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Dugic A, Verdejo Gil C, Mellenthin C, Vujasinovic M, Löhr JM, Mühldorfer S. The Clinical Utility of Soluble Serum Biomarkers in Autoimmune Pancreatitis: A Systematic Review. Biomedicines 2022; 10:1511. [PMID: 35884816 PMCID: PMC9312496 DOI: 10.3390/biomedicines10071511] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2022] [Revised: 06/17/2022] [Accepted: 06/21/2022] [Indexed: 11/17/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare etiological type of chronic pancreatitis. The clinical and radiological presentation of AIP often resembles that of pancreatic cancer. Identifying non-invasive markers for their early distinction is of utmost importance to avoid unnecessary surgery or a delay in steroid therapy. Thus, this systematic review was conducted to revisit all current evidence on the clinical utility of different serum biomarkers in diagnosing AIP, distinguishing AIP from pancreatic cancer, and predicting disease course, steroid therapy response, and relapse. A systematic review was performed for articles published up to August 2021 by searching electronic databases such as MEDLINE, Web of Science, and EMBASE. Among 5123 identified records, 92 studies were included in the qualitative synthesis. Apart from immunoglobulin (Ig) G4, which was by far the most studied biomarker, we identified autoantibodies against the following: lactoferrin, carboanhydrase II, plasminogen-binding protein, amylase-α2A, cationic (PRSS1) and anionic (PRSS2) trypsinogens, pancreatic secretory trypsin inhibitor (PSTI/SPINK1), and type IV collagen. The identified novel autoantigens were laminin 511, annexin A11, HSP-10, and prohibitin. Other biomarkers included cytokines, decreased complement levels, circulating immune complexes, N-glycan profile changes, aberrant miRNAs expression, decreased IgA and IgM levels, increased IgE levels and/or peripheral eosinophil count, and changes in apolipoprotein isoforms levels. To our knowledge, this is the first systematic review that addresses biomarkers in AIP. Evolving research has recognized numerous biomarkers that could help elucidate the pathophysiological mechanisms of AIP, bringing us closer to AIP diagnosis and its preoperative distinction from pancreatic cancer.
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Affiliation(s)
- Ana Dugic
- Department of Gastroenterology, Klinikum Bayreuth, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Medizincampus Oberfranken, 95445 Bayreuth, Germany;
- Faculty of Medicine, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Schloßplatz 4, 91054 Erlangen, Germany
| | - Cristina Verdejo Gil
- Department of Gastroenterology, Hospital Universitario Fundación de Alcorcón, 28922 Madrid, Spain;
| | | | - Miroslav Vujasinovic
- Department of Upper Abdominal Diseases, Karolinska University Hospital, 14186 Stockholm, Sweden;
- Department of Medicine, Huddinge, Karolinska Institutet, 14186 Stockholm, Sweden;
| | - J.-Matthias Löhr
- Department of Medicine, Huddinge, Karolinska Institutet, 14186 Stockholm, Sweden;
- Department of Clinical Science, Intervention, and Technology (CLINTEC), Karolinska Institutet, 14186 Stockholm, Sweden
| | - Steffen Mühldorfer
- Department of Gastroenterology, Klinikum Bayreuth, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Medizincampus Oberfranken, 95445 Bayreuth, Germany;
- Faculty of Medicine, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Schloßplatz 4, 91054 Erlangen, Germany
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Lanzillotta M, Tacelli M, Falconi M, Arcidiacono PG, Capurso G, Della-Torre E. Incidence of endocrine and exocrine insufficiency in patients with autoimmune pancreatitis at diagnosis and after treatment: a systematic review and meta-analysis. Eur J Intern Med 2022; 100:83-93. [PMID: 35367110 DOI: 10.1016/j.ejim.2022.03.014] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/14/2022] [Revised: 03/09/2022] [Accepted: 03/11/2022] [Indexed: 11/03/2022]
Abstract
BACKGROUND Autoimmune pancreatitis (AIP) is a rare form of pancreatitis that may lead to endocrine and exocrine insufficiency if left untreated. AIP clinically responds to glucocorticoids (GCs) therapy, but multiple GCs courses are often required to maintain remission with detrimental effects on glycaemic control. OBJECTIVE In this systematic review and meta-analysis, we aimed to assess the rate of endocrine and of exocrine insufficiency at diagnosis and at follow up in patients with AIP as well as the impact of GC therapy on pancreatic function in the long-term. METHODS The MEDLINE, SCOPUS, and EMBASE databases were searched from inception to August 2021 to identify studies reporting data on endocrine and exocrine insufficiency in patients with AIP. Pooled events were calculated using a random-effect model and expressed in terms of pooled prevalence rates. RESULTS A total of 6522 AIP patients and sixty-two studies were included in the analysis. The pooled estimate rate for the overall prevalence of diabetes in AIP at baseline was 37% (95% CI 32-42, I2 96%). The pooled prevalence rate of exocrine insufficiency was 45% (95%CI 32.9-57.4; I2 97%). The pooled estimate rate of diabetes at follow-up was 44% (95%CI 26.1-62.4) in studies where GCs were given to 100% of patients and 42% (95%CI 30.6-52.9) in studies where GCs were given to less than 100% of patients. CONCLUSION A large proportion of patients with AIP displays concomitant exocrine and endocrine insufficiency at the time of diagnosis. The incidence of diabetes at the longest available follow up tends to increase in patients treated with GCs.
