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Pancreatology

journal homepage: www.elsevier .com/locate/pan

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Review article 65666768697071727374757677

Clinical validation of the international consensus diagnostic criteriaand algorithms for autoimmune pancreatitis: Combined IAP and KPBAmeeting 2013 report

Tae Jun Song a, Myung-Hwan Kim a,*, Min Jae Kim b, Sung Hoon Moon c, Ji Min Han d,An International Panel of Speakers and Moderators1

aDepartment of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South KoreabDepartment of Physician Education and Training, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South KoreacDepartment of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, South KoreadDepartment of Internal Medicine, Daegu Catholic University Medical Center, Daegu, South Korea

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Keywords:Autoimmune pancreatitisDiagnosisCriteriaAlgorithms

* Corresponding author. Department of Internal MCollege of Medicine, Asan Medical Center, AsanbyeSeoul 138-736, South Korea. Tel.: þ82 2 3010 3180; f

E-mail address: mhkim@amc.seoul.kr (M.-H. Kim)1 See Appendix.

http://dx.doi.org/10.1016/j.pan.2014.04.0331424-3903/Copyright � 2014, IAP and EPC. Published

86878889909192939495

Please cite this article in press as: Song TJautoimmune pancreatitis: Combined IAP an

a b s t r a c t

There have been great developments in the diagnosis and treatment of autoimmune pancreatitis (AIP) inthe last decade. Most significantly, the international consensus diagnostic criteria (ICDC) proposed in2011 were the first attempt to provide unified diagnostic criteria incorporating most features of thepreviously existing national criteria. However, the ICDC have not yet been prospectively validated usingevidence-based studies since their introduction. An international symposium on the diagnosis of AIP washeld in Seoul, South Korea on September 6, 2013, in cooperation with the International Association ofPancreatology and the Korean Pancreatobiliary Association meeting. In contrast to other symposia in thepast, which had primarily focused on the diagnostic criteria themselves, expert panels in this symposiumdiscussed how the diagnostic criteria and algorithms had been embraced in clinical settings to diagnoseAIP in each country and conducted a comprehensive evaluation of these criteria and algorithms. It wasacknowledged that there was a room for improvement in the ICDC and their algorithms and that furthermodifications might be required in the future. Prospective clinical validation in larger series is needed forconfirmation.Copyright � 2014, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. Allrights reserved.

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1. Introduction

During the last decade, there has been growing recognition of apeculiar type of chronic pancreatitis known as autoimmunepancreatitis (AIP). Although the concept, characterization andtreatment of AIP have evolved significantly, the diagnosis of AIPremains challenging in clinical practice. Since 2002, when the JapanPancreas Society (JPS)first proposeddiagnostic criteria for AIP basedon pancreatic imaging, serology and histology [1], several sets ofdiagnostic criteria for AIP have been advocated around the world,including the revised JPS criteria (2006 and 2011) [2,3], the originaland revised HISORt criteria (2006 and 2009) [4,5], the Korean

edicine, University of Ulsanongwon-Gil 86, Songpa-Gu,ax: þ82 2 476 0824..

by Elsevier India, a division of Re

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, et al., Clinical validation ofd KPBA meeting 2013 report

criteria (2007) [6], the Asian diagnostic criteria (2008) [7], the Ver-ona criteria (2009) [8] and the Mannheim criteria (2009) [9]. Thediversity of diagnostic criteria for AIP in various countries mayreflect differences inpractice patterns in the useof various tests (e.g.,diagnostic endoscopic retrograde pancreatography [ERP]), localexpertise (e.g., endoscopic ultrasound [EUS]-guided pancreatic corebiopsy [PCB]), and clinical epidemiology (e.g., type 1 vs. type 2 AIP).

The international consensus diagnostic criteria (ICDC) formu-lated under the influential leadership of Drs. Chari and Shimose-gawa in 2011 have marked a significant step forward in thediagnosis of AIP (Table 1). The ICDC unified multiple diagnosticcriteria from different countries. Eastern and Western experts havereached a consensus on diagnostic criteria for AIP in response to theneed to diagnose AIP regardless of the practice patterns in the useof various tests and to incorporate the two different subtypes (type1 and type 2) of AIP [10].

In the ICDC, the entity of AIP consists of two distinct clinical andhistopathological forms of pancreatitis; type 1 and type 2. In

ed Elsevier India Pvt. Ltd. All rights reserved.

