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Autoimmune pancreatitis. Petr Dt Dept. of Hepatogastroenterology Univ. Hospital Brno Czech Republic. Incidence of Chronic Pancreatitis. Switzerland1.2/100 000/year Poland 4.0/100 000/year Germany7.4/100 000/year Czech Rep.7.9/100 000/year - PowerPoint PPT Presentation

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  • Autoimmune pancreatitisPetr Dt

    Dept. of HepatogastroenterologyUniv. Hospital Brno Czech Republic

  • Incidence of Chronic PancreatitisSwitzerland1.2/100 000/yearPoland 4.0/100 000/yearGermany7.4/100 000/yearCzech Rep.7.9/100 000/yearHungary8.0/100 000/yearDenmark 10.0/100 000/yearSweden 10.0/100 000/yearFinland 23.0/100 000/yearUnited States 5.7-7.6/100 000/year

  • Chronic pancreatitis is a progressive inflammatory disease of the pancreas with irreversible damage of pancreatic tissue exocrine and endocrine insufficiency

  • -oxic-metabolic -diopathic -enetic-utoimmune-ecurrent acute pancreatitis-bstructive Etemed, Whitcomb, 2001

    TIGARO ClassificationTI GARO

  • AUTOIMMUNE PANCREATITIS - chronic pancreatitis with distinct clinical, serological, histological and imaging features and it is involved in hyper- IgG4 group of diseases.

  • Autoimmune pancreatitis1961H. SarlesChronic inflammatory sclerosis of the pancreas(Patients with jaundice, painful crises, hyperglobulinemia, no dilatation of pancreatic duct, lymphatic infiltration)

    R. Waldram et al Chronic pancreatitis, sclerosing cholangitis and sicca sy in two siblings

    S. Nakano et alVanishing tumor of the abdomen in patient with Sjgrens sy

    K. YoshidaConcept of autoimmune pancreatitis

    2001B. Etemed, D. Whitcomb TIGARO classification

  • Epidemiology of autoimmune pancreatitisJapan21/4514,6% Yoshida et al. Dig.Dis.Sci. 1995

    Korea17/3155,4% Kim et al. Am.J.Gastroenterol. 2004

    Italy23/3836,0%Parson et al. Pancreas 2003

    Czech Rep. 9/1854,8%Dite et al Best Practice and Res. Clin. Gastroent., 2008

  • Sex ang age onset of autoimmune pancreatitisNishimori I. et al., Gastroent., 2007

  • Antibodies in patients with AIP%Okazaki et al.J. Gastroent. 2001

    Graf1

    75ANAANA

    75ALFALF

    55ACAIIACAII

    25RFRF

    15ASMAASMA

    ada 1

    ada 2

    ada 3

    75%

    75%

    55%

    25%

    15%

    List1

    ada 1ada 2ada 3

    ANA752.42

    ALF754.42

    ACAII551.83

    RF252.85

    ASMA15

    Chcete-li zmnit velikost oblasti dat grafu, pethnte jej prav doln roh.

  • HISORt CRITERIAS OF AIP

  • CLINICAL DIAGNOSTIC CRITERIA FOR AIP 2006 Diffuse or segmental narrowing of the MPD with irregular wall and diffuse or localized enlargement of the pancreas by imaging studies, such as abdominal US, CT, and magnetic resonance High serum -globulin, IgG, or IgG4, or the presence of autoantibodies such as antinuclear antibodies and rheumatoid factor Marked interlobular fibrosis and prominent infiltration of lymphocytes and plasma cells in the periductal area, occasionally with lymphoid follicles in the pancreas

    Diagnosis of AIP is established when criterion 1 and criterion 2 and/or 3 are fulfilled. However, it is necessary to exclude malignant diseases.