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Affiliation(s)
- Marco Lanzillotta
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy.
| | - Matteo Tacelli
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Massimo Falconi
- Division of Pancreatic Surgery and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Paolo Giorgio Arcidiacono
- Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Gabriele Capurso
- Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Emanuel Della-Torre
- Università Vita-Salute San Raffaele, IRCCS San Raffaele Scientific Institute, Milan, Italy; Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy
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Ergin E, Oruc N, Özütemiz Ö. Autoimmune Pancreatitis after a Seven-Year History of Suspicious Pancreatic Cancer. Case Rep Gastroenterol 2021; 15:195-201. [PMID: 33790705 PMCID: PMC7989778 DOI: 10.1159/000511286] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/09/2020] [Accepted: 08/31/2020] [Indexed: 11/19/2022] Open
Abstract
In this case report, we present a case of autoimmune pancreatitis (AIP) diagnosis in a patient after a 7-year history of suspicious pancreatic cancer. Kim's and Japanese criteria were used to diagnose AIP. Our case avoided undesirable invasive procedures and recovered thanks to the proper diagnosis and timely treatment with prednisone. Early and accurate diagnosis of AIP, in this case, had a significant impact on the treatment and prognosis process.
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Affiliation(s)
- Erhan Ergin
- Gastroenterology Department, Faculty of Medicine, Ege University, Izmir, Turkey
| | - Nevin Oruc
- Gastroenterology Department, Faculty of Medicine, Ege University, Izmir, Turkey
| | - Ömer Özütemiz
- Gastroenterology Department, Faculty of Medicine, Ege University, Izmir, Turkey
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Liu M, Hao M. Unique properties of IgG4 antibody and its clinical application in autoimmune pancreatitis. Scand J Gastroenterol 2018; 53:1121-1131. [PMID: 30175675 DOI: 10.1080/00365521.2018.1476915] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
BACKGROUND Autoimmune pancreatitis (AIP) is defined as a unique form of chronic pancreatitis characterized by clinical presentation with obstructive jaundice, a dense lymphoplasmacytic infiltrate and fibrosis histologically, and a dramatic response to steroids therapeutically. The possible role of IgG4 in driving the pathology of AIP is a controversial subject that has not been addressed satisfactorily. Objective: The purpose of this review is to discuss the unique biology of IgG4 that are important for its role and the clinical applications for serologic detection. METHODS Review of current literature about IgG4 antibody in the clinical application in AIP. RESULTS High serum levels of IgG4 are an important biomarker and broadly used for diagnosis, differentiation from diseases especially pancreatic cancer, and as a parameter to indicate disease activity, extra-pancreatic lesions, and treatment monitoring. However, some controversial studies show it has a limited specificity and sensitivity in these conditions. Conclusion: Although increasing studies have promoted our understanding of the structure and function of IgG4, there is still dilemma between the beneficial and the adverse aspect of IgG4 in the pathogenesis of AIP.
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Affiliation(s)
- Min Liu
- a Department of Clinical Laboratory , Jinan Dermatosis Prevention and Control Hospital , Jinan , People's Republic of China
| | - Mingju Hao
- b Department of Clinical Laboratory , Qianfo Mountain Hospital of Shandong University , Jinan , People's Republic of China
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Abstract
OBJECTIVES Serum IgG4 level is a useful diagnostic marker of autoimmune pancreatitis (AIP); however, it is difficult to predict relapse. We investigated whether a change in serum IgG4 is predictive of AIP relapse during maintenance therapy. METHODS Seventy-three patients with AIP treated with steroids were divided into 3 groups according to their initial serum IgG4 levels: level 1 group (>2-fold upper limit), level 2 group (1- to 2-fold upper limit), and a normal group. The relapse rate and the prevalence of extrapancreatic lesions were compared between the 3 groups. The correlation between the relative rise of serum IgG4 levels and relapse during maintenance therapy was analyzed. RESULTS There were no differences in serum IgG4 levels in the presence or absence of relapse. The average number of extrapancreatic lesions was 1.02, 0.47, and 0.27 in level 1, level 2, and the normal group, respectively. The relative rise of serum IgG4 levels after steroid therapy was significantly higher in relapse than in nonrelapse cases. The area under the receiver operating characteristic curve of the relative rise of IgG4 levels for discriminating between the relapse and the nonrelapse cases was 0.85. CONCLUSIONS The relative rise of serum IgG4 levels after steroid therapy may provide an indication of relapse.
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Yan T, Ke Y, Chen Y, Xu C, Yu C, Li Y. Serological characteristics of autoimmune pancreatitis and its differential diagnosis from pancreatic cancer by using a combination of carbohydrate antigen 19-9, globulin, eosinophils and hemoglobin. PLoS One 2017; 12:e0174735. [PMID: 28369140 PMCID: PMC5378371 DOI: 10.1371/journal.pone.0174735] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2016] [Accepted: 03/14/2017] [Indexed: 12/17/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis, which may be misdiagnosed as pancreatic carcinoma. This study aims to verify new biomarkers for AIP and propose a serological pattern to differentiate AIP from pancreatic adenocarcinoma with routinely performed tests. In this study, data of serum samples were collected and compared between 25 patients with AIP and 100 patients with pancreatic carcinoma. Receiver operating characteristic analysis and logistic regression was performed to evaluate the diagnostic effect of serum parameters in differentiating AIP from pancreatic carcinoma alone or in combination. Among several serum markers observed in the two groups, carbohydrate antigen 19-9 (Ca19-9), globulin, eosinophils and hemoglobin were selected as the independent markers. Serum levels of Globulin, Eosinophil percentage in AIP group were significantly higher than in pancreatic cancer group (P<0.05), while hemoglobin and tumor marker CA19-9 levels were lower (P <0.05). The combination of these markers identified patients with AIP with 92% sensitivity and 79% specificity, which indicated relatively high diagnostic value. Elevated serum eosinophils, globulin, together with decreased hemoglobin level can be used as a preoperative indicator for AIP and can help to initiate diagnosis of AIP in time.
