Autoimmune pancreatitis

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Guidelines for diagnosis & management of autoimmune pancreatitis

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  • Definitive LPSP can be diagnosed with surrogate criteria not including histology. definite IDCP requires histological confirmation.
  • Recent study compared EUS-FNA and EUS-TCB performed in 14 patients for the diagnosis of AIP. EUS-TCB showed higher sensitivity (100%) and specificity (100%) compared to EUS-FNA (36% and 33%, respectively). respectively).Both procedures were found to be safe, with no complications.However, the diagnostic accuracy of EUS-FNA for pancreatic cancer has been reported to range between 60% and 90%, and the shortcomings of EUS-TCB due to technical difficulties of the sampling of lesions in the pancreatic head should also be considered.Hence, when AIP is suspected, a sequential sampling strategy has been proposed based on using EUS-FNAfirst, which is followed by EUS-TCB when cytologic examination is inconclusive.
  • All five patients with AIP presented with a characteristic stiff elastographic pattern not only of the mass lesion but also of the surrounding pancreatic parenchyma, which was not found in 17 patients with ductaladenocarcinoma and 10 healthy subjects.EUS elastography of the pancreas shows a typical and unique finding with homogenous stiffness of the whole organ, and this distinguishes AIP from the circumscribed mass lesion in ductaladenocarcinoma.
  • fluorine-18 fluorodeoxy glucose
  • high-low-high echo
  • Usage of multiple diagnostic criteria and their continued proliferation is not in the best interest of this field.
  • Effort of Eastern & Western experts to find common bases for diagnosis of AIP worldwide.
  • Autoimmune pancreatitis

    1. 1. Autoimmune pancreatitis International consensus diagnostic criteria Samir Haffar M.D. Assistant Professor of GastroenterologyAl-Mouassat University Hospital – Damascus – Syria
    2. 2. History of autoimmune pancreatitis Sarle 1961 Idiopathic chronic pancreatitis with elevated γG Yoshida 1995 Propose concept of autoimmune pancreatitisHamano 1995 Increased serum levels of IgG4 in AIP JPS 2002 Japan Pancreas Society: 1st guidelines of AIPKamisawa 2003 Novel entity: IgG4-related sclerosing disease Chari 2010 Two distinct subtypes: type 1 & type 2 Honolulu consensus Sarles H et al. Am J Dig Dis 1961 ; 6 : 688 – 698. Yoshida K et al. Dig Dis Sci 1995 ; 40 : 1561 – 1568. Hamano H et al. New Engl JMed 1995 ; 344 : 732 – 738. Japan Pancreas Society. J Jpn Pancreas 2002 ; 17 : 585 – 7. Kamisawa T et al. J Gastroenterol 2003 ; 203 ; 38 : 982 – 984. Chari ST et al. Pancreas. 2010 ; 39 : 549 – 554.
    3. 3. Increased number of published papers on autoimmune pancreatitis Searching in Pubmed up to 2009Search terms: autoimmune pancreatitis – Limit: field title Frulloni L et al. World J Gastroenterol 2011 ; 17 : 2076 – 2079.
