DOCSLIB.ORG

Primary Pancreatic Lymphoma: What We Need to Know

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Primary Pancreatic Lymphoma: What We Need to Know 757 Review Article Primary pancreatic lymphoma: what we need to know Neda Rad1, Alireza Khafaf2, Amir Houshang Mohammad Alizadeh3 1Research Institute for Gastroenterology and Liver Diseases, 2Gastroenterology and Liver Diseases Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran; 3Shahid Beheshti University of Medical Sciences, Taleghani Hospital, Tehran, Iran Contributions: (I) Conception and design: All authors; (II) Administrative support: AH Mohammad Alizadeh; (III) Provision of study material or patients: AH Mohammad Alizadeh, N Rad; (IV) Collection and assembly of data: N Rad, A Khafaf; (V) Data analysis and interpretation: All authors; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors. Correspondence to: Prof. Amir Houshang Mohammad Alizadeh. Shahid Beheshti University of Medical Sciences, Taleghani Hospital, Parvaneh Ave, Tabnak Str, Evin, Tehran 19857, Iran. Email: [email protected]. Abstract: Hodgkin’s lymphoma is a group of malignant lymphoid which involve various organs including gastrointestinal tract. Stomach and small intestine are commonly involved more; however, pancreas can be primarily involved as well. The secondary involvement of pancreas caused by Hodgkin’s lymphoma is more prevalent than the primarily involvement (1 .25–2.2% vs. <1%). Primary pancreatic lymphomas (PPLs) consist of 1–2% of all lymphoma outside nods. The symptoms and findings of PPL imaging can be akin to that of pancreas adenocarcinoma and differentiating them is difficult without examining the tissue sample. The prognosis and treatment of PPL are different from those of adenocarcinoma and due to the superior prognosis of PPL compared to pancreas adenocarcinoma, the proper diagnosis of the disease is important. Keywords: Pancreas; lymphoma; adenocarcinoma Submitted Mar 11, 2017. Accepted for publication May 18, 2017. doi: 10.21037/jgo.2017.06.03 View this article at: http://dx.doi.org/10.21037/jgo.2017.06.03 Introduction to obtain tissue samples through endoscopic ultrasound in recent years (2). Non-hodgkin’s lymphoma (NHL) is a group of malignant lymphoids which can be seen at any age. It is often determined with enlarged lymph nodes, fever, and weight Epidemiology loss. NHL commonly affects extranodal organs while PPL consists of approximately 1% of all extranodal the involvement of pancreas is primarily rare and can be lymphomas and 5% of all pancreatic masses (1). PPLs are with or without the involvement of lymph nodes around more common in males (58%) and are usually seen in the the pancreas (1). Differentiation of primary pancreatic 5th or 6th decades of life. lymphoma (PPL) and adenocarcinoma is important due The most histological type of PPL is diffuse large B cell to different treatment and better prognosis; however, lymphoma which forms 80% of all cases, however, other the symptoms of PPL are not specific and can mimic histologic types may rarely be seen (3). adenocarcinoma. PPL can often be seen as a mass larger Rad et al. (4) reported on patient with pancreatic mass than 5 cm in the pancreas while unlike adenocarcinoma, no and icter who had low grade B cell lymphoma in the vascular involvement is commonly observed. The definitive histological examination (Figures 1 and 2). diagnosis of PPL is impossible based on imaging and it requires pathological examination. CT-guided biopsy Clinical symptoms and laparotomy have been proposed for obtaining tissue samples. Fine needle aspiration (FNA) through endoscopic Clinical symptoms of PPL are not specific for the disease ultrasound (EUS) (EUS-FNA) has been a good method and include epigastric pain, abdominal mass, weight loss, © Journal of Gastrointestinal Oncology. All rights reserved. jgo.amegroups.com J Gastrointest Oncol 2017;8(4):749-757 750 Rad et al. PPL: what we need to know A B Figure 1 Low grade B cell lymphoma (hematoxylin-eosin stain; original magnification ×200). A B Figure 2 CD20-BCL2-CD10-CD43-CD3 positive atypical lymphocytic cells (immunohistochemistry; magnification, ×25). jaundice, nausea, vomiting, diarrhea, pancreatitis, and (IV) Pancreatic mass specified in surgery with the intestinal obstruction (5,6). involvement of lymph nodes confined to the PPLs rarely represent B symptoms which are often seen pancreas; in other lymphoma and