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Affiliation(s)
- Tianlian Yan
- Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Yini Ke
- Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Yi Chen
- Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Chengfu Xu
- Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Chaohui Yu
- Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Youming Li
- Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
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Sekiguchi H, Horie R, Kanai M, Suzuki R, Yi ES, Ryu JH. IgG4-Related Disease: Retrospective Analysis of One Hundred Sixty-Six Patients. Arthritis Rheumatol 2016; 68:2290-9. [DOI: 10.1002/art.39686] [Citation(s) in RCA: 84] [Impact Index Per Article: 9.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2015] [Accepted: 03/15/2016] [Indexed: 12/19/2022]
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Katabathina VS, Dasyam AK, Dasyam N, Hosseinzadeh K. Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics 2015; 34:565-86. [PMID: 24819781 DOI: 10.1148/rg.343125211] [Citation(s) in RCA: 68] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Bile duct strictures in adults are secondary to a wide spectrum of benign and malignant pathologic conditions. Benign causes of bile duct strictures include iatrogenic causes, acute or chronic pancreatitis, choledocholithiasis, primary sclerosing cholangitis, IgG4-related sclerosing cholangitis, liver transplantation, recurrent pyogenic cholangitis, Mirizzi syndrome, acquired immunodeficiency syndrome cholangiopathy, and sphincter of Oddi dysfunction. Malignant causes include cholangiocarcinoma, pancreatic adenocarcinoma, and periampullary carcinomas. Rare causes include biliary inflammatory pseudotumor, gallbladder carcinoma, hepatocellular carcinoma, metastases to bile ducts, and extrinsic bile duct compression secondary to periportal or peripancreatic lymphadenopathy. Contrast material-enhanced magnetic resonance (MR) imaging with MR cholangiopancreatography is extremely helpful in the noninvasive evaluation of patients with obstructive jaundice, an obstructive pattern of liver function, or incidentally detected biliary duct dilatation. Some of these conditions may show characteristic findings at MR imaging-MR cholangiopancreatography that help in making a definitive diagnosis. Although endoscopic retrograde cholangiopancreatography with tissue biopsy or surgery is needed for the definitive diagnosis of many of these strictures, certain MR imaging characteristics of the narrowed segment (eg, thickened wall, long-segment involvement, asymmetry, indistinct outer margin, luminal irregularity, hyperenhancement relative to the liver parenchyma) may favor a malignant cause. Awareness of the various causes of bile duct strictures in adults and familiarity with their appearances at MR imaging-MR cholangiopancreatography are important for accurate diagnosis and optimal patient management.
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Affiliation(s)
- Venkata S Katabathina
- From the Department of Radiology, University of Texas Health Science Center at San Antonio, San Antonio, Tex (V.S.K.); and Department of Radiology, University of Pittsburgh Medical Center, Presby South Tower, Suite 4895, 200 Lothrop St, Pittsburgh, PA 15213 (A.K.D., N.D., K.H.)
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Fritz S, Bergmann F, Grenacher L, Sgroi M, Hinz U, Hackert T, Büchler MW, Werner J. Diagnosis and treatment of autoimmune pancreatitis types 1 and 2. Br J Surg 2014; 101:1257-65. [DOI: 10.1002/bjs.9574] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2013] [Revised: 01/31/2014] [Accepted: 04/24/2014] [Indexed: 01/05/2023]
Abstract
Abstract
Background
Autoimmune pancreatitis (AIP) is characterized by diffuse or focal swelling of the pancreas. AIP has been divided into types 1 and 2. The aim of the study was to evaluate and compare the clinicopathological characteristics, therapy and outcome of patients with AIP.
Methods
The medical records of patients diagnosed with AIP between January 2003 and July 2011 were reviewed. Characteristics of patients with AIP types 1 and 2 were compared with those of patients with pancreatic ductal adenocarcinoma (PDAC).
Results
AIP was classified as type 1 in 40 patients and type 2 in 32 according to the HISORt (Histology, Imaging, Serology, Other organ involvement, Response to therapy) criteria. Patients with histologically confirmed AIP type 2 were younger than those with type 1 (P = 0·005). Some 30 of 32 patients with AIP type 2 were found to have a localized tumour-like pancreatic mass and underwent pancreatectomy, compared with only 16 of 40 with type 1 (P < 0·001). Three of 25 patients with AIP type 2 presented with raised serum levels of IgG4 compared with 21 of 38 with type 1 (P < 0·001). There was no difference in symptoms and involvement of other organs between AIP types 1 and 2. Presentation with weight loss was more common among patients with PDAC than those with AIP, but there was no difference in pain or jaundice between the groups. Raised serum carbohydrate antigen 19-9 levels were more prevalent in patients with PDAC.
Conclusion
Patients with AIP type 2 frequently present with abdominal pain and a tumour-like mass. Differentiating AIP from PDAC is difficult, so making the clinical decision regarding operative versus conservative management is challenging.
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Affiliation(s)
- S Fritz
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - F Bergmann
- Department of Pathology, University of Heidelberg, Heidelberg, Germany
| | - L Grenacher
- Diagnostic and Interventional Radiology, University of Heidelberg, Heidelberg, Germany
| | - M Sgroi
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - U Hinz
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - T Hackert
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - M W Büchler
- Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany
| | - J Werner
- Department of General, Visceral and Transplantation Surgery, Ludwig-Maximilians University of Munich, Munich, Germany
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Bosco JJ, Suan D, Varikatt W, Lin MW. Extra-pancreatic manifestations of IgG4-related systemic disease: a single-centre experience of treatment with combined immunosuppression. Intern Med J 2014; 43:417-23. [PMID: 23013529 DOI: 10.1111/j.1445-5994.2012.02964.x] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/19/2011] [Accepted: 08/23/2012] [Indexed: 12/24/2022]
Abstract
BACKGROUND/AIM IgG4-related systemic disease (IgG4-RSD) is a systemic inflammatory disease distinguished by tissue infiltrates of IgG4(+) plasma cells and elevated serum IgG4 levels. While IgG4-RSD often involves the pancreas, extra-pancreatic organs are also frequently affected. Here, we review the presentation and management of patients with extra-pancreatic IgG4-RSD. METHODS A retrospective analysis was performed on patients diagnosed with extra-pancreatic IgG4-RSD identified from a single centre. RESULTS Six patients with extra-pancreatic IgG4-RSD were identified. The median age of the patients was 64 years. The range of involved organs included lymph nodes (three patients), ocular adnexa, lung, kidneys, meninges and exocrine glands. The median delay in diagnosis was 13.5 months (4-60 months). Four patients had elevated serum IgG4 levels at diagnosis. Five symptomatic patients were commenced on combination immunosuppression, which included corticosteroids. Maintenance therapy with azathioprine was used in one patient, methotrexate and mycophenolate were each used in two patients, and cyclophosphamide in one patient. Four treated patients went into remission, while two patients had persistent radiological disease. One patient experienced two relapses. CONCLUSION IgG4-RSD can manifest in a variety of organs. Lack of awareness regarding this entity may delay diagnosis. Combination treatment of corticosteroids and conventional immunosuppression is effective.
