Autoimmune Pancreatitis

Posted by e-Medical PPT
AUTOIMMUNE PANCREATITIS - chronic pancreatitis with distinct clinical, serological, histological and imaging features and it is involved in hyper- IgG4 group of diseases.

HISORt CRITERIAS OF AUTOIMMUNE PANCREATITISHistology
1. Diagnostic (any one):
    a) Pancreatic histology showing periductal lymphoplasmacytic
        infiltrate with obliterative hlebitis (LPSP)
    b) Lymphoplasmacytic infiltrate with abundant (>10 cells/hpf) IgG4
        positive cells in the pancreas
2. Supportive (any one)
    a) Lymphoplasmacytic infiltrate with abundant (>10 cells/hpf) IgG4
        positive cells in involved extra-pancreatic organ
    b) Lymphoplasmacytic infiltrate with fibrosis in the pancreas

Imaging
Typical imaging features:
1. CT/MR: diffusely enlarged gland with delayed (rim) endhancement
2. ERCP: Diffusely irregular, attenuated main pancreatic duct
Atypical Imaging Features: Pancreatitis, focal pancreatic mass, focal pancreatic duct stricture, pancreatic atrophy, pancreatic calcification

Serology
Elevated serum IgG4 level (normal 8-140 mg/dl)

Other Organ involvement
Hilar/intrahepatic biliary strictures, persistent distal biliary stricture, Parotid/lacrimal gland involvement, Mediastinal lymphadenopathy, Retroperitoneal fibrosis

Response to steroid therapy
Resolution/marked improvement of pancreatic/extrapancreatic manifestation with steroid therapy

CLINICAL DIAGNOSTIC CRITERIA FOR AIP 2006
     Diffuse or segmental narrowing of the MPD with irregular wall and diffuse or localized enlargement of the pancreas by imaging studies, such as abdominal US, CT, and magnetic resonance
     High serum γ-globulin, IgG, or IgG4, or the presence of autoantibodies such as antinuclear antibodies and rheumatoid factor
     Marked interlobular fibrosis and prominent infiltration of lymphocytes and plasma cells in the periductal area,     occasionally with lymphoid follicles in the pancreas

Diagnosis of AIP is established when criterion 1 and criterion 2 and/or 3 are fulfilled. However, it is necessary to exclude malignant diseases

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