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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
slide21

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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