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Affiliation(s)
- J J Bosco
- Department of Clinical Immunology and Allergy, Westmead Hospital, Sydney, New South Wales, Australia.
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Clinical features of IgG4-related dacryoadenitis. Graefes Arch Clin Exp Ophthalmol 2013; 252:491-7. [DOI: 10.1007/s00417-013-2541-y] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2013] [Revised: 11/18/2013] [Accepted: 11/20/2013] [Indexed: 12/24/2022] Open
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PLANNER AC, BUNGAY HK. Autoimmune pancreatitis and IgG4 associated sclerosing cholangitis. IMAGING 2013. [DOI: 10.1259/imaging/52857384] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
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Kuruma S, Kamisawa T, Tabata T, Hara S, Fujiwara T, Kuwata G, Egarashira H, Koizumi K, Setoguchi K, Fujiwara J, Arakawa T, Momma K, Mitsuhashi T, Sasaki T. Clinical Characteristics of Patients with Autoimmune Pancreatitis with or without Mikulicz's Disease and Mikulicz's Disease Alone. Gut Liver 2012; 7:96-9. [PMID: 23422705 PMCID: PMC3572327 DOI: 10.5009/gnl.2013.7.1.96] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2012] [Accepted: 02/09/2012] [Indexed: 12/24/2022] Open
Abstract
Background/Aims The objective of this study was to compare the clinical characteristics of patients with autoimmune pancreatitis (AIP) with or without Mikulicz's disease (MD) and with MD alone. Methods We investigated the clinical findings in 15 AIP patients with MD (group A+M), 49 AIP only patients (group A), and 14 MD only patients (group M). Results The male-female ratio was significantly higher in group A+M (73%, p<0.05) and group A (78%, p<0.01) than group M (21%). Serum immunoglobulin G (IgG) levels were significantly higher in group A+M than in group A (p<0.01) and group M (p<0.05). Serum IgG4 levels were significantly higher in group A+M than in group A (p<0.01). Other organ involvement was observed in 73% (11/15) of patients in group A+M. The number of patients with diabetes mellitus was significantly higher in group A+M (66%, p<0.01) and group A (51%, p<0.05) than in group M (7%). All of the patients responded well to steroid therapy, but the relapse rate in group A+M (33%) was significantly higher than that in group A (3%, p<0.01). Salivary gland function was impaired in all groups compared with the control group, but the degree of dysfunction was less in group A compared with group A+M and group M. Conclusions The relapse rate of AIP in MD patients was significantly higher than that of AIP in patients without MD.
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Affiliation(s)
- Sawako Kuruma
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
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Zhang X, Zhang X, Li W, Jiang L, Zhang X, Guo Y, Wang X. Clinical analysis of 36 cases of autoimmune pancreatitis in China. PLoS One 2012; 7:e44808. [PMID: 23028628 PMCID: PMC3445578 DOI: 10.1371/journal.pone.0044808] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2012] [Accepted: 08/14/2012] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND To improve the early identification of autoimmune pancreatitis in China by a retrospective analysis of clinical data from AIP patients. METHODOLOGY/PRINCIPAL FINDINGS The analysis included 36 patients admitted by the surgery department of our hospital from January 2003 to October 2011 whose postoperative pathological confirmations were consistent with the histological criteria of Honolulu Consensus Document. The clinical phenotypes associated with the histopathologic patterns of LPSP and IDCP were referred to as type 1 and type 2 of AIP, respectively. A retrospective analysis of clinical features, serological data, pathological findings and imageological records was performed in line with the subtypes of AIP. Type 1 showing a sex predilection (males) was commonly more dominant than type 2 in all AIP. Type 2 without a gender predilection was, on average, a decade younger than type 1. Type 1 was inferior to type 2 in ALT, ALP and γ-GT with statistical significance (P = 0.044, 0.025 and 0.013). Type 1 was inferior to type 2 in AST with difference close to statistical significance (P = 0.072). Histopathology revealed frequent lymphoplasmacytic infiltration with less frequent infiltration of neutrophils, eosinophils and fibroblasts. Diffuse and intensive interstitial fibrosis could be seen. The changes of pancreatic head were more frequently seen in type 2 than in type 1 (P = 0.05). Plasma cells staining of IgG4 at a density of over 30 or more cells per high-power field appeared to be a specific finding in China with type 1. Imageology found a diffusely or focally enlarged pancreas, most frequently a mass or enlargement in the pancreatic head, characteristic capsule-like rim, calcification or pancreatic calculus and cystic degeneration. CONCLUSIONS/SIGNIFICANCE AIP is a unique type of chronic pancreatitis and has distinctive serological, pathological and imageological characteristics, which should be used for differentiation from pancreatic cancer.
