Type 2 Idiopathic ductcentric pancreatitis GELs granulocite ephitelial lesions IgG4 Related Diseases Various organ manifestations of a fibroinflammatory condition c haracterized by ID: 774723
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Slide1
RECAP: Autoimmune Pancreatitis
Type 2: Idiopathic duct-centric pancreatitis
GELs: granulocite, ephitelial lesions
Slide2IgG-4 Related Diseases
Various
organ
manifestations of a fibro-inflammatory
condition
c
haracterized 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.
Slide3Pathophysiology
Stone J, Zen Y. IgG4-Related Disease N Engl J Med 2012;366:539-51.
Slide4IgG-4 Related Diseases
Slide5IgG-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
Slide6IgG-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
Slide7IgG-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
Slide8IgG4 Pancreatitis: DD
Slide9IgG-4 Pancreatitis
Role of EUS-FNA Histological proof of the disease: GOLD STANDARD Exclusion of carcinoma Discrimination of type 1 from type 2 AIP
IgG4-positive plasma cells> 10/hpf
Diffuse lymphoplasmacytic
infiltration and storiform fibrosis
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
Ratio
IgG4
-positive/
IgG
-positive plasma cells > 40%
Slide11Storiform Fibrosis
Slide12IgG-4 Pancreatitis: diagnosis
The MAYO Clinic HISORt criteria
Slide13IgG-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
Slide14IgG-4 Cholangitis
Imaging features Isolated intrapancreatic CBD strictures Localized hilar hepatic lesion (strictures or masses) Intense and diffuse extension of bile duct wall homogeneous thickening (often circular) IDUS: inflamed submucosa and preserved epithelium, circular symmetric wall thickening
Slide15IgG-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
Slide16IgG-4 Cholangitis DD
IDUS: all bile duct layers inflamed, bile duct epithelium severely damaged, disappearance of the three layersImaging: diverticulum-like out-pouching, and beaded - tree appearance
Slide17INDUCTION 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)
Therapy
Slide18Outcomes
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
Slide19Outcomes
Response to steroid therapy in 90% to 95% of both parenchymal and ductal changes
Before (A) steroid therapy and after (B)
Slide20Suspect!May have acute or chronic manifestationTypical imaging findings (CT/MRI)Mandatory is exclusion of carcinomaCharacteristic histopathological appearance: histology is the GOLD STANDARDNot always elevated IgG and IgG-4 levelsSystemic disease: look for biliary and retroperitoneal involvement
TAKE HOME MESSAGES
Slide21Stone 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.
Bibliografia