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IgG4 Pancreatitis. Dr Chan Lok Lam Laura United Christian Hospital JHSGR 6 th Aug, 2011. IgG4 pancreatitis. Recently described disorder with protean manifestations Important diagnostic consideration in patients with obstructive jaundice associated with pancreatic mass lesion

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Igg4 pancreatitis

IgG4 Pancreatitis

Dr Chan Lok Lam Laura

United Christian Hospital

JHSGR 6th Aug, 2011


Igg4 pancreatitis1
IgG4 pancreatitis

  • Recently described disorder with protean manifestations

  • Important diagnostic consideration in patients with obstructive jaundice associated with pancreatic mass lesion

  • Mimics pancreatic cancer clinically and radiologically

  • Dramatic response to steroid

  • Correct diagnosis allows medical treatment and avoids major surgery


Igg4 pancreatitis autoimmune pancreatitis
IgG4 pancreatitis = autoimmune pancreatitis?

  • In previous literature  YES!

  • Concept evolving

Autoimmune pancreatitis (AIP)

Type I AIP (IgG4 pancreatitis)Pancreatic manifestation of systemic IgG4-related disease

Type II AIP

Specific pancreatic disease occasional association with ulcerative colitis


Igg4 pancreatitis2
IgG4 pancreatitis

  • Chronic inflammatory disease of presumed autoimmune origin

  • Pathogenesis not well understood

  • Lymphoplasmacytic infiltration with abundant IgG4 positive cells

  • Inflammatory process responds well to steroid therapy


Epidemiology
Epidemiology

  • Uncommon

  • 0.82 per 100,000 patients in a Japanese nationwide survey (2002)

  • 4.6-6% in patients with chronic pancreatitis

  • 3-5% undergoing pancreatic resection for suspected pancreatic cancer


Epidemiology1
Epidemiology

  • Elderly Male


Extra pancreatic manifestations
Extra-pancreatic manifestations

  • Biliary strictures

  • Sclerosing sialadenitis

  • Retroperitoneal fibrosis

  • Sclerosing cholecystitis

  • Interstitial nephritis

  • Diffuse lymphadenopathy

  • Characteristic lymphoplasmacytic infiltrate rich in IgG4-positive cells

  • Can precede/ accompany / follow pancreatic involvement


Clinical presentation
Clinical presentation

  • Painless obstructive jaundice (65%)

  • Vague abdominal pain

  • Weight loss

  • Exocrine insufficiency (88%)

  • Endocrine dysfunction (67%)


Laboratory findings
Laboratory findings

  • Amylase/ lipase: normal/ mildly elevated

  • Gamma globulin, total IgG, IgG4

    • Commonly elevated

    • Serum IgG4 :

      • 140 mg/dl: Sensitivity 76%; Specificity 93%

      • 280 mg/dl: Sensitivity 53%; Specificity 99%

      • Elevated in 7-10% cases of Pancreatic CA (usually mild)

  • Autoantibodies

    • ANA, RF: elevated (non-specific)


Radiological
Radiological

  • CT/ MRI:

    • Diffuse enlargement of the entire pancreas ‘sausage-like’

    • Low density capsule-like rim due to inflammation and fibrosis

    • Delayed contrast enhancement


Ct mri
CT/ MRI

  • Focally enlarged pancreas ‘inflammatory mass’


Ercp mrcp
ERCP/ MRCP

  • Diffuse narrowing of main pancreatic duct


Ercp mrcp1
ERCP/ MRCP

  • Segmental narrowing of main pancreatic duct

  • Biliary stricture ( can occur anywhere )



Eus guided fnac
EUS guided FNAC

  • Detecting adenocarcinoma

    • Sensitivity 70-90%

    • Negative bx does not rule out CA

  • Not for diagnosis of IgG4 pancreatitis

    • Inadequate cells

    • Lack of architecture


Eus guided core biopsy
EUS guided core biopsy

  • Allow diagnosis of IgG4 pancreatitis

  • Technically difficult

  • Increased risk of bleeding

  • Not widely available


Biopsy of extra pancreatic site
Biopsy of extra-pancreatic site

  • Bile ducts, major duodenal papilla

  • 80% pancreatic head involvement had IgG4-positive cells on biopsy of the major duodenal papilla



Response to steroid1
Response to steroid

  • Radiographic response seen at 2-3 wks and normalization at 4-6 wks


Response to steroid2
Response to steroid

  • Steroid trial controversial

  • No response within 2 weeks makes IgG4 pancreatitis unlikely

  • Failed response to steroid

    • Prompt re-evaluation of diagnosis

    • Consider surgery to look for cancer


  • Making the correct diagnosis is challenging

    • Rare disease

    • Mimic the more common pancreaticobiliary malignancy

    • No single diagnostic test available

  • Price of misdiagnosis is heavy

    • Unnecessary surgery for benign disease

    • Delay potentially curative surgery


Japanese diagnostic criteria
Japanese Diagnostic Criteria

1. Imaging

  • Diffuse/ segmental narrowing of main pancreatic duct

  • Diffuse/ localized enlargement of pancreas

    2. Serology

  • Elevated gamma-globulin, IgG or IgG4 OR

  • Presence of autoantibodies eg ANA/ RF

    3. Histology

  • Lymphoplasmacytic sclerosing pancreatitis

    Diagnosis: 1 + 2/3


Take home message
Take Home Message

  • Increasing recognition

  • Important diagnostic consideration in obstructive jaundice due to pancreatic mass lesion

  • High index of suspicion

  • Multidisciplinary collaboration

    • Surgeons/GI physician/Radiologist/Pathologist



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