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Affiliation(s)
- Xingang Zhang
- Department of Rheumatology, Shengjing Hospital of China Medical University, Shenyang, Liaoning Province, China.
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Terzin V, Földesi I, Kovács L, Pokorny G, Wittmann T, Czakó L. Association between autoimmune pancreatitis and systemic autoimmune diseases. World J Gastroenterol 2012; 18:2649-2653. [PMID: 22690073 PMCID: PMC3370001 DOI: 10.3748/wjg.v18.i21.2649] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/05/2011] [Revised: 01/05/2012] [Accepted: 04/10/2012] [Indexed: 02/06/2023] Open
Abstract
AIM To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sjögren's syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimoto's thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.
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Distinguishing autoimmune pancreatitis from pancreaticobiliary cancers: current strategy. Ann Surg 2012; 255:248-58. [PMID: 21997803 DOI: 10.1097/sla.0b013e3182324549] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
OBJECTIVE A review of the literature to identify current modalities for the diagnosis of autoimmune pancreatitis (AIP) with the objective of establishing a strategy to distinguish it from pancreaticobiliary cancers. BACKGROUND Pancreatic and biliary manifestations of AIP mimic pancreaticobiliary cancers. Misdiagnosis of AIP can result in major surgery for a steroid-responsive disease. METHODS A review of the literature was performed to identify recent advances in the diagnosis of AIP and evaluate outcomes with various diagnostic strategies to minimize operative intervention for an autoimmune disease. RESULTS Diagnostic criteria for AIP are based on histology, imaging, serology, extrapancreatic organ involvement, and response to steroid therapy. The most commonly involved extrapancreatic sites are bile duct, kidney, and retroperitoneum. The Mayo Clinic diagnostic strategy utilizes core biopsy of the pancreas and the Japanese strategy depends on a characteristic pancreatogram. The rate of operative intervention was similar with both strategies and none of the patients with cancer received steroid therapy. Immunoglobulin G subtype 4 (IgG4)-associated cholangitis mimics cholangiocarcinoma and presence of more than 10 IgG4-positive plasma cells/high power field on endoscopic biopsy of the bile duct was diagnostic for AIP in 88% patients. Biliary complications and early relapse are common after surgical resection and immunomodulatory drugs can maintain long-term remission. CONCLUSION Criteria based on histology, imaging, endoscopy, serology, extrapancreatic organ involvement, and response to steroid therapy improve the diagnostic yield for AIP. Application of diagnostic and therapeutic protocols by a multidisciplinary team will optimize outcomes with a decline in the rate of operative intervention for AIP, a steroid-responsive disease with propensity for relapse.
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18
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Pasquali T, Schoenfield L, Spalding SJ, Singh AD. Orbital inflammation in IgG4-related sclerosing disease. Orbit 2012; 30:258-60. [PMID: 21957960 DOI: 10.3109/01676830.2011.593677] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
IgG4-related sclerosing disease is a recently described systemic inflammatory disease that should be considered when evaluating patients with nonspecific orbital inflammation (pseudotumor). Orbital biopsy is necessary to establish a diagnosis and demonstrates lymphoplasmacytic infiltration, fibrosis, obliterative phlebitis of medium and small veins, and variable degrees of eosinophilia. We report the clinical and histopathological findings of 2 patients who developed chronic orbital inflammation as a manifestation of IgG4-related sclerosing disease. The 2 cases illustrate the widely varying clinical characteristics of this elusive disease.
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Kaji R, Takedatsu H, Okabe Y, Ishida Y, Sugiyama G, Yonemoto K, Mitsuyama K, Tsuruta O, Sata M. Serum immunoglobulin G4 associated with number and distribution of extrapancreatic lesions in type 1 autoimmune pancreatitis patients. J Gastroenterol Hepatol 2012; 27:268-72. [PMID: 21929654 DOI: 10.1111/j.1440-1746.2011.06933.x] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND AND AIM Type 1 autoimmune pancreatitis (AIP) is characterized by the increase of serum immunoglobulin (Ig)G4 and abundant IgG4 plasma cell infiltration in the pancreas and various extrapancreatic lesions (EPL), which are proposed as IgG4-related disease. We assessed the correlation between serum IgG4 and the number of EPL, and the association between serum IgG4 and the distribution of EPL in type 1 AIP patients. METHODS Serum IgG4 was measured in 35 type 1 AIP patients and 71 non-AIP patients. The clinical characteristics and distribution of eight EPL were determined in 35 type 1 AIP patients. RESULTS Serum IgG4 in type 1 AIP was significantly higher than in non-AIP (P < 0.001). A total of 33 patients had EPL among 35 patients with type 1 AIP (94.3%). There was a significant correlation between serum IgG4 and the number of EPL (ρ = 0.75, P < 0.001). Further, to assess the association between serum IgG4 and the distribution of EPL, type 1 AIP patients were divided into two groups: as abdominal localized EPL and systemic EPL. Both serum IgG4 and total numbers of EPL in systemic EPL were remarkably higher than those in abdominal localized EPL. Serum IgG4 cut-off value was 346 mg/dL to distinguish between abdominal localized EPL and systemic EPL according to the receiver-operator characteristic curve data. CONCLUSIONS Our findings indicated that serum IgG4 was useful in both the diagnosis of type 1 AIP and the detection of systemic EPL. Our finding may help the concept and diagnostic criteria of IgG4-related disease with type 1 AIP.