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contrast to type 2 AIP that appears to exclusively affect thepancreas, type 1 AIP is now viewed as the pancreatic manifestationof a systemic fibroinflammatory disease referred to as IgG4-relateddisease. To diagnose AIP, the ICDC use varying combinations of thefive cardinal features of AIP; pancreatic imaging (parenchymal andductal), serology (immunoglobulin G4; IgG4), other organinvolvement (OOI), histopathology and immunostaining, and ste-roid responsiveness. The ICDC feature a grading for each category aslevel 1 or level 2, depending on the strength of the associationwithAIP. The criteria and algorithms for types 1 and type 2 AIP weredeveloped separately. How these recent advances have beenembraced in general practice, however, is not clearly known,especially with regard to the algorithmic approach. It is time tovalidate the ICDC and their algorithms using more objectiveevidence.

2. Seoul symposium on the diagnosis of AIP

The Joint Meeting of the International Association of Pan-creatology and the Korean Pancreatobiliary Associationwas held inSeoul, South Korea, between September 4 and 7, 2013. An inter-national symposium on the diagnosis of AIP was held on September6 during which experts from Japan, Korea, the U.S., Germany andItaly (see Appendix) engaged in vigorous debate on the diagnosticcriteria and algorithms for AIP and discussed future directions toimprove them. In contrast to other international symposia in thepast, which mainly dealt with diagnostic criteria themselves, thissymposium aimed to assess how the international consensusdiagnostic algorithms have been integrated in each country to di-agnose AIP in clinical practice. This meeting provided a good op-portunity to evaluate the current use of the ICDC and theiralgorithms in clinical settings and to identify their potential limi-tations that may require further clarifications and modifications inthe future.

Herein, we report a brief summary of the discussions regardingthe current use of the ICDC and their algorithms as a diagnostic toolin clinical settings.

3. Controversies in the ICDC

3.1. Issues related to other organ involvement

3.1.1. Grading of OOIUnder the ICDC, for type 1 AIP, proximal bile duct stricture and

retroperitoneal fibrosis are classified as level 1 (highly suggestiveof) OOI, while enlarged salivary glands and renal involvement areclassified as level 2 (only supportive) OOI. Level 1 OOI constitutesfindings that strongly suggest AIP, such as imaging findings that arevery rarely observed in pancreatic cancer and do not require his-tological verification. However, it is not clear why renal involve-ment is classified as level 2 OOI [11e13]. It could be argued that

Table 1The international consensus diagnostic criteria for autoimmune pancreatitis.

Diagnosis Imaging evidence Collateral eviden

Definitive type 1 AIP Typical/indeterminate Histologically coTypical Any non-D levelIndeterminate Two or more froIndeterminate Level 1 S/OOI þ

Probable type 1 AIP Indeterminate Level 2 S/OOI/HDefinitive type 2 AIP Typical/indeterminate Histologically coProbable type 2 AIP Typical/indeterminate Level 2 H/clinicaAIP-not otherwise specified Typical/indeterminate D1/2 þ Rt

LPSP, lymphoplasmacytic sclerosing pancreatitis; H, histology of the pancreas; D, ductal iidiopathic duct-centric pancreatitis.

Please cite this article in press as: Song TJ, et al., Clinical validation ofautoimmune pancreatitis: Combined IAP and KPBA meeting 2013 report

both retroperitoneal fibrosis and renal involvement provide cluesfor diagnosing AIP and permit reliable distinction between AIP andpancreatic cancer. As pancreatic cancer rarely metastasize to thekidney or retroperitoneum, the findings of renal involvement orretroperitoneal fibrosis would favor the diagnosis of AIP whendifferentiating between pancreatic cancer and AIP. Moreover, bothfindings can be recognized on abdominal CT which is part of theroutine workup for AIP, and the retroperitoneum is not easilyaccessible for nonsurgical biopsy. On the other hand, the imagingfeatures of lymph nodes involvement are non-specific and requirehistological confirmation to exclude pancreatic cancer-relatedlymphadenopathy. It was therefore suggested to classify lymphnode involvement as lower level of evidence compared to proximalbile duct stricture, retroperitoneal fibrosis and renal involvement interms of strength of association with AIP. Two organs/sites (prox-imal bile duct and retroperitoneal fibrosis) do not seem to havemore evidence of strength to be superior to kidney involvement inthe diagnosis and differential diagnosis of AIP. Renal involvementcould be of the same level as retroperitoneal fibrosis.