  • AUTOIMMUNE PANCREATITIS - SUBTYPESTYP 1 LYMPHOPLASMATIC SCLEROSING PANCREATITIS LPSP - PERIDUCTAL LYMPHOPLASMATIC INFILTRATE - HIGH AMMOUNT IgG4 - POSITIVE PLASMA CELLS - SWIRLING FIBROSIS - OBLITERATIVE VENULITISTYP 2 IDIOPATHIC DUCT-CENTRIC PANCREATITIS IDCP (non-alcoholic duct destructive pancreatitis) - DUCTAL EPITHELIAL GRANULOCYTIC INFILTRATION

    DUCTAL DAMAGE

    OBLITERATION

  • COMPARISON OF TYPE 1 AND TYPE 2 AIPAIP,autoimmune pancreatitis, IgG4, immunoglobulin G4

  • CLINICAL PRESENTATIONS OF TYPE 1 AUTOIMMUNE PACREATITISClinical presentations of type I AIPPancreaticPredominantly extra-pancreatic

    Biliary stricture, sclerosing cholangitis Interstitial nephritis, renal failureRetroperitoneal fibrosiswith complications(e.g., ureteral obstruction)AcutePost-acute/lateObstructive jaundicePancreatitisSteatorrheaPersistent massSteatorrheaCalcification,atrophyPark, D.H. 2009

  • AUTOIMMUNE PANCREATITIS23,0% FOCAL FORM(LIKE MALIGNANT LESION)DIFFUSE FORM 77,0%(LIKE ACUTE PANCREATITIS)

  • Scattergram of IgG4 values for patients with autoimmune pancreatitis and related diseases. PBC primary biliary cirrhosis, PSC primary sclerosing cholangitisKawa et al., Gastroent., 2007

  • Usefulness of IgG4 in differentiating between pancreatic cancer and autoimmune pancreatitisKawa et al., Gastroent., 2007

  • Abundant IgG4 bearing plasma cell infiltration in patients with autoimmune pancreatitis and gastric ulcer23 pts with AIP and 230 control patients examined by EGDIn 8 pts with autoimmune pancreatitis gastric ulcer was found (34.8%). In control group during EGD was gastric ulcer found in 31 pts (13.3%) = p.0007Conclusion: AIP is closely associated with gastric ulcer with abundant IgG4-bearing plasma cell infiltrationShinji, A. et al.Gastrointest. Endosc. 2004

  • SET OF PATIENTS WITH AUTOIMMUNE PANCREATITIS(N = 10)Dt,P. al 2010One patient died during hospitalization pancreatic cancer

  • Review of AIP cases with systemic extrapancreatic lesionsOhara et al, Pancreas 2005

  • AUTOIMMUNE PANCREATITIS IN PATIENTS WITH IDIOPATHIC CHRONIC PANCREATITIS66 PATIENTS WITH IDIOPATHIC CHRONIC PANCREATITIS /ICP/

    AUTOIMMUNE DISEASE WAS PRESENT IN 10 PATIENTS (UC 5 pts, PSC 2 pst, Sjgren sy 1 pts, Hashimotos thyroiditis 1 pts, Graves disease 1 pts)

    POSITIVITY OF BIOCHEMICAL AND CLINICAL PARAMETRES IN 40%

    CONCLUSION: CLINICAL OR BIOCHEMICAL AUTOIMMUNE STIGMATA ARE PRESENT IN 40% pts WITH ICP, AUTOIMMUNE MECHANISMS MAY BE FREQUENT IN ICP.Uzan,K.N. et al. Clin Gastroent. Hepatol. 2005

  • CHRONIC PANCREATITIS IN CHILDREN AUTOIMMUNE ETIOLOGY?In the set of 31 children (age 3-18 years) markers of AIP were found in 17 pts (41,5%)Genetic markers 10 pts (32,5%)

    Oracz G. et al, Clin Gastroent Hepat 2006

  • Steroid therapy in patients with AIPInitial doses30 40 mg per day for 2 4 weeksThe steroid therapy could be stopped after the period of 6 12 months.Monitoring of laboratory and clinical symptoms are essential.When AIP still appears after steroid therapy --- re-evaluation should be carried out taking pancreatic CARCINOMA into consideration! J.Jpn.Pacreas Soc., 2002