    4. 4. Definition of AIP Distinct form of pancreatitis characterized by• Clinic Frequently present with obstructive jaundice With or without a pancreatic mass• Histology Lympho-plasmacytic infiltrate & fibrosis• Treatment Dramatic response to steroids Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    5. 5. Pancreatic presentation of AIP• Acute Pancreatic mass/obstructive jaundice Acute pancreatitis• Chronic Asymptomatic pancreatic massBurnt out stage Painless chronic pancreatitis Steatorrhea with atrophic pancreas Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    6. 6. How is autoimmune pancreatitis found?• Gastroenterologist Differential diagnosis of pancreatic or biliary cancers Differential diagnosis of PSC• Otolaryngologist, ophthalmologist, or rheumatologist Sjögren syndrome• Urologist Examination for retroperitoneal fibrosis Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    7. 7. When not to suspect AIP?• Features of cancer Narcotic requiring pain Marked anorexia/cachexia Dialated PD/ pancreatic atrophy• Recurrent pancreatitis without biliary involvement• Dyspepsia with mild increased of pancreatic enzymes
    8. 8. Diagnosis of AIP Combination of 1 or more of 5 cardinal features HISORt Histology LPSP – IDCP TCB/resection Imaging Parenchyma US – EUS – CT – PET – MRI Pancreatic Duct ERCP – MRCP Serology IgG4Other Organ Involvement IgG4-related diseases – IBD Response to therapy Steroid trial LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    9. 9. Diagnosis of AIP Combination of 1 or more of 5 cardinal features HISORt Histology LPSP – IDCP TCB/resection Imaging Parenchyma US – EUS – CT – PET – MRI Pancreatic Duct ERCP – MRCP Serology IgG4Other Organ Involvement IgG4-related diseases – IBD Response to therapy Steroid trial LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    10. 10. Comparison of type 1 & type 2 AIP Type 1 Type 2 AIP without GELs AIP with GELsAge Elderly YoungGender Predominantly male EqualDistribution Whole word Western countriesSerum IgG4 Elevated NormalHistopathology LPSP IDCPInfiltrating cells IgG4 + plasma cells GranulocytesRelapse rate High LowExtra-pancreatic lesions IgG4-related disease IBD (30%) GEL: Granulocyte Epithelial Lesions LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Chari ST et al. Pancreas 2010 ; 39 : 549 – 554.
    11. 11. IgG4-related sclerosing diseaseKamisawa T et al. Expert Opin Pharmacother 2011 ; 12 : 2149 – 2159.
    12. 12. Lympho-plasmacytic sclerosing pancreatitis (LPSP) AIP without GEL* Systemic disease: IgG4-related disease• Periductal lympho-plasmacytic infiltrate• Peculiar storiform fibrosis• Obliterative Venulitis: by lymphocytes & plasma cells• Abundant IgG4 positive plasma cells: > 10 cells/hpf Definite diagnosis can be made without histology * GEL: Granulocyte Epithelial Lesions Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    13. 13. Histopathological findings of AIP / LPSP H&E staining IgG4 immuno-stainingInfiltration of plasma cells & lymphocytes Abundant infiltration of ‘storiform fibrosis’’ IgG4-positive plasma cells Shimosegawa T & Kanno A. J Gastroenterol. 2009 ; 44 : 503 – 17.
    14. 14. Obliterative venulitis H&E stain Movat pentachrome stain Lymphoplasmacytic infiltration Artery easily found Fibrosis destroying vein wallPoorly visualizes obliterative venulitis resulting in narrowing & occlusion Law R et al. Clev Clin J Med 2009 ; 76 : 607 – 615.
    15. 15. Idiopathic Duct-Centric Pancreatitis (IDCP) AIP with GEL* Pancreas-specific disorder• Periductal lympho-plasmacytic infiltrate• Peculiar storiform fibrosis• None or very few IgG4-positive plasma cells: < 10 cells/hpf• GEL Intra-luminal & intra-epithelial neutrophils Medium-sized & small ducts as well as acini Destruction & obliteration of duct lumen Definite diagnosis requires histological examination * GEL: Granulocyte Epithelial Lesions Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    16. 16. Idiopathic Duct-Centric Pancreatitis (IDCP) H&E staining H&E staining Periductal inflammation Inflammatory cells few in fibrosisDestruction of pancreatic epithelia Microabscess in intra-lobular duct Suggested GEL GEL: Granulocyte Epithelial Lesions Kusuda T et al. Intern Med 2010 ; 49 : 2569 – 2575.
    17. 17. What to biopsy? Histopathology is diagnostic but not usually available• Pancreatic biopsy EUS-FNA: not reliable EUS-TCB: better sen & sp Surgery• Papillary biopsy Specific, not very sensitive• Intraductal BD biopsy Still under debate• Liver biopsy Not strictly necessary Maillette de BuyWenniger L et al. Endoscopy 2012 ; 44 : 66 – 73.
