| Autoimmune Pancreatitis and Cholangitis |
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Autoimmune Pancreatitis and Cholangitis
Autoimmune pancreatitis (AIP) is a disease mainly affecting men aged 60-70 yrs. Features of this disease include absence of severe pain (attacks) and absence of major serum elevations of amylase and/or lipase levels, prominent weight loss, jaundice, imaging studies showing (focal or diffuse) swelling of the pancreas with or without a pancreatic rim, endocrine - and exocrine pancreatic insufficiency, elevated serum IgG and/or IgG4 levels and a favorable response to treatment with corticosteroids. There is an increased prevalence of other auto-immune mediated disorders such as retroperitoneal fibrosis, interstial nephritis and Sjogren's disease. Given the clinical and radiological features this disease may mimic pancreas cancer. AIP can also be accompanied by extrapancreatic intra- and extrahepatic biliary strictures that may mimic cholangiocarcinoma or PSC. This entity has been described as autoimmune pancreatico-cholangitis (APC), autoimmune pancreatitis and cholangitis, IgG4 related pancreatic and biliary disease, IgG-4 systemic disease a.o. Current evidence indicates that APC is the predominant phenotype of this disease in the Netherlands. Present studies include: - multicentre case evaluation of patients with AIP and APC
- evaluation of cases with previous Whipple resection but histology showing benign disease
- assessment of the diagnostic value of IgG, IgG4 and a panel of antibodies
- analysis of radiological features of AIP versus pancreas cancer
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