Grading (level 1 vs. level 2) of each criterion (pancreatic ductalimaging, serum IgG4, OOI and histology) may not be establishedbased on high-level of evidence.

3.1.2. Bile duct stricture as OOI (IgG4-related sclerosing cholangitis)It remains controversial among experts whether isolated distal

bile duct stricture represents a part of pancreatic lesion or OOI(IgG4-related sclerosing cholangitis [IgG4-SC]). In the ICDC, prox-imal (hilar/intrahepatic) bile duct stricture are classified as level 1OOI whereas an isolated distal bile duct stricture confined to theintrapancreatic portion is not included in OOI as the distal bile ductstricture may be caused by extrinsic compression by pancreaticinflammation. Distal bile duct stricture is therefore regarded as apart of AIP and is not included in IgG4-SC. Recently, experts in Japanhave established separate criteria for the diagnosis of IgG4-SC [14].In contrast to the ICDC, the Japanese consensus criteria includedisolated distal common bile duct stricture as IgG4-SC, becauseresection specimens of these patients with isolated intrapancreaticcommon bile duct stricture often show that ductal wall thickeningspreads continuously from intrapancreatic common bile duct to thesuprapancreatic middle bile duct [14]. Japanese investigators sug-gested that both IgG4-related biliary inflammation and pancreatichead swelling affect lower bile duct stricture, which may beincluded in IgG4-SC [15]. Therefore, consensus on an accuratedefinition of bile duct involvement as OOI would be necessary.

3.2. Diagnostic groups according to the ICDC

In the ICDC, AIP diagnosis was stratified by the strength of thesupporting evidence for AIP into definite and probable. Thus thediagnosis of type 1 and type 2 AIP can be definite or probable, andAIP cases clinically indistinguishable between type 1 and type 2 AIP

ce

nfirmed LPSP (level 1 H)1/level 2m level 1 (þlevel 2 D)Rt or level 1 D þ level 2 S/OOI/H þ Rtþ Rtnfirmed IDCP (level 1 H) or clinical inflammatory bowel disease þ level 2 H þ Rtl inflammatory bowel disease þ Rt

maging; S, serology; OOI, other organ involvement; Rt, steroid responsiveness; IDCP,

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are classified as AIP-not otherwise specified (NOS). For example,histologically unconfirmed but clinically suspected type 2 AIP iscategorized as probable type 2 AIP. However, in the absence ofsignificantly elevated IgG4 levels the distinction between probabletype 1 AIP, probable type 2 AIP and AIP-NOS is often ambiguous.Probable type 2 AIP may possibly be type 1 AIP and vice versabecause there is a potential overlap in clinical, radiological andhistopathological features between type 1 and type 2 AIP. Someexperts therefore proposed that the diagnostic groups of probabletype 1, probable type 2 and AIP-NOS could be combined into asingle entity called probable AIP, with no subtype specification.

Although the adoption of probable diagnosis and AIP-NOS mayenhance the diagnostic sensitivity, doing so may compromisediagnostic specificity. According to a recent study, out of five majordiagnostic criteria (ICDC, HISORt, revised JPS, Asian and Korean),ICDC was found to be the most sensitive for the diagnosis of AIP[16]. One plausible explanation for this excellent sensitivity of theICDC is that the ICDC incorporates most of the features of therevised HISORt criteria and combines the salient features of Japa-nese/Korean criteria. The ICDC for AIP may provide a high diag-nostic specificity to distinguish malignancy since steroidresponsiveness is also used to confirm the diagnosis. Instead, thechallenge on maintaining high specificity of diagnostic criteria maylie in differentiating AIP from the early stage of other types ofchronic pancreatitis, or some forms of mild acute pancreatitis.There remains a possibility that non-specific acute or chronicpancreatitis can be mistakenly classified as probable type 2 AIP,especially when making a type 2 AIP diagnosis solely based on corebiopsy findings.