    18. 18. Diagnosis of AIP Combination of 1 or more of 5 cardinal features HISORt Histology LPSP – IDCP TCB/resection Imaging Parenchyma US – EUS – CT – PET – MRI Pancreatic Duct ERCP – MRCP Serology IgG4Other Organ Involvement IgG4-related diseases – IBD Response to therapy Steroid trial LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    19. 19. Ultrasonograpy in AIPTrans-abdominal transverse US Diffuse enlargement of pancreas Minimal decreased echotexture “sausage-like appearance”Sahani DV et al. Radiology 2004 ; 233 : 345 – 352.
    20. 20. EUS findings in autoimmune pancreatitis• Diffuse form Diffuse pancreatic enlargementChronic pancreatitis Reduced echogenicity Hyperechoic foci & strands• Focal form Solitary irregular hypoechoic massPancreatic cancer Upstream dilatation of MPD Vascular invasion of PV & MV Real-time tissue elastography Buscarini E et al. World J Gastroenterol 2011 ; 17 : 2080 – 2085.
    21. 21. Diffuse form of autoimmune pancreatitis EUS Diffuse pancreatic enlargement Parenchymal lobularity Echopoor echotexture Hyperechoic strandsLoss of interface with splenic vein Buscarini E et al. World J Gastroenterol 2011 ; 17 : 2080 – 2085.
    22. 22. Focal form of autoimmune pancreatitis EUS Interventional EUS Focal lesion of pancreatic head FNA: Sen 36% – Sp 33%Echopoor with hyperechoic strands TCB: Sen 100% – Sp 100% FNA first then TCB FNA: Fine Needle Aspiration – TCB: Tru-Cut Biopsy Mizuno N et al. J Gastroenterol 2009 ; 44 : 742 – 750. Buscarini E et al. World J Gastroenterol 2011 ; 17 : 2080 – 2085.
    23. 23. Focal form of AIP Real-time tissue elastography5 AIP – 17 ductal adenocarcinoma – 10 healthy subjectsStiff pattern of pancreatic mass & surrounding parenchyma Distinguishes AIP from ductal adenocarcinoma Dietrich CF et al. Endoscopy 2009 ; 41 : 718 – 720.
    24. 24. Localized form of AIPLocalized hypoechoic mass Hyperechoic inclusions “duct-penetrating sign” “tortoiseshell pattern” Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    25. 25. Enlarged lymph nodes in hepatic hilum Buscarini E et al. World J Gastroenterol 2011 ; 17 : 2080 – 2085.
    26. 26. EUS nodal features predicting metastasis• Size: > 1 cm in diameter on short axis• Hypoechoic appearance• Round shape• Smooth border Identified in esophageal cancer 1Inaccurate for other cancers including biliopancreatic 2 1 Catalano MF et al. Gastrointest Endosc 1994 ; 40 : 442 – 446. 2 Gleeson FC et al. Gastrointest Endosc 2008 ; 67 : 438 – 443.
    27. 27. CT scan in auto-immune pancreatitis• Diffusely or locally enlarged pancreas• Distinctive delayed enhancement pattern with various images depending on activity or stages of disease• Capsule-like rim: highly specific Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    28. 28. Dynamic CT of AIP Early imaging Delayed imagingSwollen pancreas Delayed gradual enhancement Low density „„capsule-like rim‟‟ Shimosegawa T & Kanno A. J Gastroenterol. 2009 ; 44 : 503 – 17.
    29. 29. Dynamic CT in auto-immune pancreatitis Diffusely enlarged pancreas Slow and delayed enhancement Capsule-like rim Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    30. 30. Positron emission tomography in AIP• Accumulation of FDG in pancreatic & extra-pancreatic lesions, which disappear shortly after steroid treatment• Characteristic accumulation pattern & kinetics in pancreatic & extra-pancreatic lesions after steroid treatment can be used for diagnosis of disease FDG: Fluoro Deoxy Glucose Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    31. 31. Whole-body FDG-PET imaging in AIP Before steroid After steroidFDG taken to pancreatic body & tail, FDG disappears shortly aftersalivary glands, pulmonary hilar LN starting steroid treatment & large pseudotumor of liver Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    32. 32. PET/CT scan image65-year-old man with autoimmune pancreatitis Diffuse pancreatic involvement Increased 18 F-FDG uptake in enlarged pancreas Bodily KD et al. Am J Roentol 2009 ; 192 : 431 – 437.