  • THERAPEUTIC OPTIONS IN PATIENTS WITH AIPMAYO CLINIC 11 WEEKS STEROIDS WITH TAPPERING DOSE 5 mg / WEEKKIM 1 mg/kg FOR 4 WEEKS AND TAPPERING THE DOSE 5 mg/WEEKFRULLONI 0,5 mg/kg FOR 4 WEEKS AND TAPPERING THE DOSE 5 mg/WEEK

    UNEFFECTIVE THERAPY PANCREATIC CANCER

  • Long-term follow up study treating patients with AIPSteroid therapy

    60 mg/day1 pts40 mg/day1 ptsDuration from 21 37 months30 mg/day7 pts5 mg/day1 pts

    Dose was tappered by 2.5 5.0 mg every two weeksMaintenance therapy: 5mg dailyFollow up period 4 years 6 montsKamisawa et al.Pancreatology 2005

    23 patients with AIPPancreatoduode nectomy (N=6)Choledocho duodenostomy (N=4)Supportive therapy (N=3)Steroids (N=10)

  • Long term therapy patients with AIP - prognosisKamisawa et al.Pancreatology 2005

  • AIP ENDOCRINE AND EXOCRINE FUNCTION AFTER STEROID THERAPY21 CASES AIP WITH STEROID THERAPY10 CASES WITH EXOCRINE INSUFICIENCYNORMALIZATION8NO CHANGE211 CASES WITH DIABETES MELLITUSIMPROVEMENT5AGGRAVATION3NO CHANGE3Ito et al. 2007

  • Recurrence of autoimmune pancreatitisTakayama et al (Amer.J.Gastroent. 2004)42(11)26%Wakabyashi et al.(Pancreas 2005)36( 6)17%Zamboni et al. Wirchow Arch. 2004)22( 5)23%Kim et al. (A.J. Gastroent. 2004)17( 1) 6%Ramisawa et al. (J.Gastroenterol. 2007)32( 2) 6%

  • THE THERAPY OF AIP RECCURENCE STEROID + AZATHIOPRINE 1mg/kg 2mg/kg FOR 11 WEEKS

    Mycophenolate or Rituximab are not effective

    S.CHari Abstr. DDW 2009

  • AUTOIMMUNE PANCREATITIS VS PANCREATIC CANCER - RADIOLOGIC IMAGINGKim et al., 2004

    Autoimmune pancreatitisPancreatic cancerComplete cutoff of main pancreatic ductUncommonCommonDuctal strictureMultipleLocalized (Single)Upstream duct dilatationMildMarkedDuct in the massPresentAbsentDiffuse swelling of the pancreasAlmost alwaysRareDouble duct signCommonCommon

  • Usefulness of IgG4 in differentiating between pancreatic cancer and autoimmune pancreatitisKawa et al., Gastroent., 2007

  • COMPARISON OF SUBJECTS WITH AIP AND PANCREATIC CANCERGhazele A. et al. 2007

  • CONCLUSION AND PRACTICE POINTSAIP IS NOT FREQUENT DISEASE IN EUROPE, MORE FREQUENT IN ASIA. CLINICAL SYMPTOMS ARE USUALLY MILD (MOSTLY ABDOMINAL DISCOMFORT WITHOUT PAIN ATTACKS)IN CT, EUS OR US-DIFFUSE ENLARGEMENT OF PANCREAS (sausage pancreas), IN NMR-CP OR ERCP IRREGULAR NARROWING OF THE MAIN PANCREATIC DUCT ARE TYPICALPRESENCE NON-SPECIFIC ANTIBODIES IN BLOOD SERUM AND INCREASED LEVEL OF IgG AND IgG4 IN SERUM AND TISSUEASSOCIATION WITH OTHER AUTOIMMUNE DISEASE-TYP1 AIPPANCREATIC CALCIFICATIONS AND/OR CYSTOIDS ARE NOT FREQUENTTHERAPY WITH STEROIDS IS EFFECTIVEIN DIF. DG DIAGNOSIS AIP VS PANCREATIC CANCER EUS GUIDED BIOPSY IS FUNDAMENTAL PROCEDURE

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