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3.3. Definition of granulocytic epithelial lesion (GEL)

According to the ICDC, type 1 AIP can definitely be diagnosed onthe basis of PCB that fulfills three or more of the following condi-tions; periductal lymphoplasmacytic infiltrate without granulo-cytic infiltration, obliterative phlebitis, storiform fibrosis, andabundant IgG4-positive cells. On the other hand, definite diagnosisof type 2 AIP can be made solely based on the presence of granu-locytic epithelial lesion (GEL). The finding of the so called GEL,which is characterized by the infiltration of neutrophils into thelumen or epithelium of the pancreatic duct, is known to be specificto type 2 AIP. If GELs are identified in a PCB specimen, it is patho-gnomonic for type 2 AIP [17]. Interestingly, it now doesn’t appearthat GELs arise exclusively in type 2 AIP. Recent studies reportedthat GELs could be found in 27% of type 1 AIP cases although itconsisted only of intraepithelial neutrophils [18], and there is areport that typical lymphoplasmacytic sclerosing pancreatitis(LPSP) coexisted with GELs [19]. GELs seen in type 1 AIP may resultfrom secondary acute inflammation developing on the backgroundof LPSP, probably due to a ductal stricture. A previous study tookinto account of GELs present in small intralobular ducts as well asthose seen in medium- and large-sized interlobular ducts [20].However, some experts found that GELs in the intralobular ductsare not uncommon in type 1 AIP [21]. Although the presence ofGELs in the interlobular ducts is a highly specific finding for type 2AIP, it may be difficult to identify interlobular GELs in small biopsyspecimens acquired by PCB.

Given the conflicting data from recent studies, there would be aneed for a consensus definition of GELs based on more stringentcriteria. Most experts agreed that the location of the ductal infil-tration of neutrophils (e.g., interlobular large/medium-sized ductvs. intralobular small-sized duct, and intraepithelial vs. intra-luminal), the density of ductal neutrophilic infiltrate and theseverity of the intraductal epithelial destruction (e.g., abscess) need

Please cite this article in press as: Song TJ, et al., Clinical validation ofautoimmune pancreatitis: Combined IAP and KPBA meeting 2013 report

to be more clearly specified in defining GELs for the correct diag-nosis of type 2 AIP.

3.4. Role of ulcerative colitis in the diagnosis of AIP

According to the ICDC, patients can be diagnosed with definitetype 2 AIP even though GELs are not observed on histology, whenthey have level 2 histology (both granulocytic and lympho-plasmacytic acinar infiltrate and absent/scant IgG4-positive cells)on PCB, ulcerative colitis (UC) and steroid responsiveness. However,this combination can also be present in some patients with type 1AIP. UC may incidentally accompany AIP without a significant as-sociation with AIP in the West because UC is a fairly common dis-order; the prevalence of UC in adults in the U.S. has been reportedto 238 per 100,000 population [22]. Although UC is more commonin type 2 than type 1 AIP, patients with type 1 AIP have a higherprevalence of UC compared with the general population [23,24].Another criterion for the diagnosis of type 2 AIP, absent or scantIgG4-positive plasma cells in the inflamed pancreatic tissue, is aless specific finding. IgG4-positive cells may not be detected on corebiopsy specimen due to patchy involvement of the inflammatoryprocess seen in type 1 AIP [21]. Some cases of type 1 AIP mayexhibit mild acinar neutrophilic infiltrate, presumably owing to thenarrowing of interlobular and/or intralobular ducts [25]. This raisesthe possibility that type 1 AIPmay bemistakenly classified as type 2AIP due to accompanying UC, particularly whenmaking a diagnosisusing core biopsy specimens. While this does certainly not affectinitial treatment selection, it may have an impact on predictingrecurrence or on decision for a maintenance therapy.

4. Diagnostic algorithms

In the ICDC classification, different diagnostic algorithms areused for the AIP subtypes (type 1 vs. type 2). The presence of OOIand elevation of serum IgG4 level are the main determining factorsin guiding which algorithms to follow (type 1 vs. type 2). However,an absence of serological abnormalities and lack of OOI in patientswith AIP does not necessarily imply diagnosis of type 2 AIP, as type1 AIP can also be seronegative and without OOI. There may be aninsufficient rationale for applying separate algorithms particularlyin the beginning stage of diagnostic workup when it is often un-clear whether the patient qualifies as type 1 or type 2 AIP. A moresimplified and unified algorithms could address this issue.