    33. 33. Magnetic resonance images of AIP• Diffusely enlarged pancreas with Low signal on T1-weighted images Delayed enhancement pattern on dynamic MRI• Capsule-like rim Strong fibrosis of peripancreatic lesion: highly specific Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    34. 34. MR imaging of AIP T2-weighted MRI Gd-enhanced MRI Swollen pancreas (low signal) „„Capsule-like rim‟‟„„Capsule-like rim‟‟ (low signal) Depicted more clearly Shimosegawa T & Kanno A. J Gastroenterol. 2009 ; 44 : 503 – 17.
    35. 35. ERCP criteria to diagnose AIP International multicentre study• 21 physicians from four centers in Asia, Europe & USA 40 ERPs: 20 AIP, 10 chronic pancreatitis, 10 pancreatic cancer• Phase I → Washout period (3 months) → Phase II• Key features Long stricture: > 1/3 length of PD Lack of upstream dilatation: < 5 mm Multiple strictures Side branches arising from strictured segment• Results Sen 71% – Sp 83% – IOA 0.40 Sugumar A et al. Gut 2011 ; 60 : 666 – 670.
    36. 36. ERCP criteria to diagnose AIP International multicentre studyAbility to diagnose AIP based on ERP features alone is limited Diagnosis improved with knowledge of some key features Sugumar A et al. Gut 2011 ; 60 : 666 – 670.
    37. 37. MRCP in auto-immune pancreatitis Narrowing of main pancreatic duct (tail)MRCP not recommended for accurate evaluation of MPD narrowing Okazaki K et al. Pancreas 2009 ; 38 : 849 – 866.
    38. 38. Diagnosis of AIP Combination of 1 or more of 5 cardinal features HISORt Histology LPSP – IDCP TCB/resection Imaging Parenchyma US – EUS – CT – PET – MRI Pancreatic Duct ERCP – MRCP Serology IgG4Other Organ Involvement IgG4-related diseases – IBD Response to therapy Steroid trial LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    39. 39. Serum IgG4 & autoimmune pancreatitis• Normal value 8 – 140 mg/dl• Initial reports Pathognomonic• Subsequent reports Characteristic not diagnostic• Sen & Sp 75% – 93%• PPV Low (not used alone for dg)• Level > 2 times ULN is highly specific Park DH et al. Gut 2009 ; 58 : 1680 – 1689.
    40. 40. Serum IgG4 in diagnosing AIP 510 patientsCutoff > 140 mg/dL: Sen 76% – Sp 93% – PPV 36%Cutoff > 280 mg/dL: Sen 53% – Sp 99% – PPV 75% Ghazale A et al. Am J Gastroenterol 2007 ; 102 : 1646 – 1653.
    41. 41. Diagnosis of AIP Combination of 1 or more of 5 cardinal features HISORt Histology LPSP – IDCP TCB/resection Imaging Parenchyma US – EUS – CT – PET – MRI Pancreatic Duct ERCP – MRCP Serology IgG4Other Organ Involvement IgG4-related diseases – IBD Response to therapy Steroid trial LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    42. 42. Predominantly extra-pancreatic presentation• Biliary stricture Resembling: PSC – Pancreatic cancer – Cholangiocarcinoma• Interstitial nephritis• Retroperitoneal fibrosis• Diffuse lymphoanenopathy• Sjögren‟s syndrome
    43. 43. Diagnosis of other organ involvement• Clinical examination Symmetrical salivary gland enlargement• Imaging Proximal bile duct stricture Retroperitoneal fibrosis Renal or pulmonary lesion• Histology Lymphoplasmacytic infiltrate > 10 IgG4 + plasma cells/hpf Storiform fibrosis Obliterative phlebitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    44. 44. Thickening of bile duct wall in AIPThree-layer type Parenchymal echo type Koyama R et al. Pancreas 2008 ; 37 : 259 – 264.
    45. 45. Biliary & peripancreatic findings in AIPDilated CBD upstream to distal funnel-shaped stenosis Diffuse thickening of biliary wall Enlarged lymph nodes in hepatic hilum Buscarini E et al. World J Gastroenterol 2011 ; 17 : 2080 – 2085.