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Affiliation(s)
- Ryohei Kaji
- Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Fukuoka, Japan
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Psarras K, Baltatzis ME, Pavlidis ET, Lalountas MA, Pavlidis TE, Sakantamis AK. Autoimmune pancreatitis versus pancreatic cancer: a comprehensive review with emphasis on differential diagnosis. Hepatobiliary Pancreat Dis Int 2011; 10:465-473. [PMID: 21947719 DOI: 10.1016/s1499-3872(11)60080-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
BACKGROUND Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis with a discrete pathophysiology, occasional diagnostic radiological findings, and characteristic histological features. Its etiology and pathogenesis are still under investigation, especially during the last decade. Another aspect of interest is the attempt to establish specific criteria for the differential diagnosis between autoimmune pancreatitis and pancreatic cancer, entities that are frequently indistinguishable. DATA SOURCES An extensive search of the PubMed database was performed with emphasis on articles about the differential diagnosis between autoimmune pancreatitis and pancreatic cancer up to the present. RESULTS The most interesting outcome of recent research is the theory that autoimmune pancreatitis and its various extra-pancreatic manifestations represent a systemic fibro-inflammatory process called IgG4-related systemic disease. The diagnostic criteria proposed by the Japanese Pancreatic Society, the more expanded HISORt criteria, the new definitions of histological types, and the new guidelines of the International Association of Pancreatology help to establish the diagnosis of the disease types. CONCLUSION The valuable help of the proposed criteria for the differential diagnosis between autoimmune pancreatitis and pancreatic cancer may lead to avoidance of pointless surgical treatments and increased patient morbidity.
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Affiliation(s)
- Kyriakos Psarras
- Second Surgical Propedeutical Department, Medical School, Aristotle University of Thessaloniki, Hippocration Hospital, Konstantinoupoleos 49, 54642 Thessaloniki, Greece
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Sánchez-Castañón M, de Las Heras-Castaño G, Gómez C, López-Hoyos M. Differentiation of autoimmune pancreatitis from pancreas cancer: utility of anti-amylase and anti-carbonic anhydrase II autoantibodies. AUTOIMMUNITY HIGHLIGHTS 2011; 3:11-7. [PMID: 26000123 PMCID: PMC4389022 DOI: 10.1007/s13317-011-0024-x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 12/02/2010] [Accepted: 07/29/2011] [Indexed: 12/20/2022]
Abstract
Purpose To investigate the utility of different combinations of serum anti-carbonic anhydrase II antibodies (CA II Abs), anti-α amylase antibodies (AMY-α Abs) and IgG4 levels for the diagnosis of autoimmune pancreatitis (AIP). Methods We recruited 93 patients with clinical suspicion for AIP and 94 patients as control groups between June 2003 and October 2009. Serum antibodies were measured using homemade enzyme linked immunosorbent assay and IgG4 levels were determined by nephelometry. Results Both CA-II Abs and AMY-α Abs had the highest sensitivity (83%) although AMY-α Abs (89%) were more specific than CA-II Abs (75%). The presence of increased IgG4 levels was the most specific serological marker (94%), but it had the lowest sensitivity (58%). The combination of the three serological markers altogether had the highest specificity (99%) and positive predictive value (PPV) (86%), but they had a rather low sensitivity (50%). When we combined CA-II Abs and AMY-α Abs without IgG4 levels, we got the highest sensitivity (75%) and negative predictive value (98%) but the specificity and the PPV decreased to 93 and 50%, respectively. Importantly, AMY-α Abs were not detected in pancreas cancer. Conclusions The presence of serum CA-II and AMY-α Abs with increased IgG4 is useful in the differential diagnosis of AIP from pancreatic cancer.
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Affiliation(s)
- M Sánchez-Castañón
- Servicio Inmunología, Hospital Universitario Marqués de Valdecilla-IFIMAV, Avda. Valdecilla s/n, 39008 Santander, Spain
| | - G de Las Heras-Castaño
- Gastroenterology Service, Hospital Universitario Marqués de Valdecilla-IFIMAV, Santander, Spain
| | - C Gómez
- Servicio Inmunología, Hospital Universitario Marqués de Valdecilla-IFIMAV, Avda. Valdecilla s/n, 39008 Santander, Spain
| | - M López-Hoyos
- Servicio Inmunología, Hospital Universitario Marqués de Valdecilla-IFIMAV, Avda. Valdecilla s/n, 39008 Santander, Spain
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Takuma K, Kamisawa T, Tabata T, Inaba Y, Egawa N, Ozaki N, Horiguchi SI, Takuma S. Visual field deficit: a rare initial symptom of autoimmune pancreatitis. Intern Med 2011; 50:887-91. [PMID: 21498937 DOI: 10.2169/internalmedicine.50.5012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
An autoimmune pancreatitis (AIP) patient with metachronous and multiple extrapancreatic lesions is reported. Initial symptoms were proptosis, oculomotor deficits, and a visual field deficit of the left eye, and swelling of bilateral lacrimal glands. Swelling of the right salivary gland and elevated serum levels of hepatobiliary enzymes were detected. AIP associated with IgG4-related orbital pseudotumor, IgG4-related sclerosing dacryoadenitis and sialadenitis, and IgG4-related sclerosing cholangitis was diagnosed. All symptoms and lesions improved with steroid therapy. Although an orbital pseudotumor is a rare extrapancreatic lesion of AIP, we should know that AIP patients may describe unusual symptoms such as abnormal visual field.
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Affiliation(s)
- Kensuke Takuma
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Japan
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Autoimmune pancreatitis: the clinicopathological characteristics of the subtype with granulocytic epithelial lesions. J Gastroenterol 2010; 45:787-93. [PMID: 20549251 DOI: 10.1007/s00535-010-0265-x] [Citation(s) in RCA: 95] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2010] [Accepted: 05/17/2010] [Indexed: 02/04/2023]
Abstract
Autoimmune pancreatitis (AIP) has been established as a distinct form of chronic pancreatitis that is distinguishable from other types such as alcoholic, hereditary or obstructive chronic pancreatitis. AIP seems to be a global disease, since it has been reported in many different countries, especially from Japan, USA and Europe (Germany, Italy, United Kingdom). Typical histopathological findings in the pancreas in AIP include a periductal lymphoplasmacytic infiltration with fibrosis, causing narrowing of the involved ducts. The typical clinical features include presentation with obstructive jaundice/pancreatic mass and a dramatic response to steroids. However, while the reports from Japan describe uniform changes called lymphoplasmacytic sclerosing pancreatitis (LPSP) in the pancreas from AIP patients, the reports from Europe and USA distinguish two histopathologic patterns in AIP patients: one with the characteristics of LPSP and another with slightly different histological features, called idiopathic duct centric pancreatitis (IDCP) or AIP with granulocytic epithelial lesions (GELs). This article reviews the evidence that GEL-positive AIP or IDCP is a second type of AIP, distinct from LPSP, in regard to pancreatic pathology, immunology and epidemiology.
