Type 1 autoimmune pancreatitis aip is a distinctive type of pancreatitis characterized by the diffuse irregular narrowing of the main pancreatic duct, sausagelike. It was presumed that repeated acute pancreatitis and chronic pancreatitis, especially with a family history of pancreatic disease, may have a. Autoimmune pancreatitis aip is a rare, heterogeneous, fibroinflammatory disorder of the pancreas. Acute pancreatitis can be a lifethreatening illness with severe complications. Eus in the diagnosis of autoimmune pancreatitis pancreapedia. Although the pathogenesis of aip remains unclear, an immunemediated mechanism has been postulated.
Autoimmune pancreatitis is a rare type of chronic pancreatitis characterized by fibrosis with abundant infiltration of igg4positive plasma cells and lymphocytes and. However, in a considerable number of individuals, even many attacks of gallstones and multiple episodes of alcohol abuse do not lead to acute pancreatitis 2, 3. Each year, about 210,000 people in the united states are admitted to the hospital with acute pancreatitis. Review of the diagnosis, classification and management of. It can be initiated by several factors, including gallstones, alcohol, trauma, infections and hereditary factors. Autoimmune pancreatitis aip is an immunemediated tumefactive fibroinflammatory disorder that primarily affects the pancreas. Autoimmune pancreatitis aip is a distinct type of chronic pancreatitis that reportedly occurs in 7% of chronic pancreatitis cases and typically affects patients without a history of alcohol abuse, biliary stone disease, or duodenal wall inflammation 1 5. Pancreatitis autoinmune gastroenterologia y hepatologia.
Acute pancreatitis is an inflammatory disease of the pancreas. The most frequent causes of acute pancreatitis ap are gallstones and alcohol. Pdf r e v i s i o n e s medicina intensiva pancreatitis. To retrospectively determine imaging findings in patients with autoimmune pancreatitis. Type 1 aip is now regarded as a manifestation of igg4related disease, and those. Igg4related sclerosing disease chari 2010 two distinct. About 75% of pancreatitis is caused by gallstones or alcohol.
Autoimmune pancreatitis aip is a unique form of chronic pancreatitis that is characterized by a dramatic response to steroid therapy. Type 1 and type 2, each with distinct clinical profiles. Mayor incidencia en japn asociada a otros trastornos inmunolgicos. Request pdf pancreatitis autoinmune autoimmune pancreatitis is a benign fibroinflammatory disease of the pancreas of probable autoimmune origin, which includes 2 different. Autoimmune pancreatitis aip 1 is a benign fibroinflammatory disease that frequently presents as obstructive jaundice, which may or may not be associated with a pancreatic mass. Diagnosis of autoimmune pancreatitis by eusfna by using a 22gauge needle based on the international consensus diagnostic criteria.
The estimated number of aip patients was 900 in the first japanese nationwide survey conducted in 2002, and it was 2790 in 2007, which was 3. Pdf igg4related disease igg4rd is a fibroinflammatory disorder recognized as a. Autoimmune pancreatitis is a special form of chronic pancreatitis. It has gained increasing recognition due to a presentation that can mimic difficulttotreat disorders such as pancreatic cancer, cholangiocarcinoma and primary sclerosing cholangitis. The etiology and pathogenesis of acute pancreatitis have been intensively investigated for centuries worldwide. This poster was originally presented at the seram 2012 meeting, may 2428, in granadaes. Objective autoimmune pancreatitis aip is a treatable form of chronic pancreatitis that has been. Autoimmune pancreatitis aip is an unusual etiology of chronic pancreatitis, involved in approximately 6% of. Naitoh i, nakazawa t, hayashi k, okumura f, miyabe k, shimizu s, kondo h, yoshida m, yamashita h, ohara h, and joh t.
Type 1 autoimmune pancreatitis aip is the pancreatic manifestation of a multiorgan disease, named immunoglobulin g4 igg4related disease while type 2 aip is a pancreas specific disorder not associated with igg4. Forsmark division of gastroenterology, hepatology, and nutrition, university of florida, gainesville, florida advances in our understanding of chronic pancreatitis have improved our care of patients with this. History of autoimmune pancreatitis sarle 1961 idiopathic chronic pancreatitis with elevated. Extracellular vesicles microrna analysis in type 1 autoimmune.
Pdf autoimmune pancreatitis aip is a benign, igg4related, fibroinflammatory. R e v i s i o n e s medicina intensiva pancreatitis aguda. Strategy to differentiate autoimmune pancreatitis from pancreas cancer. Management of chronic pancreatitis gastroenterology. Pancreatitis 1 especialidades medicas medicina clinica. Chronic pancreatitis medical expert as medical experts, physicians integrate all of the canmeds roles, applying medical knowledge, clinical skills, and professional attitudes in their provision of patientcentered care. Pancreatitis is an uncommon disease characterized by inflammation of the pancreas. This type is more common in younger people of western european and american ethnicity, does not show a sex predilection, and rarely recurs. Acute pancreatitis affects about 50,000 80,000 americans each year.
It shows characteristic histological changes, and there is excellent response to corticosteroid therapy, as published in the 2011 international consensus on aip. Clinical differences between massforming autoimmune pancreatitis and pancreatic cancer. Autoimmune pancreatitis is an increasingly recognized type of chronic pancreatitis that can be difficult to distinguish from pancreatic carcinoma but which responds to treatment with corticosteroids, particularly prednisone. Objective autoimmune pancreatitis aip is a treatable form of chronic pancreatitis that has been increasingly recognised over the last decade. The remission rate for induction treatment with steroids is essentially 100%, and steroids remain highly effective when used for treating disease relapses. We set out to better understand the current burden of aip at several academic institutions diagnosed using the international consensus diagnostic criteria, and to describe longterm outcomes, including organs involved, treatments, relapse frequency and. Immunomodulators and rituximab in the management of.
Download fulltext pdf download fulltext pdf autoantibodies in autoimmune pancreatitis article pdf available in international journal of rheumatology 2012 5. Localized autoimmune pancreatitis mimicking pancreatic cancer. Recent advances in the diagnosis and management of. With increased recognition of the concept of autoimmune pancreatitis aip and with the development of diagnostic criteria, the number of aip patients in japan has increased rapidly. Pdf current perspectives on autoimmune pancreatitis and igg4. It is a condition that arises suddenly and may be quite severe, although patients usually have a. Making the correct diagnosis and differentiating the disease from pancreatic cancer is of the utmost importance. Epidemiology of autoimmune pancreatitis the pancreas. The role of endoscopy in the diagnosis of autoimmune pancreatitis. It is associated with ibd but has no known serologic biomarker. G yoshida 1995 propose concept of autoimmune pancreatitishamano 1995 increased serum levels of igg4 in aip jps 2002 japan pancreas society.
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