    46. 46. Bile duct wall thickening “sandwich-pattern”Intermediate echo-poor layer & echo-rich inner & outer layers Buscarini E et al. Dig Liver Dis 2010 ; 42 : 92 – 98.
    47. 47. Thickening of IHBD Trans-abdominal US Parenchymal-echo type thickeningKoyama R et al. Pancreas 2008 ; 37 : 259 – 264.
    48. 48. Thickening of bile duct wall• Acute cholangitis More or less symmetric• Primary sclerosing cholangitis Asymmetric• Secondary sclerosing cholangitis Symmetric European Foundation of Societies of Ultrasound in Medicine & Biology. Barreiros AP et al. European Course Book – Ultrasound of the biliary sydtem – 2011.
    49. 49. Thickening of bile duct wall/Acute cholangitis More or less symmetrical thickening of bile duct walls European Foundation of Societies of Ultrasound in Medicine & Biology.Barreiros AP et al. European Course Book – Ultrasound of the biliary sydtem – 2011.
    50. 50. Thickening of bile duct wall/PSC Asymmetric thickening of bile duct walls Benign strictures & alternating dilatations European Foundation of Societies of Ultrasound in Medicine & Biology.Barreiros AP et al. European Course Book – Ultrasound of the biliary sydtem – 2011.
    51. 51. Secondary causes of sclerosing cholangitis Distinguishing PSC from SSC may be challenging Choledocholithiasis Recurrent pyogenic cholangitis Cholangiocarcinoma AIDS cholangiopathy Diffuse intrahepatic metastasis Eosinophilic cholangitis Hepatic inflammatory pseudo-tumor Histocytosis X IgG4-associated cholangitis Intra-arterial chemotherapy Ischemic cholangitis Portal hypertensive biliopathy Recurrent pancreatitis Surgical biliary trauma Chapman R et al. Hepatology 2010 ; 51 : 660 – 678.
    52. 52. AIP with common bile duct involvement Stenosis of the distal CBD ERCP hallmark of AIP Buscarini E et al. Dig Liver Dis 2010 ; 42 : 92 – 98.
    53. 53. Cholangiography in PSC & AIPPSC AIP Kawa S et al. J Gastroenterol 2010 ; 45 : 355 – 369.
    54. 54. Does ERC distinguish IgG4-associated cholangitis from PSC or cholangiocarcinoma?• 17 physicians from USA, Japan, & UK• Unaware of clinical data• 40 ERCs IgG4-associated cholangitis: 20 patients PSC: 10 patients Cholangiocarcinoma: 10 patients• Results Sensitivity: 45% Specificity: 88% Inter-observer agreement: 0.18 IAC may be misdiagnosed with PSC or cholangiocarcinoma Kalaitzakis E et al. Clinical Gastroenterol Hepatol 2011 ; 9 : 800 – 803.
    55. 55. ERC in IgG4-associated cholangitis & PSCIgG4-associated cholangitis PSC Difficulty to distinguish IAC from PSC based on ERC de BuyWenniger LM et al. Endoscopy 2012 ; 44 : 66 – 73.
    56. 56. In all patients with possible PSC, we suggest measuring serum IgG4 levelsto exclude IgG4-associated sclerosing cholangitis AASLD practice guidelines: Diagnosis & management of PSC. Chapman R et al. Hepatology 2010 ; 51 : 660 – 678.
    57. 57. HISORt criteria for diagnosis of AIP-SC H Lymphoplasmacytic sclerosing cholangitis on resection: Bile duct LP infiltrate, > 10 IgG4 + cells/hpf, storiform fibrosis, phlebitis I One or more strictures involving IH, EH, or intrapancreatic BD Bile duct Fleeting/migrating biliary strictures S IgG4 > 2 ULN value O Pancreas: Classic features of AIP on imaging or histology Suggestive imaging findings: mass, stricture, atrophy Retroperitoneal fibrosis Renal: single/multiple parenchymal low-attenuation lesions Salivary/lacrimal gland enlargement Rt Normalization of liver enzyme or resolution of BD strictureDefinitive dg Group A: diagnostic histology on resection or TCB Group B: typical imaging of AIP + serologyProbable dg Group C: ≥ 2 of suggestive pancreatic imaging, S, OOI & Rt Ghazale A et al. Gastroenterology 2008 ;134 :706 – 715.