include fever, night sweats, and (V) Lack of involvement of liver or spleen. weight loss. The most symptoms of PPLs are with vague abdominal discomforts such as dyspepsia, pain, nausea, Imaging techniques vomiting, feeling of fullness, and body weight loss (7). Dawson et al. proposed five criteria for the diagnosis of Transabdominal ultrasound (TUS) PPL (8): TUS is a technique dependent on the accuracy of operator (I) Lack of peripheral lymphadenopathy; and has a low precision to see small masses in the head of the (II) Lack of involvement of mediastinal lymph node; pancreas. TUS can indicate dilatation and obstruction of the (III) Counting normal peripheral white blood cells; bile ducts and liver metastases (9). Using new methods of © Journal of Gastrointestinal Oncology. All rights reserved. jgo.amegroups.com J Gastrointest Oncol 2017;8(4):749-757 Journal of Gastrointestinal Oncology Vol 8, No 4 August 2017 751 ultrasound such as Color-power Doppler us, 3 dimensional and focal nodular in T1W1 images and high or low signal (3D) US, and harmonic imaging, contrast-enhanced us, the intensity in T2W1 images. In DCE-MRI, low enhanced diagnosis of pancreatic masses has significantly enhanced (10). area surrounded by parenchyma is usually observed (5,18). Using contrast enhanced us, vascular involvement can Unlike CT, the lesions of pancreatic lymphoma are mildly be investigated in the pancreas masses that can help the heterogeneous in MRI, especially in T2W images. Tumors differentiation of pancreatic masses (11). of Islet cell have more hyper intensity than lymphoma in T2w images. Whole body DW1 had formerly a significant role in Abdominal CT scan diagnosing and staging the disease in lymphoma patients. To examine pancreas masses and liver metastases, CT scan By receiving intravenous gadolinium contrast, lymphoma which is an accessible and noninvasive method can be used is homogeneously enhanced in a less degree than normal and based on the results of CT scan, decisions can be made parenchyma. A number of lymphomas appear softly on sampling to prove the diagnosis. The quality of CT inhomogeneous in MRI. Due to the desmoplastic content, scan images has been improved using multiple detector CT the pancreas adenocarcinoma is less enhanced and after which creates images with high resolution 3-D imaging receiving gadolinium, they are typically inhomogeneous. and multiplanar ones. Rapid injection of iodine-containing Due to the difference in the treatment and prognosis of contrast and images that are immediately taken after pancreatic lymphoma, its differentiation from similar injection are among the methods to increase the sensitivity lesions such as pancreatic autoimmune and autoimmune of CT in the evaluation of pancreatic masses (11). CT pancreatitis seems to be of great significance (20). sensitivity is low for lesions less than 2 cm (12-14). Merkle et al. (5) offered the following criteria for CT scan guide can be used for the biopsy of pancreatic distinguishing lymphoma from pancreatic autoimmune: lesions with the sensitivity about 95% (15,16). Radiological (I) Huge homogeneous mass without cystic-necrosis results from the previous studies in which the secondary lesions or MPD involvement; lymphoma of the pancreas was similar to primary lymphoma (II) Adjacent arteries involvement without obstruction; include nodular, diffuse and multinodular (6,17,18). Most (III) If there were lymphadenopathy under the left renal masses have been recognized through intravenous injection vein, it would be limited to the area around the by CT scan well and sometimes are bulky and infiltrated. pancreas. Lesions with the homogeneous low attenuation are along However, despite the above criteria, CT-guided biopsy with a slight enhancement of pancreatic parenchyma (5). or EUS FNA is important for an accurate diagnosis in most Pancreatic lymphoma with a lower incidence may show cases. the symptoms of acute pancreatitis on CT scan which appears as diffuse pancreatic enlargement while the results EUS of typical pancreatitis in CT including inflammation around the pancreas or fat stranding do not exist; otherwise, they Although EUS is dependent on operator, it is more can be seen usually minimal. Fluid accumulation around sensitive than CT or MRI to detect suspicious pancreatic the pancreas, pancreatic fat necrosis and rupture of the lesions or lesions less than 2 cm on CT if it is done by an