International consensus diagnostic algorithms heavily rely onPCB; the indication for ERP is reserved for patients who have failedcore biopsy diagnosis. The purpose of the ICDC for AIP was todevelop diagnostic criteria that can be applied worldwide, takinginto consideration the marked differences in clinical practice pat-terns. However, the community practice setting reflects manage-ment issues faced by a majority of practicing gastroenterologists,especially in situations where expertise such as EUS-guided PCBmay not be available and therefore rarely performed as part of thediagnostic workup for AIP. In institutions where PCB is not avail-able, therefore, alternative ERCP-based algorithmic approachincluding direct pancreatogram, tissue sampling from the bile ductand ampullary biopsy for IgG4 immunostaining may be consideredin presumed type 1 AIP patients. In Japan and Korea, ERCP-baseddiagnostic approach is still being frequently employed. This maybe due to limited availability of PCB in contrast to rich experiencewith direct pancreatogram and relative rarity of type 2 AIP in Japanand Korea. For these reasons, despite the original purpose of uni-fying multiple diagnostic criteria, the reality is that modified ICDCare currently being used to diagnose AIP in many countriesdepending on local expertise. In this symposium, modified

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diagnostic algorithms of Italy, Japan and Korea on the basis of eachcountry’s conditions were presented.

5. Future perspectives

The ICDC offers one of the best and most comprehensive levelsof evidence agreed upon to date. AIP can be diagnosed using ICDCwith a high degree of sensitivity; however, nonsurgically diag-nosing and subtyping AIP remains challenging and the gold stan-dard for the diagnosis of AIP is still largely dependent onhistopathological and immunohistochemical findings on surgicalspecimens. All experts agreed that we need more practical, sensi-tive, and specific biomarkers or phenotypic markers that distin-guish type 1 and type 2 AIP. In addition, it would seem reasonableto set up prospective international registry and tomaintain ongoingcumulative data collection for all patients with AIP [26].

In addition, new EUS-guided PCB needles with enhanced flexi-bility and improved tissue acquisition have been developed [27].These newly developed core biopsy needles can access all areas ofthe pancreas including pancreatic head portion. This approach mayachieve the two goals of histological examination in one sitting: theexclusion of pancreatic cancer and the histological diagnosis of AIP.Recently, a new technique that enhances tissue acquisition yieldusing 22-gauge needle has been introduced [28]. In the future, EUS-guided PCB may play a larger and more contributory role in thediagnosis of AIP [29].

6. Conclusions

The ICDC represents a giant leap towards unification of themultiple diagnostic criteria, and they are a crucial achievement inthe history of AIP. However, for the ICDC to be fully embraced inclinical practice, the issues raised here need to be resolved andfuture modifications may be required, driven by a systematic re-view of existing data. With the momentum provided by this sym-posium, researchers should join forces via prospective internationalmulticenter studies to address the issues that have not beenresolved in the diagnosis of AIP. It appears that there is room forimprovement in the ICDC; yet, prospective clinical validation inlarger series is needed for confirmation. Diagnostic criteria andalgorithms for AIP are still evolving.

Conflicts of interest

The authors declare no conflict of interest.

Appendix A. An International Panel of Speakers andModerators.

Suresh T. Chari, Department of Internal Medicine, Mayo Clinic,Rochester, Minnesota, USA.

Luca Frulloni, Department of Medicine, University of Verona,Verona, Italy.

Terumi Kamisawa, Department of Internal Medicine, TokyoMetropolitan Komagome Hospital, Tokyo, Japan.

Myung-Hwan Kim, Department of Internal Medicine, Universityof Ulsan College of Medicine, Asan Medical Center, Seoul, SouthKorea.

Jong Kyun Lee, Department of Internal Medicine, Sungkyunk-wan University, Seoul, South Korea.

Markus M. Lerch, Department of Medicine A, University Medi-cine Greifswald, Greifswald, Germany.

Kenji Notohara, Department of Pathology, Kurashiki CentralHospital, Japan.

Please cite this article in press as: Song TJ, et al., Clinical validation ofautoimmune pancreatitis: Combined IAP and KPBA meeting 2013 report

Kazuichi Okazaki, Department of Gastroenterology and Hep-atology, Kansai Medical University, Osaka, Japan.

Ji Kon Ryu, Department of Internal Medicine, Seoul NationalUniversity, Seoul, South Korea.

Tooru Shimosegawa, Department of Gastroenterology, TohokuUniversity, Sendai, Japan.

* The names are listed in alphabetical order.

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