of 0.5 mg/kg/d × Tapered by 5–10 mg/month to 15 following imaging features: 1, diffuse pancreatic enlargement 1–2 months dose of 2.5–7.5 mg/d with or without capsule-like rim; 2, delayed enhancement of pancreatic mass; 3, diffusely attenuated MPD with irregular wall; 4, none-to-mild upstream duct dilation despite long stricture; 5, double duct sign without a pancreatic mass in a patient with obstructive jaundice; 6, association of hilar or intrahepatic duct strictures; or 7, other organ involvement unusual for pancreatic cancer such as a salivary gland, kidney, or retroperitoneal fibrosis Ghazale [11] 30 Mayo 40 mg/d × 4 wk Tapered by 5 mg/wk for total 29 of 11 wk * Mayo criteria: Histology: 1, periductal lymphoplasmacytic infiltrate with obliterative phlebitis and storiform fibrosis or 2, lymphoplasmacytic infiltrate with storiform fibrosis showing abundant (> 10 cells/high-powered field) IgG4-positive cells; Typical: diffusely enlarged gland with delayed (rim) enhancement; diffusely irregular, attenuated main pancreatic duct; Other: focal pancreatic mass/enlargement; focal pancreatic duct stricture; pancreatic atrophy; pancreatic calcification; or pancreatitis; elevated serum IgG4 level (normal, 8–140 mg/dL); hilar/ intrahepatic biliary strictures, persistent distal biliary strictures, persistent distal biliary stricture, parotid/lacrimal gland involvement, mediastinal lymphadenopathy, retroperitoneal fibrosis; resolution/marked improvement of pancreatic/extrapancreatic manifestation with steroid therapy. † Japan Pancreas Society criteria: Typical imaging: diffuse enlargement of pancreas along with diffuse (> 33%) main pancreatic duct narrowing with an irregular wall and 1, serology: autoantibodies, elevated gamma-globulins or IgG or 2, histopathology: lymphoplasmacytic infiltrate and pancreatic fibrosis. ‡ Main pancreatic duct. § Not indicated in paper.14 WORLD GASTROENTEROLOGY NEWS MAY 2009
  15. 15. Scientific news et al. described the course of 125 described by Kamisawa et al. [5]. Ten Improvement in serologic values also patients diagnosed with AIP as defined patients were diagnosed with AIP using improved with the symptoms. by the Japanese Pancreas Society the following criteria: enlargement A number of radiologic and serologic (JPS) criteria [3]. The patients were of the pancreas, irregular narrowing criteria can be used to monitor response started on a prednisone dose of 30 mg of the main pancreatic duct (MPD), to therapy. Kamisawa et al. tracked the or 40 mg for 2 weeks and tapered hypergammaglobulinemia (over 2 g/ serologic, morphologic, and imaging over 2–3 months, leading to a 98.4% dL), presence of autoantibodies, and response to corticosteroids [7]. Ten remission rate (123 of 125). The lymphoplasmacytic infiltration with patients diagnosed with the criteria authors found that patients who had fibrosis of the pancreas. All 10 patients indicated in Table 1 were started on corticosteroid therapy took 89.7 days on were started on 30–40 mg/day of initial prednisone doses of 5, 30, 40, or average to reach complete remission, prednisone, and the dose was tapered 6 mg per day. The doses were tapered whereas those patients without by 5 mg every 1–2 weeks. Steroid by 2.5–5.0 mg every 1–2 weeks, and all corticosteroid therapy took 149.8 days therapy was effective morphologically 10 patients improved clinically as well as to reach complete remission. It is and serologically in all 10 patients with with objective criteria. In a small series of important to note that untreated AIP. Pancreatic enlargement showed patients, Ito et al. have demonstrated the patients demonstrated a high rate of improvement from 1 to 2 weeks after response to prednisone 30 mg/day [8]. spontaneous remission. initiation of treatment, and the pancreas Three patients were treated with 30 mg/ With increasing experience with returned to normal size within 3– day for 1 month, and all responded the initial corticosteroid therapy, it has 4 weeks after the start of therapy. to treatment as defined by clinical become apparent that short courses symptoms and radiologic changes. Since might be as effective as a moderate Symptom response to the pancreas can sometimes undergo course of corticosteroids. In the series corticosteroids marked atrophy after corticosteroid by Kubota et al., 12 patients were The symptoms of AIP, including therapy, the authors suggest that diagnosed with AIP using the JPS jaundice will also resolve with steroids should be discontinued after criteria and started on 30 mg/day for corticosteroid therapy [6]. Eleven adequate morphological improvement. 2 weeks [4]. The dose was tapered patients were started on a corticosteroid The pancreatic manifestations of AIP by 5 mg per week until a daily dose dose of 30 mg per day, which was should be responsive to corticosteroid of 5 mg was reached. All 12 patients tapered over 2–3 months, and in all therapy. In the study by Chari et al., showed a complete response to steroid patients there was an improvement of 17 AIP patients were followed for a therapy. symptoms within 4 weeks. Jaundice period of between 2 and 56 months, The pace of corticosteroid tapering and abdominal pain were eliminated and all patients showed complete in the initial therapy of AIP has been over the course of treatment. resolution of pancreatic manifestations First author, Patients (n) Type of surgical resection n Response to therapy (n) ref. Table 2. Response of Song [1] 21/43 Pancreaticoduodenectomy 4 All resections done in patients with autoimmune this study for suspected Choledochojejunostomy 14 pancreatitis (AIP) treated pancreatic malignancy with surgical resection. Radiofrequency ablation of pancreatic 1 head Intraoperative biopsy of pancreatic tis- 2 sues Ito [3] 2/21 Pancreatic resection 2 Toomey [12] 2 Diagnostic laparoscopy with choledocho- 1 1 jejunostomy Diagnostic laparoscopy with biopsies and 1 1 subsequent steroid therapy Kamisawa [7] 10 Pancreatoduodenectomy 6 6 Choledochojejunostomy 4 4 Hardacre [13] 37 Pancreaticoduodenectomy 37 68% improved (subjec- tive) Weber [14] 29 Pancreaticoduodenectomy 23 8/29 developed recur- rence of symptoms Distal pancreatectomy 4 Total pancreatectomy 2MAY 2009 WORLD GASTROENTEROLOGY NEWS 15

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