pancreatic duct are not seen in lymphoma. Vascular experienced person (12,14,21-23). Tissue sampling by EUS involvement by tumor can rarely be seen in lymphoma (5). can be done in two ways: either by EUS guided FNA (EUS- Although pressure may be observed on vessels due to the FNA) or EUS guided fine-needle core biopsy (EUS-FNB). effect of the mass, there are no changes resulting from the The sensitivity of EUSFNA is 95% and its specificity is conflict involvement of tumor including a change in caliber 100% (24-27). It is the preferred method for sampling and irregularity (19). the pancreatic tissue, especially when the biopsy results of other methods are negative or ambiguous for malignancy (28,29). Although EUSFNA along with cytopathology are MRI usually suitable for the diagnosis of adenocarcinoma and MRI is
Load more

Recommended publications
  • Autoimmune Pancreatitis: an Autoimmune Or Immunoinflammatory Disease?
    10 The Open Autoimmunity Journal, 2011, 3, 10-16 Open Access Autoimmune Pancreatitis: An Autoimmune or Immunoinflammatory Disease? Yvonne Hsieh, Sudhanshu Agrawal, Leman Yel and Sudhir Gupta* Division of Basic and Clinical Immunology, University of California, Irvine, CA 92697, USA Abstract: Autoimmune pancreatitis (AIP) has been widely presumed to be an autoimmune disease that is characterized by elevated IgG and/or IgG4, the presence of autoantibodies, and an infiltration of lymphocytes and plasma cells with fi- brosis. However, no detailed immunological studies have been published. To define immunological changes in AIP in de- tail, and to review evidence for autoimmunity which may be antigen specific and may play a role in the pathogenesis of AIP, and therefore, to determine whether AIP is an autoimmune disease. A detailed immunological investigation for both innate and adaptive immune responses was performed in a patient with AIP. Review of literature was performed from Pub med, and Medline search. Immunological analysis of patient with AIP revealed increased production of proinflammatory IL-6, and IL-17, and increased NK cell activity. No organ-specific or non-specific antibodies were detected. There was no correlation between serum IgG4 with disease activity or response to steroid therapy. Review of literature revealed lack of auto-antigen-specific T and B cell responses in AIP, and autoantibodies are present only in a subset of patients, and are not specific to pancreatic tissue antigens. Therefore, we propose the term Immunoinflammatory pancreatitis rather than an autoimmune pancreatitis. Keywords: NK cells, IL-6, IL-17, autoantibodies, IgG4. INTRODUCTION collectively has been suggested to increase the sensitivity of detection of the disease [6].
    [Show full text]
  • Steroid Therapy and Steroid Response in Autoimmune Pancreatitis
    International Journal of Molecular Sciences Review Steroid Therapy and Steroid Response in Autoimmune Pancreatitis Hiroyuki Matsubayashi 1,2,* , Hirotoshi Ishiwatari 1, Kenichiro Imai 1, Yoshihiro Kishida 1, Sayo Ito 1, Kinichi Hotta 1, Yohei Yabuuchi 1, Masao Yoshida 1, Naomi Kakushima 1, Kohei Takizawa 1, Noboru Kawata 1 and Hiroyuki Ono 1 1 Division of Endoscopy, Shizuoka Cancer Center 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan; [email protected] (H.I.); [email protected] (K.I.); [email protected] (Y.K.); [email protected] (S.I.); [email protected] (K.H.); [email protected] (Y.Y.); [email protected] (M.Y.); [email protected] (N.K.); [email protected] (K.T.); [email protected] (N.K.); [email protected] (H.O.) 2 Genetic Medicine Promotion, Shizuoka Cancer Center 1007, Shimonagakubo, Nagaizumi, Suntogun, Shizuoka 411-8777, Japan * Correspondence: [email protected]; Tel.: +81-55-989-5222; Fax: +81-55-989-5692 Received: 25 November 2019; Accepted: 25 December 2019; Published: 30 December 2019 Abstract: Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. AIP is classified into two distinct subtypes on the basis of the histological subtype: immunoglobulin G4 (IgG4)-related lymphoplasmacytic sclerosing pancreatitis (type 1) and idiopathic duct-centric pancreatitis (type 2). Type 1 AIP is often accompanied by systemic lesions, biliary strictures, hepatic inflammatory pseudotumors, interstitial pneumonia and nephritis, dacryoadenitis, and sialadenitis. Type 2 AIP is associated with inflammatory bowel diseases in approximately 30% of cases. Standard therapy for AIP is oral corticosteroid administration.