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Kamisawa T, Takuma K, Egawa N, Tsuruta K, Sasaki T. Autoimmune pancreatitis and IgG4-related sclerosing disease. Nat Rev Gastroenterol Hepatol 2010; 7:401-9. [PMID: 20548323 DOI: 10.1038/nrgastro.2010.81] [Citation(s) in RCA: 148] [Impact Index Per Article: 9.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Autoimmune pancreatitis (AIP) is a unique form of pancreatitis in which the pathogenesis is suspected to involve autoimmune mechanisms. AIP sometimes mimics pancreatic cancer in its presentation, but as AIP responds dramatically to steroid therapy, accurate diagnosis is necessary. AIP is currently diagnosed on the basis of a combination of characteristic clinical, serological, morphological and histopathological features. However, its diagnosis remains a clinical challenge and there are no internationally agreed diagnostic criteria. Another type of AIP called 'idiopathic duct-centric chronic pancreatitis' or 'AIP with granulocytic epithelial lesion' has been reported in Western countries. IgG4-related sclerosing disease is a systemic disease in which IgG4-positive plasma cells and T lymphocytes extensively infiltrate various organs. Organs with tissue fibrosis and obliterative phlebitis, such as the pancreas, salivary gland and retroperitoneum, show clinical manifestations; AIP seems to represent one manifestation of IgG4-related sclerosing disease. As a mass is formed in most cases of IgG4-related sclerosing disease, a malignant tumor is frequently suspected on initial presentation. Clinicians should consider IgG4-related sclerosing disease in the differential diagnosis to avoid unnecessary surgery.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan.
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Abstract
BACKGROUND Autoimmune pancreatitis (AIP) is a relatively newly recognized type of pancreatitis that is characterized by diffuse or focal swelling of the pancreas due to lymphoplasmacytic infiltration and fibrosis of the pancreatic parenchyma. MATERIAL AND METHODS A PubMed literature search was performed using the keywords "autoimmune pancreatitis". Sometimes, bibliographies were cross-referenced and related article searches were performed once an article of interest was identified. RESULTS Pathologically, AIP shows narrowing of the pancreatic ducts and the intrapancreatic portion of the common bile duct. Obstructive jaundice is a common symptom at presentation, and pancreatic cancer represents an important clinical differential diagnosis. In late stages of the disease, the normal pancreatic parenchyma is often replaced by large amounts of fibrosis. Histologically, there seem to be two subtypes of the disease-one showing infiltration with IgG4-positive plasma cells but lacking granulocytic epithelial lesions (GELs), the other showing GELs but lacking strong IgG4 positivity. AIP is in at least some instances the pancreatic manifestation of a clinicopathological entity of IgG4-related systemic sclerosing disease. On the basis of pancreatic imaging, together with serological measurement of IgG4 and evaluation of other organ involvement, many AIP patients can be identified. The remaining patients require further diagnostic work-up. In these patients, pancreatic core needle biopsy and, as AIP responds to steroid treatment, also a trial with steroids, can help to differentiate AIP from pancreatic cancer. OUTLOOK AND DISCUSSION: This review presents the pathological, radiologic and laboratory findings of AIP. Moreover, the treatment and pathogenesis are discussed.
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Affiliation(s)
- Sönke Detlefsen
- Department of Pathology, Odense University Hospital, Odense, Denmark.
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Matsushita M, Fukui T, Uchida K, Nishio A, Okazaki K. Atypical retroperitoneal fibrosis associated with biliary stricture: IgG4-related sclerosing disease? Scand J Gastroenterol 2010; 44:1146-7. [PMID: 19585375 DOI: 10.1080/00365520903100499] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
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Lee FJ, Varikatt W, Kairaitis K, D'Souza F, Lin MW. IgG4-related dacryoadenitis. Ophthalmology 2010; 117:398.e3-4. [PMID: 20141856 DOI: 10.1016/j.ophtha.2009.09.011] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2009] [Accepted: 09/10/2009] [Indexed: 01/13/2023] Open
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Matsuki Y, Sato K, Fujikawa A, Kyoto Y, Hashimoto H, Hakozaki Y. A case of incidentally detected IgG4-related sclerosing disease involving inflammatory abdominal aortic aneurysm and autoimmune pancreatitis. Mod Rheumatol 2010; 20:306-10. [PMID: 20157837 DOI: 10.1007/s10165-010-0272-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2009] [Accepted: 01/12/2010] [Indexed: 11/25/2022]
Abstract
A 59-year-old asymptomatic man was incidentally found to have a periaortic mass and an elevated serum amylase level during his medical check-up. Additional findings, such as infiltration of immunoglobulin G4 (IgG4)-producing plasma cells in the mass lesion, elevation of serum IgG4 (1000 mg/dl), and pancreatic duct narrowing as evidenced on a magnetic resonance cholangiopancreatography scan, confirmed the diagnosis as retroperitoneal fibrosis complicated with autoimmune pancreatitis. The patient responded favorably to steroid treatment before the appearance of symptoms.
