Unit:
Κατεύθυνση Μοριακή και Εφαρμοσμένη ΦυσιολογίαLibrary of the School of Health Sciences
Author:
Αλεξανδρή Ολυμπία
Supervisors info:
Σουγιουλτζής Σταύρος Επικ. Καθηγητής
Original Title:
Autoimmune pancreatitis and IgG4
Translated title:
Αυτοάνοση παγκρεατίτιδα και IgG4
Summary:
AIP is a rare, heterogeneous, fibroinflammatory disorder of the pancreas. It
can occur as a primary disorder of the pancreas or as a systemic disease with
elevated IgG4 levels. In this IgG4-related disease other organs, such as bile
duct, gallbladder, gastrointestinal duct, salivary glands, lymph nodes and
others, are involved. There are two distinct subtypes of AIP, based mainly on
histological findings. Type I AIP is the most common, characterized as a
systemic disease with extrapancreatic manifestations and elevated IgG4 plasma
cells. Type II is typically found in the pancreas, with a lack of IgG4 plasma
cells. The diagnosis of AIP is based on clinical, laboratory and imaging
findings. The response to steroids is also a characteristic feature of this
disease, which differentiate it from malignancies such as pancreatic cancer and
cholageiocarcinoma or primary sclerosing cholangitis. The pathogenesis still
remains unknown, although an autoimmune mechanism seems to be the most
predominant causative factor. The purpose of this review is to summarize the
existing literature of AIP, with special emphasis on the progress made in the
pathophysiology of this rare disease the last decade and the association with
IgG4-related disease.
Keywords:
Autoimmune pancreatitis, Lymphoplasmacytic sclerosing pancreatitis, Chronic pancreatitis, IgG4-related disease, IgG4
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