    [Show full text]
  • Autoimmune Pancreatitis in a Patient Presenting with Obstructive Jaundice and Pancreatic Mass
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE HPB 2004 Volume 6, Number 2 126±127 provided by Elsevier - Publisher Connector DOI 10.1080/13651820410026128 Case report Autoimmune pancreatitis in a patient presenting with obstructive jaundice and pancreatic mass Kevin Ooi and Neil Merrett Liverpool Hospital, New South Wales, Australia Background Discussion Autoimmune pancreatitis (AIP) is a rare cause of chronic Recognition of the disease by its typical radiological and pancreatitis. serological ®ndings permits trial of steroid therapy and may avoid resection. Case outline A case of obstructive jaundice with pancreatic mass mimicking Keywords malignancy is described. autoimmune pancreatitis, obstructive jaundice, pancreatic mass, steroid therapy Case report Postoperatively, the patient remains asymptomatic with normal liver functions. There is no serological evi- An abstinent 70-year-old Armenian lady presented with dence for other autoimmune diseases. Immunoglobulin G a 2-week history of jaundice, epigastric discomfort, (IgG) is 15.5 g/L (normal 6.2 14.4); IgG4 is 0.92 g/L anorexia and 4 kg weight loss. Background history – (normal 0.07 0.88). Insulin injections were no longer included insulin-dependent diabetes mellitus. Physical – necessary for diabetic control. examination was unremarkable. Liver function tests were deranged: AST 365 U/L (normal 7–40 U/L), ALT 700 U/L (normal 7–40 U/L), ALP 520 U/L (normal 39– Discussion 151 U/L), GGT 1640 U/L (normal 6–46 U/L), bilirubin 164 mmol/L (normal 0–18 mmol/L). Abdominal com- Pancreatitis associated with hypergammaglobulinaemia puted tomography (CT) (Figure 1) revealed dilated was ®rst reported in 1961, suggesting an immune common and intrahepatic bile ducts, thickened dis- component to some pancreatic disease [1].
    [Show full text]
  • Autoimmune Pancreatitis, Pancreatic Cancer and Immunoglobulin-G4
    JOP. J Pancreas (Online) 2010 Jan 8; 11(1):89-90. PANCREAS NEWS Autoimmune Pancreatitis, Pancreatic Cancer and Immunoglobulin-G4 Raffaele Pezzilli, Roberto Corinaldesi Department of Digestive Diseases and Internal Medicine, “Sant’Orsola-Malpighi” Hospital. Bologna, Italy The occurrence of pancreatic cancer associated with pancreatitis. Peptide-specific antibodies were detected autoimmune pancreatitis (AIP) has sometimes been in serum specimens obtained from the patients. Among reported [1, 2, 3, 4]. Of the four cases reported, the peptides detected, peptide AIP(1-7) was recognized pancreatic cancer was diagnosed simultaneously with by the serum specimens from 18 of 20 patients with AIP in two cases [1, 4]; one case of pancreatic cancer autoimmune pancreatitis and by serum specimens from developed five years after a pancreaticoduodenectomy 4 of 40 patients with pancreatic cancer, but not by for AIP [2] and in the other case, the pancreatic cancer serum specimens from healthy controls. The peptide developed three years after steroid therapy was begun showed homology with an amino acid sequence of the [3]. The possibility that pancreatic cancer may develop plasminogen-binding protein (PBP) of Helicobacter in AIP patients has recently been pointed out by pylori and with ubiquitin-protein ligase E3 component Kamisawa et al. [5]. These authors assessed the n-recognin 2 (UBR2), an enzyme highly expressed in possible relationship between AIP and pancreatic acinar cells of the pancreas. Antibodies against the PBP cancer by analyzing the K-ras mutation in the peptide were detected in 19 of 20 patients with pancreatobiliary tissue of patients with AIP and they autoimmune pancreatitis (95.0%) and in 4 of 40 found a significant K-ras mutation in the patients with pancreatic cancer (10.0%).
