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2015 ; 6
(4
): 199-206
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Autoimmune pancreatitis and cholangitis
#MMPMID26558153
Jani N
; Buxbaum J
World J Gastrointest Pharmacol Ther
2015[Nov]; 6
(4
): 199-206
PMID26558153
show ga
Autoimmune pancreatitis (AIP) is part of a systemic fibrosclerotic process
characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4
(IgG4) positive cells. It characteristically presents with biliary obstruction
due to mass-like swelling of the pancreas. Frequently AIP is accompanied by
extra-pancreatic manifestations including retroperitoneal fibrosis, thyroid
disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia,
and prompt resolution of pancreatic and extrapancreatic findings with steroids
signify its autoimmune nature. Refractory cases are responsive to
immunomodulators and rituximab. Involvement of the biliary tree, termed IgG4
associated cholangiopathy, mimics primary sclerosing cholangitis and is
challenging to manage. High IgG4 levels and swelling of the pancreas with a
diminutive pancreatic duct are suggestive of autoimmune pancreatitis. Given
similarities in presentation but radical differences in management and outcome,
differentiation from pancreatic malignancy is of paramount importance. There is
controversy regarding the optimal diagnostic criterion and steroid trials to make
the diagnosis. Additionally, the retroperitoneal location of the pancreas and
requirement for histologic sampling, makes tissue acquisition challenging.
Recently, a second type of autoimmune pancreatitis has been recognized with
similar clinical presentation and steroid response though different histology,
serologic, and extrapancreatic findings.
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