1 / 24

IgG4 Pancreatitis

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

lexiss
Download Presentation

IgG4 Pancreatitis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. IgG4 Pancreatitis Dr Chan Lok Lam Laura United Christian Hospital JHSGR 6th Aug, 2011

  2. 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

  3. 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

  4. 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

  5. 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

  6. Epidemiology • Elderly Male

  7. 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

  8. Clinical presentation • Painless obstructive jaundice (65%) • Vague abdominal pain • Weight loss • Exocrine insufficiency (88%) • Endocrine dysfunction (67%)

  9. 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)

  10. Radiological • CT/ MRI: • Diffuse enlargement of the entire pancreas ‘sausage-like’ • Low density capsule-like rim due to inflammation and fibrosis • Delayed contrast enhancement

  11. CT/ MRI • Focally enlarged pancreas ‘inflammatory mass’

  12. ERCP/ MRCP • Diffuse narrowing of main pancreatic duct

  13. ERCP/ MRCP • Segmental narrowing of main pancreatic duct • Biliary stricture ( can occur anywhere )

  14. Differentiation

  15. 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

  16. EUS guided core biopsy • Allow diagnosis of IgG4 pancreatitis • Technically difficult • Increased risk of bleeding • Not widely available

  17. 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

  18. Response to steroid • Dramatic

  19. Response to steroid • Radiographic response seen at 2-3 wks and normalization at 4-6 wks

  20. 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

  21. 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

  22. 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

  23. 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

  24. The END

More Related