    [Show full text]
  • Autoimmune Pancreatitis: a Multiorgan Disease Presenting a Conundrum for Clinicians in the West
    Autoimmune Pancreatitis: A Multiorgan Disease Presenting a Conundrum for Clinicians in the West Eileen Kim, MD, Rebecca Voaklander, MD, Franklin E. Kasmin, MD, William H. Brown, MD, Rifat Mannan, MD, and Jerome H. Siegel, MD, RPh All of the authors are affiliated with Abstract: Autoimmune pancreatitis (AIP), a clinical entity originally Mount Sinai Beth Israel in New York, described in East Asia and more recently recognized in the United New York. Dr Kim and Dr Voaklander are States and Europe, poses a diagnostic conundrum for clinicians residents in the Department of Internal in the West due to immunoglobulin G4 seronegativity. Although Medicine. Dr Mannan is a resident in the Department of Pathology. Dr expert panels classify this disease into 2 types, it remains difficult Kasmin, Dr Brown, and Dr Siegel are to stratify the disease given that both types share most clinical, attending physicians in the Department biochemical, and imaging characteristics. The classic presentation of Internal Medicine and codirectors of of AIP can mimic that of pancreatic carcinoma, which increases the Advanced Fellowship in Therapeutic the urgency of evaluation, diagnosis, and treatment. In this article, Endoscopy. Dr Siegel is also a clinical we elucidate the differences between the 2 types of AIP, highlight professor of medicine at the Icahn School of Medicine at Mount Sinai in New York, the shortcomings of the current classification system, and propose New York. a more inclusive view of the disorder. Address correspondence to: Dr Jerome H. Siegel 305 Second Avenue, Suite #3 utoimmune pancreatitis (AIP), a clinical entity originally New York, NY 10003 described in East Asia1-3 and more recently recognized in the Tel: 212-734-8874 United States and Europe,4-6 poses a diagnostic conundrum Fax: 212-249-5628 Afor clinicians in the West due to the prevalence of immunoglobu- E-mail: [email protected] lin G4 (IgG4) seronegativity and extrapancreatic manifestations.
    [Show full text]
  • Autoimmune Pancreatitis, a Single Centre Experience Within The
    JOP. J Pancreas (Online) 2016 Jan 08; 17(1):98-101. CASE REPORT Acute Pancreatitis with Normal Serum Lipase and Amylase: A Rare Presentation Arvind K Mathur1, Angela Whitaker2, Hemchand Kolli1, Trinh Nguyen2 1 2 Hemet Valley Medical Center, 1117 East Devonshire Ave, Hemet CA 92545 Western University of Health Sciences, 309 E. Second St, Pomona CA 91766 ABSTRACT Acute pancreatitis presenting with normal serum amylase and lipase levels is a rare phenomenon. It is thought that typically, acute inflammation and auto-digestion of the pancreas leads to the release of both amylase and lipase, leading to elevated levels in the blood. For this reason, normal serum amylase and lipase levels in a patient with acute abdominal pain would typically rule out acute pancreatitis in favor of another diagnosis. Here we present two cases of acutely ill patients that were confirmed to have acute pancreatitis radiologically but with serum amylase and lipase levels that remained within the normal range throughout their illnesses for both patients. These cases equallysuggest withthat whilethe presenting an important signs, diagnostic symptoms, tool, and serum imaging amylase studies and to lipasehelp guide should toward not be a useddiagnosis. as the sole factor to either diagnose or rule out acute pancreatitis. Instead, these laboratory markers should be viewed in the context of the patient’s overall presentation, weighted INTRODUCTION numerous medical conditions, certain drugs or surgical throughout the digestive tract and may be elevated from can remain localized, involve regional and distant organs, LAP is an inflammatory process of the pancreas that procedures, or can remain normal in alcohol-induced withinpancreatitis the pancreatic or in cases acinar caused cells, by with hypertriglyceridemia lipase activity in or cause overwhelming illness or death.