    58. 58. IgG4-associated cholecystitisAnother clue in diagnosis of autoimmune pancreatitis Leise MD et al. Dig Dis Sci 2011 ; 56 : 1290 – 1294.
    59. 59. Duodenal papilla in AIP IgG4 immuno-staining of papilla in 19 AIP & 100 controls Sensitivity 53% – 100 % specificityEndoscopic view of papilla IgG4 immuno-staining Swollen duodenal papilla 50 IgG4-positive cells/HPF Kubota K et al. Gastrointest Endosc 2008 ; 68 : 1204 – 1208. Moon SH et al. Gastrointest Endosc 2010 ; 71 : 960 – 966.
    60. 60. AIP with idiopathic retroperitoneal fibrosis CECT scan CECT scan slightly inferiorDiffusely enlarged pancreas Bilateral peri-pelvic lesions Low-density rim Left peri-renal lesions Fukukura Y et al. Am J Roentgenol 2003 ; 181: 993 – 995.
    61. 61. Retroperitoneal fibrosisTransverse CT scan at level of origin of IMA Circumferential thickening of aortic wall with peri-aortic soft tissue Sahani DV et al. Radiology 2004 ; 233 : 345 – 352.
    62. 62. AIP with renal involvement Contrast-enhanced axial CTMultiple well-defined round lesions in both kidneys Bodily KD et al. AJR 2009 ; 192 : 431 – 437.
    63. 63. Diagnosis of AIP Combination of 1 or more of 5 cardinal features HISORt Histology LPSP – IDCP TCB/resection Imaging Parenchyma US – EUS – CT – PET – MRI Pancreatic Duct ERCP – MRCP Serology IgG4Other Organ Involvement IgG4-related diseases – IBD Response to therapy Steroid trial LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis IDCP: Idiopathic Duct-Centric Pancreatitis Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    64. 64. Steroid trial in AIP Mass in pancreatic body Long narrowing of MPD 0.6 – 1 mg/kg of oral prednisolone/day for 2 weeks Mass markedly reduced Almost normal MPD2-week steroid trial may be helpful to confirm diagnosis of AIP Moon SH et al. Gut 2008 ; 57 : 1704 – 1712.
    65. 65. IgG4-associated sclerosing cholangitis Before treatment After 12 weeks of steroid therapyIH strictures mimicking PSC Resolution of IH strictures Ghazale A et al. Gastroenterology 2008 ;134 :706 – 715.
    66. 66. Inflammatory pseudo-tumor Before steroid therapy After steroid therapyNodular lesion of inflammatory Nodular lesion disappeared pseudo-tumor Kawa S et al. J Gastroenterol 2010 ; 45 : 355 – 369.
    67. 67. Caution regarding steroid trial• Not to be used as substitute for thorough search for etiology• Do not use it if there is no objective way to define response
    68. 68. Challenges to diagnosing AIP• Closely mimics other well known diseases Pancreatic cancer & PSC: need high index of suspicion• Rare compared to diseases it mimics 2 – 3 % of patients suspected to have pancreatic cancer• No single test is diagnostic Histology is diagnostic but rarely available• Heavy price of misdiagnosis AIP mistaken for cancer results in major surgery Cancer mistaken for AIP results in delay in surgery
    69. 69. Diagnostic criteria for AIP Lack of universally accepted criteria Dg criteria References Japan Japan Pancreas Society. J Jpn Pancreas 2002;17:585-7. Okazaki K et al. J Gastroenterol 2006;41 626-31. Okazaki K et al. Pancreas 2009;38: 849-866. Pearson RK et al. Pancreas 2003;27:1-13. Italy Frulloni L et al. Am J Gastroenterol 2009;104:2288-94. Korea Kim KP et al. World J Gastroenterol 2006;12:2487-96.US (Mayo Clinic) Chari ST et al. Clin Gastroenterol Hepatol 2006;4:1010-6. HISORt Chari ST et al. Clin Gastroenterol Hepatol 2009;7:1097-2003. Asia Otsuki M et al. J Gastroenterol 2008;43:403-408. Germany Schneider A & Löhr JM. Internist (Berlin) 2009;50:318-330.
