1 / 21

RECAP: Autoimmune Pancreatitis

RECAP: Autoimmune Pancreatitis. Type 2: Idiopathic duct-centric pancreatitis GELs: granulocite, ephitelial lesions. IgG-4 Related Diseases. Various organ manifestations of a fibro-inflammatory condition c haracterized by tumefactive lesions recognised as a systemic condition in 2003

byfield
Download Presentation

RECAP: Autoimmune 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. RECAP: Autoimmune Pancreatitis Type 2: Idiopathic duct-centric pancreatitis GELs: granulocite, ephitelial lesions

  2. IgG-4 Related Diseases Various organ manifestations of a fibro-inflammatory condition characterized by tumefactive lesions recognised as a systemic condition in 2003 Linked by the same histopathological characteristics and elevated serum IgG4 concentrations. Multiple immune-mediated mechanism contribute to the fibro-inflammatory process, being autoimmunity and infectious agents potential triggers of a Th-2 and T-reg response.

  3. Pathophysiology Stone J, Zen Y. IgG4-Related Disease N Engl J Med 2012;366:539-51.

  4. IgG-4 Related Diseases

  5. IgG-4 Related Pancreato-biliary Diseases Majority of patients are men (60-73%), > 60 years Prevalence 0,2-0,8/100.000 Lack of familiarity! Up to 40% of patients have allergic diseases (atopy, eczema, asthma, chronic sinusitis) 30% normal serum IgG-4 concentrations IgG-4 related sclerosing cholangitis associated with AIP in 47–92% of pt

  6. IgG-4 Pancreatitis Clinical presentation Acute vs Chronic symptoms Painless obstructive jaundice 33–60% Steatorrhea - exocrine functional abnormalities in up to 80% Abdominal pain 32% Back pain and weight loss 15% Serologic marker:  titers of g-globulin (>2000 mg/dL)  IgG (>1800 mg/dL)  IgG4 (>140 mg/dL) Serum IgG4 >140 mg/dL 86% SN, 96% SP

  7. IgG-4 Pancreatitis Imaging features Enlarged pancreas “sausage-like appareance” 50-70% Focal masses 30% Soft tissue hypoenhancing rim Narrowing of main pancreatic duct “Duct-penetrating” sign at secretin-MRCP

  8. IgG4 Pancreatitis: DD

  9. IgG-4 Pancreatitis Role of EUS-FNA Histological proof of the disease: GOLD STANDARD Exclusion of carcinoma Discrimination of type 1 from type 2 AIP Diffuse lymphoplasmacytic infiltration and storiform fibrosis IgG4-positive plasma cells > 10/hpf

  10. IgG-4 Pancreatitis Hystology is the GOLD STANDARD: Diffuse lymphoplasmacytic infiltration with mild-moderate eosinophilia Obliterative flebitis and storiform fibrosis IgG4 immunostaining: > 50 IgG4 plasma cells/HPF for surgical specimens > 10 IgG4 plasma cells/HPF for biopsy samples RatioIgG4-positive/IgG-positive plasma cells > 40%

  11. Storiform Fibrosis

  12. IgG-4 Pancreatitis: diagnosis The MAYO Clinic HISORt criteria

  13. IgG-4 Cholangitis Clinical presentation 70% obstructive jaundice with pruritus and abdominal pain Asymptomatic jaundice less common than AIP 7-10% cirrhosis manifestation (hepatic failure, ascites, hepatic encephalopathy or variceal bleeding) Serologic marker:  titers of g-globulin (>2000 mg/dL)  IgG (>1800 mg/dL)  IgG4 (>140 mg/dL)  reumatoid factor, antinucleus antibody

  14. IgG-4 Cholangitis Imaging features Isolated intrapancreatic CBD strictures Localized hilar hepatic lesion (strictures or masses) Intense and diffuse extension of bile duct wall homogeneousthickening (often circular) IDUS: inflamed submucosa and preserved epithelium, circular symmetric wall thickening

  15. IgG-4 Cholangitis DD IDUS: eccentric wall thickening with an irregular luminal surface, disruption of the bile duct wall layered structure, and a hypoechoic mass with irregular margins

  16. IgG-4 Cholangitis DD IDUS: all bile duct layers inflamed, bile duct epithelium severely damaged, disappearance of the three layers Imaging: diverticulum-like out-pouching, and beaded - tree appearance

  17. Therapy INDUCTION 0,6-1 mg/kg oral prednisolone TAPERING 5 mg/wk reduction MAINTENANCE: 2,5-5 mg/die oral prednisolone or AZA Pancreatic enzyme supplementation (pancrelipasis) for exocrine insufficiency if: steatorrhoea, weight loss, metabolic bone disease, vitamin deficiency Oral hypoglicemic agents or insulin for diabetes mellitus Biliary stenting in obstructive jaundice (case-by-case)

  18. Outcomes Spontaneous resolution in up to 30% Response to steroid therapy in 90% to 95% with improvement of imaging findings and serology within 2 weeks + symptoms regression New onset diabetes mellitus usually improves with corticosteroid therapy Chronic pancreatitis in about 10% Very rarely progression to cirrhosis in IgG-4 cholangitis

  19. Outcomes Response to steroid therapy in 90% to 95% of both parenchymal and ductal changes Before (A) steroid therapy and after (B)

  20. TAKE HOME MESSAGES • Suspect! • May have acute or chronic manifestation • Typical imaging findings (CT/MRI) • Mandatory is exclusion of carcinoma • Characteristic histopathological appearance: histology is the GOLD STANDARD • Not always elevated IgG and IgG-4 levels • Systemic disease: look for biliary and retroperitoneal involvement

  21. Bibliografia • Stone J, Zen Y. IgG4-Related Disease N Engl J Med 2012; 366: 539-51. • Kamisawa T, Zen Y. Advances in IgG4-related pancreatobiliary diseases. Lancet Gastroenterol Hepatol 2018; 3: 575–85. • Sandrasegaran K, Menias C. Imaging in Autoimmune Pancreatitis and Immunoglobulin G4–Related Disease of the Abdomen. Gastroenterol Clin N Am. 2018. Article in press.

More Related