    [Show full text]
  • Case Report: Painless Obstructive Jaundice Caused by Igg4
    F1000Research 2020, 9:1344 Last updated: 02 SEP 2021 CASE REPORT Case Report: Painless obstructive jaundice caused by IgG4 autoimmune pancreatitis; the role of endoscopic ultrasound in diagnosis [version 1; peer review: 2 not approved] Sofia Voidila 1, Panagiotis Sideris2, Constantinos Letsas3, Elias Anastasopoulos4, Ioanna Oikonomou5 1Radiology Department, General Hospital of Anatoliki Achaia, Poulitsa Korinthias, 20006, Greece 2Internal Medicine, General Hospital of Anatoliki Achaia, Aigio, 25100, Greece 3Pathology Department, General Hospital of Anatoliki Achaia, Aigio, 25100, Greece 4Department of Gastrenterology, Iaso General Hospital, Athens, 15239, Greece 5General Hospital of Anatoliki Achaia, Aigio, 25100, Greece v1 First published: 18 Nov 2020, 9:1344 Open Peer Review https://doi.org/10.12688/f1000research.27017.1 Latest published: 18 Nov 2020, 9:1344 https://doi.org/10.12688/f1000research.27017.1 Reviewer Status Invited Reviewers Abstract We report the case of a 60-year-old woman, presenting with painless 1 2 obstructive jaundice of unknown etiology, who was finally found to suffer from type I autoimmune pancreatitis (AIP). This case version 1 emphasizes AIP as a rare cause in the differential diagnosis of 18 Nov 2020 report report obstructive jaundice and the role of endoscopic ultrasound (EUS) in final diagnosis, which is difficult to establish. According to diagnostic 1. Shin Miura , Tohoku University Graduate criteria, we combined the results from serologic, imaging and histological features (specifically lgG4 levels, computed tomography, School of Medicine, Sendai, Japan magnetic resonance imaging/magnetic resonance 2. Zaheer Nabi, Asian Institute of cholangiopancreatography and EUS) with cytological results, leading to a final diagnosis. Our patient’s response to corticosteroids was Gastroenterology, Hyderabad, India impressive, confirming the diagnosis, leading to complete remission of the disease.
    [Show full text]
  • Diagnosis and Management of Igg4 Autoimmune Pancreatitis
    JOP. J Pancreas (Online) 2015 Jul 08; 16(4):326-334 REVIEW ARTICLE Review Article: Diagnosis and Management of Igg4 Autoimmune Pancreatitis Ahmed Salem, Diaa Hamouda, Alyssa Parian Johns Hopkins University School of Medicine, Baltimore, Maryland, USA ABSTRACT Autoimmune pancreatitis is a rare form of chronic pancreatitis that has only recently been recognized as a separate type of pancreatitis French Henry Sarles. in the last two decades. The histopathological features of this distinct form of pancreatitis was first described as early as 1961 when the INTRODUCTION Autoimmune pancreatitis (AIP) is a rare form of chronic [8] found intensely positive IgG4 cells in extrapancreatic pancreatitis that has only recently been recognized as organ systems in AIP patients. Thus, the concept of IgG4- a separate type of pancreatitis in the last two decades. diseaserelated systemicwhereas typedisease 2 AIPemerged. appears Type to be1 AIPa pancreas is now The histopathological features of this distinct form of considered to be a pancreatic manifestation IgG4-related specificEPIDEMIOLOGY disorder. pancreatitis was first described as early as 1961 when pancreatitis associated with hypergammaglobulinemia. Up till now the true incidence of AIP is in the United the French Henry Sarles [1] described a type of sclerosing Subsequently, most of the early literature about AIP came States is unknown with reports limited to case series from Japan where the concept of Autoimmune Pancreatitis and descriptions of tertiary referrals. The estimated et al. many authors had reported a form of chronic pancreatitis (AIP)was first proposed in 1995 by Yoshida [2] after estimatedprevalence the in prevalenceJapan, where of autoimmune AIP was first pancreatitis described, in AIP was widely accepted and AIP was differentiated from is 0.82 per 100,000 persons [9].