    70. 70. Why an international consensus on AIP?• ERP Routinely used in Japan (mandatory criterion) AIP diagnosed without ERP in the West• Biopsy Core biopsy for diagnosis by Mayo Clinic group Not routinely used elsewhere• 2 types Asian & American criteria diagnose type 1 Italian criteria have mixture of types 1 & 2 Criteria applied worldwide Safely diagnose AIP Avoid misdiagnosis of AIP as pancreatic cancer or PSC Chari ST et al. Pancreas. 2010 ; 39 : 549 – 554. Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    71. 71. Honolulu consensus conference on AIP 1AIP International Cooperative Study GroupHonolulu, Hawaii: November 4, 200933 international experts – Categorization into type 1 & type 2 International consensus diagnostic criteria for AIP 214th congress of International Association of PancreatologyFukuoka, Japan: July 11 – 13, 201014 international experts – Consensus opinion of working group 1 ChariST et al. Pancreas. 2010 ; 39 : 549 – 554. 2 Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    72. 72. Level 1 & level 2 criteria for type 1 AIP Criterion Level 1 Level 2Histology of pancreas LPSP (TCB or resection) LPSP (TCB) At least 3 of 4 At least 2 of 4Parenchyma imaging Typical Indeterminate/atypical* Diffuse enlargement Segmental/focal enlargement Delayed enhancement rim Delayed enhancement Ductal imaging Long stricture (>1/3 MPD) Segmental/focal narrowing (ERP) or multiple strictures without dilatation (< 5 mm) without dilatation (< 5mm) Serology IgG4: > 2 ULN IgG4: 1 – 2 ULN OOI a: histology (3 of 4) a: histology (LP & >10 /hpf) a or b b: radiology (bile duct, RPF) b: clinic (salivary, lachrymal) radiology (renal lesion)Response to therapy Rapid (≤ 2 wk) radiological resolution/marked improvement LPSP: Lympho-Plasmacytic Sclerosing Pancreatitis * Atypical parenchymal imaging: low-density mass, ductal dilatation, distal atrophy Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    73. 73. Diagnosis of definitive & probable type 1 AIPDiagnosis Basis for dg Imaging evidence Collateral evidence Histology Typical/indeterminate LPSP (level 1 H) Imaging Typical/indeterminate Any non-D level 1/ level 2Definitive ≥ 2 from level 1(+level 2 D) Response to steroid Indeterminate Level 1 S/O or Level 1 D + level 2 S/O/HProbable Indeterminate Level 2 S/O/H + Rt Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    74. 74. Level 1 & level 2 criteria for type 2 AIP Criterion Level 1 Level 2Histology of pancreas ICDP: both of the following Both of the following: (TCB or resection) 1- GEL G acinar inflamation 1- LP & G acinar infiltrate 2- Scanty to no IgG4 + cells 2- Scanty to no IgG4 + cellsParenchyma imaging Typical Indeterminate/atypical* Diffuse enlargement Segmental/focal enlargement Delayed enhancement rim Delayed enhancement Ductal imaging Long stricture (>1/3 of MPD) Segmental/focal narrowing (ERP) or multiple strictures without dilatation (< 5 mm) without dilatation (< 5mm) OOI – Clinically diagnosed IBDResponse to therapy Rapid (≤ 2 wk) radiological resolution/marked improvement after negative workup for cancer including EUS-FNA GEL: Granulocyte Epithelial Lesions IDCP: Idiopathic Duct-Centric Pancreatitis Atypical parenchymal imaging: low-density mass, ductal dilatation, distal atrophy Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    75. 75. Diagnosis of definitive & probable type 2 AIPDiagnosis Imaging evidence Collateral evidenceDefinitive Typical/indeterminate Histologically confirmed IDCP (level 1 H) or clinical IBD + level 2 H + RtProbable Typical/indeterminate Level 2 H / clinical IBD + Rt Shimosegawa T et al. Pancreas 2011 ; 40 : 352 – 358.
    76. 76. References
    77. 77. Thank You

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