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Affiliation(s)
- Yasunori Matsuki
- Department of Medicine, Japan Self-Defense Force Central Hospital, 1-2-24 Ikejiri, Setagaya, Tokyo 154-8532, Japan.
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Sánchez-Castañón M, de las Heras-Castaño G, López-Hoyos M. Autoimmune pancreatitis: An underdiagnosed autoimmune disease with clinical, imaging and serological features. Autoimmun Rev 2010; 9:237-40. [DOI: 10.1016/j.autrev.2009.07.003] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2009] [Accepted: 07/09/2009] [Indexed: 01/06/2023]
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Kamisawa T, Anjiki H, Takuma K, Egawa N, Itoi T, Itokawa F. Endoscopic approach for diagnosing autoimmune pancreatitis. World J Gastrointest Endosc 2010; 2:20-4. [PMID: 21160674 PMCID: PMC2999079 DOI: 10.4253/wjge.v2.i1.20] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2009] [Revised: 08/26/2009] [Accepted: 09/02/2009] [Indexed: 02/05/2023] Open
Abstract
It is of utmost importance to differentiate autoimmune pancreatitis (AIP) from pancreatic cancer (PC). Segmental AIP cases are sometimes difficult to differentiate from PC. On endoscopic retrograde cholangiopancreatography, long or skipped irregular narrowing of the main pancreatic duct (MPD), less upstream dilatation of the distal MPD, side branches derived from the narrowed portion of the MPD, absence of obstruction of the MPD, and stenosis of the intrahepatic bile duct suggest AIP rather than PC. Abundant infiltration of IgG4-positive plasma cells is frequently and rather specifically detected in the major duodenal papilla of AIP patients. IgG4-immunostaining of biopsy specimens obtained from the major duodenal papilla is useful for supporting a diagnosis of AIP with pancreatic head involvement. On endoscopic ultrasonography (EUS), hyperechoic spots in the hypoechoic mass and the duct-penetrating sign suggest AIP rather than PC. EUS and intraductal ultrasonography sometimes show wall thickening of the common bile duct even in the segment in which abnormalities are not clearly observed with cholangiography in AIP patients. EUS-guided fine needle aspiration, especially EUS-guided Tru-Cut biopsy, is useful to diagnose AIP, as well as to exclude PC.
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Affiliation(s)
- Terumi Kamisawa
- Terumi Kamisawa, Hajime Anjiki, Kensuku Takuma, Naoto Egawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo 113-8677, Japan
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Abstract
OBJECTIVES To clarify the frequency and clinical significance of extrapancreatic lesions in autoimmune pancreatitis (AIP). METHODS The frequency and clinical characteristics of extrapancreatic lesions during the clinical course of AIP were investigated retrospectively in 64 patients with AIP. The predictive factors for relapse of AIP at clinical onset were also examined. RESULTS Extrapancreatic lesions occurred in 95% (61/64) during the clinical course of AIP. The frequencies of sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis, and mediastinal or hilar lymphadenopathy were 84% (54/64), 23% (15/64), 16% (10/64), and 77% (27/35), respectively. Patients with sclerosing sialadenitis or extrapancreatic bile duct sclerosing cholangitis had a significantly higher serum immunoglobulin G concentration than those without (P = 0.005 and P = 0.016, respectively). Univariate analysis revealed that sclerosing sialadenitis (P = 0.005), diffuse pancreatic ductal changes (P = 0.028), and a high serum immunoglobulin G concentration (P = 0.030) at clinical onset of AIP were significant predictive factors for relapse. Multivariate analysis revealed that diffuse pancreatic ductal changes (P = 0.005) and sclerosing sialadenitis (P = 0.012) were significant independent predictive factors for relapse of AIP. CONCLUSIONS The frequency of extrapancreatic lesions with AIP during the clinical course was high. The presence of sclerosing sialadenitis at clinical onset is a significant predictive factor for relapse of AIP.
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Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4-related systemic disease and lymphoplasmacytic aortitis. ACTA ACUST UNITED AC 2009; 60:3139-45. [DOI: 10.1002/art.24798] [Citation(s) in RCA: 131] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
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Kamisawa T, Takuma K, Kuruma S, Fujiwara J, Anjiki H, Koizumi K, Egawa N, Kubota N, Ozaki N, Sasaki T. Lacrimal gland function in autoimmune pancreatitis. Intern Med 2009; 48:939-43. [PMID: 19525578 DOI: 10.2169/internalmedicine.48.2107] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
OBJECTIVE Autoimmune pancreatitis (AIP) may be a pancreatic lesion of IgG4-related systemic disease. Lacrimal gland swelling is a rare extrapancreatic lesion of AIP. The aim of the present study was to investigate lacrimal gland function in AIP patients, and to determine changes after steroid therapy. PATIENTS AND METHODS Schirmer's test and sialochemistry were done prospectively in 11 AIP patients. These tests were also performed after steroid therapy in 7 patients. RESULTS Dysfunction of tear secretion was found in at least one eye in 7 (64%) patients. The average lower level in both eyes was 4.3+/-1.5 mm in the 7 patients with lacrimal gland dysfunction, which was significantly lower than the 8.2+/-2.4 mm in patients with normal lacrimal gland function (p=0.005). There were no significant differences between the two groups in age at diagnosis of AIP, sex ratio, and the presence of swelling of the lacrimal glands and the salivary glands. Although there was no significant difference, mean serum IgG4 levels and mean salivary Na+ and beta2 microglobulin levels were lower in patients with normal lacrimal gland function. After steroid therapy, lacrimal gland function improved in 3 of 5 patients with impaired lacrimal gland function, though the degree of improvement was not marked compared to the improvement of salivary gland function. CONCLUSION Lacrimal gland function was frequently impaired in AIP patients, even when no lacrimal gland swelling was observed clinically. Lacrimal gland function impairment appears to be similar to impairment of salivary gland function in AIP patients.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo.
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