    [Show full text]
  • Autoimmune Pancreatitis/Igg4-Associated Cholangitis
    Journal of Hepatology 51 (2009) 398–402 www.elsevier.com/locate/jhep Review Autoimmune pancreatitis / IgG4-associated cholangitis and primary sclerosing cholangitis – Overlapping or separate diseases?q George J.M. Webster1,2,*, Stephen P. Pereira1,2, Roger W. Chapman3 1Department of Gastroenterology, University College Hospital, 235 Euston Road, London NW1, UK 2Institute of Hepatology, University College London, London, UK 3Department of Gastroenterology and Hepatology, The John Radcliffe Hospital, Oxford, UK Autoimmune pancreatitis is a recently described fibroinflammatory disease which is characterised by raised serum levels of IgG4 (in >70% of cases), and an IgG4-positive lymphoplasmacytic tissue infiltrate. A favourable and rapid clinical response to oral steroid therapy is often seen. Biliary involvement is common, and the term IgG4-associated cholangitis has recently been coined. The cholangiographic appearances of IgG4-associated cholangitis and primary sclerosing cho- langitis can be difficult to differentiate. Moreover, raised levels of serum IgG4 have been recently found in 9% of patients with primary sclerosing cholangitis (a much higher frequency than for other gastrointestinal diseases), and those with raised levels appear to progress more rapidly to liver failure. Here we review the similarities and differences between the biliary disease in autoimmune pancreatitis and primary sclerosing cholangitis, and address the issue of disease overlap. Improvements in understanding the relationship between these conditions might lead to an enhanced understanding of the aetiopathogenesis, and improved treatment of both conditions. Ó 2009 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved. Keywords: Autoimmune pancreatitis; Primary sclerosing cholangitis; Immunoglobulin G4; IgG4-associated cholangitis 1.
    [Show full text]
  • Autoimmune Pancreatitis: Current Concepts
    SCIENCE CHINA Life Sciences • REVIEW • March 2013 Vol.56 No.3: 246–253 doi: 10.1007/s11427-013-4450-z Autoimmune pancreatitis: current concepts WANG Qian, ZHANG Xuan* & ZHANG FengChun Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China Received August 5, 2012; accepted October 9, 2012 Autoimmune pancreatitis (AIP) is a distinct type of chronic pancreatitis with unique clinical, pathological, serological, and imaging features. AIP usually presents with obstructive jaundice. Imaging studies often reveal enlargement of the pancreas with a pancreatic mass and strictures of the main pancreatic duct. Two subtypes of AIP have recently been identified. Type I AIP is more prevalent in elderly Asian males and is characterized by lymphoplasmacytic sclerosing pancreatitis, obliterative phlebitis, and infiltration of large numbers of IgG4-positive plasma cells. Type II AIP is more prevalent in Caucasians and is characterized by granulocyte epithelial lesions. Most patients with type I AIP have a significantly elevated serum IgG4 con- centration, which is an important feature for diagnosis and for differentiating between AIP and other conditions such as pan- creatic cancer. Extrapancreatic complications are common, such as sclerosing cholangitis, sclerosing sialadenitis, retroperito- neal fibrosis in type I AIP, and ulcerative colitis in type II AIP. A rapid response to glucocorticoids treatment is suggestive of AIP, but the relapse rate is high, warranting the use of immunosuppressant treatment. B-cell depletion with rituximab may be a promising therapy. The prognosis of AIP is generally benign if treated promptly, and spontaneous remission occurs in a pro- portion of patients.
