Autoimmune pancreatitis

Abstract

Autoimmune pancreatitis is a rare systemic fibrotic inflammatory disorder that can affect organs such as the bile ducts, salivary glands, and retroperitoneal lymph nodes, in addition to the pancreas. Morphological characteristics of autoimmune pancreatitis include a diffusely enlarged 'sausage-shaped' pancreas and an irregularly narrowed duct of Wirsung. According to the revised Japan Pancreas Society criteria, the diagnosis of autoimmune pancreatitis requires that one or more secondary serologic or histologic criteria are also met: the presence of autoantibodies, elevated levels of gamma-globulins, IgG or IgG(4), a lymphoplasmacytic infiltrate, or pancreatic fibrosis. The presence in any affected organ of a lymphoplasmacytic inflammatory infiltrate containing greater than 10 IgG(4)-positive cells per high-power field is pathognomonic for autoimmune pancreatitis. Precise data on the incidence and prevalence of autoimmune pancreatitis are currently not available because most reports involve either limited patient series or resection specimen cohorts. New diagnostic tools and further studies of the underlying pathophysiology and prognosis of autoimmune pancreatitis are needed for adequate and effective treatment strategies to be developed. The most crucial issue when caring for patients with suspected autoimmune pancreatitis is to differentiate autoimmune pancreatitis from pancreatic carcinoma, because pancreatic carcinoma requires surgery, whereas autoimmune pancreatitis responds well to steroid treatment.

Autoimmune pancreatitis in Japan: overview and perspective

“…The spread of knowledge on AIP in the world promoted the recognition of this disease not only in Asian countries but also in Western countries [18][19][20][21][22]. Recently, an increasing number of case reports have been made from outside of Japan, confirming that this disease exists beyond regions and races.…”

Pulmonary manifestations of immunoglobulin G4-related sclerosing disease

“…In a survey of 65 patients with autoimmune pancreatitis, HAMANO et al [11] reported hilar lymphadenopathy detected by CT scanning and gallium-67 scintigraphy to be the most frequent extrapancreatic lesion (80%). Other studies have identified intrathoracic lymphadenopathy in the majority of patients who undergo 18 F-fluoro-2-deoxy-D-glucose positron emission tomography [42,43].…”

“…Studies published over the past few years have documented the association of a high serum IgG4 level with the characteristic lesion of dense lymphoplasmacytic infiltrates containing IgG4-positive plasma cells in various organs, including the bile duct (sclerosing cholangitis) [8][9][10][11], salivary gland (sclerosing sialadenitis) [2,[9][10][11], lacrimal gland (sclerosing dacryoadenitis) [2,10,11], liver (IgG4-hepatopathy) [2], kidney (inflammatory pseudotumour) [2,10], retroperitoneum (retroperitoneal fibrosis) [2,[9][10][11], aorta (inflammatory aneurysm) [2,12], and lymph nodes [2,9,13], as well as the lung [2,10,[14][15][16][17]. ISD can be localised to one or two organs or be present with diffuse systemic disease [1][2][3][18][19][20]. Furthermore, lesions in different organs can present simultaneously or metachronously.…”

Comprehensive Analysis of Serum microRNAs in Autoimmune Pancreatitis

“…The former one is suspected to be the pancreatic lesions of IgG4-related diseases [4]. In type 1 AIP, most of the infiltrating cells in the pancreatic parenchyma are positive for IgG4 and the serum IgG4 levels are frequently elevated [5,6]. However, the role of IgG4 in the pathogenesis of AIP remains elusive.…”