    [Show full text]
  • Autoimmune Pancreatitis Presenting As a Pancreatic Mass Mimicking Malignancy Lo R S C, Singh R K, Austin a S, Freeman J G
    Case Report Singapore Med J 2011; 52(4) : e79 Autoimmune pancreatitis presenting as a pancreatic mass mimicking malignancy Lo R S C, Singh R K, Austin A S, Freeman J G ABSTRACT Autoimmune pancreatitis is a rare cause of chronic pancreatitis and pancreatic mass. We describe a case of focal autoimmune pancreatitis in a 51-year-old man presenting with obstructive jaundice and pancreatic mass, mimicking malignancy. The immunological test was suggestive of autoimmune pancreatitis, and the patient responded well to a course of steroids, with complete resolution of the pancreatic mass. Autoimmune pancreatitis, therefore, must be kept in mind as a differential diagnosis of Fig. 1 CT image of the abdomen shows a pancreatic mass pancreatic mass. Recognition of this disease by its (arrow). typical radiological and serological findings may help to avoid unnecessary surgical resection. Blood test revealed a mixed hepatitic and obstructive picture (bilirubin 130 μmol/L, alkaline phosphatase Keywords: autoimmune diseases, chronic 540 U/L, alanine transaminase 357 U/L, aspartate pancreatitis, obstructive jaundice, pancreatic transaminase 123 U/L, γGT 1117 U/L). Liver synthetic neoplasm, steroids functions were normal (albumin 40 g/dl and international Singapore Med J 2011; 52(4): e79-e81 normalised ratio 0.9). A full screening confirmed exclusion of infective, autoimmune and metabolic INTRODUCTION causes of liver disease. Ultrasonography and subsequent Autoimmune pancreatitis (AIP) is a form of chronic computed tomography (CT) of the abdomen revealed pancreatitis of presumed autoimmune aetiology.(1) It was a large, solid enhancing mass lesion in the head of the first described by Yoshida et al in 1995.(2) The number pancreas measuring 6.0 cm × 4.0 cm × 4.6 cm, with of cases has since increased due to the increasing ability involvement of the uncinate process.
    [Show full text]
  • Acute Pancreatitis: Genetic Risk and Clinical Implications
    Journal of Clinical Medicine Review Acute Pancreatitis: Genetic Risk and Clinical Implications Frank U. Weiss * , Felix Laemmerhirt and Markus M. Lerch Department of Medicine A, University Medicine Greifswald, 17475 Greifswald, Germany; [email protected] (F.L.); [email protected] (M.M.L.) * Correspondence: [email protected]; Tel.: +49-038-3486-7233 Abstract: Acute pancreatitis (AP) is one of the most common gastroenterological indications for emergency admittance and hospitalization. Gallstones, alcohol consumption or the presence of additional initiating factors give rise to a disease with a diverse clinical appearance and a hard-to predict course of progression. One major challenge in the treatment of AP patients is the early identification of patients at risk for the development of systemic complications and organ failure. In addition, 20%–30% of patients with a first episode of AP later experience progress to recurrent or chronic disease. Complex gene–environment interactions have been identified to play a role in the pathogenesis of pancreatitis, but so far no predictive genetic biomarkers could be implemented into the routine clinical care of AP patients. The current review explains common and rare etiologies of acute pancreatitis with emphasis on underlying genetic aberrations and ensuing clinical management. Keywords: acute pancreatitis; genetic risk; diagnosis; disease severity; progression 1. Introduction Pancreatitis, a primarily sterile inflammatory condition of the pancreas, is frequently triggered by gallstones, alcohol consumption or the presence of a variety of other initiating factors. Complex gene–environment interactions are involved in the pathogenesis of Citation: Weiss, F.U.; Laemmerhirt, pancreatitis, giving rise to a diverse clinical appearance and a sometimes hard-to predict F.; Lerch, M.M.
    [Show full text]