Materiały źródłowe

• #1 Autoimmune Pancreatitis: Symptoms, Risk Factors and Treatment

  https://my.clevelandclinic.org/health/diseases/17936-autoimmune-pancreatitis

  Autoimmune pancreatitis is an uncommon disorder. It causes your immune system to attack healthy cells in your pancreas. People with other autoimmune conditions face a higher risk of getting it. […] Researchers are still determining the precise cause of autoimmune pancreatitis. The condition often develops in people between the ages of 50 and 60, but it can occur sooner. It affects men twice as often as women. […] In some cases, autoimmune pancreatitis may develop due to your body’s immune response after a bacterial infection. In addition, people with other autoimmune disorders face a higher risk, such as: Hashimoto’s thyroiditis (which causes low thyroid levels or hypothyroidism), inflammatory bowel disease (especially ulcerative colitis), primary biliary cholangitis, retroperitoneal fibrosis (a condition where scar-like tissue forms in the lining at the back of your abdomen), rheumatoid arthritis, sarcoidosis, Sjögren’s syndrome.

• #2 Mayo Clinic Health Library – Autoimmune pancreatitis | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20369783

  Autoimmune pancreatitis is an inflammation in the pancreas. It may be caused by the immune system attacking the pancreas. […] Experts don’t know what causes autoimmune pancreatitis, but it is thought to be caused by the body’s immune system attacking healthy body tissue. This is known as an autoimmune disease.

• #3 Autoimmune Pancreatitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560769/

  Autoimmune pancreatitis is a clinical condition characterized by inflammation of the pancreas from an autoimmune etiology. […] The etiology of autoimmune pancreatitis is considered to be autoimmune, as demonstrated by the presence of lymphocytic infiltration on histology. […] As an IgG4 related disease, various immune-mediated mechanisms have been suggested that could initiate the inflammatory response. […] Some potential initiating mechanisms include bacterial infection and molecular mimicry in the setting of genetic risk factors and autoimmunity. […] T-regulatory cells mediated immune reaction and the shift of peripheral blood T lymphocytes towards T helper 2 response could also contribute to the recruitment of cytokines and interleukins that subsequently induce inflammation and fibrosis. […] The IgG4 antibodies are thought to behave as tissue-destructive immunoglobulins both locally and systemically, but there remains a gap in the understanding of the etiology of many IgG4-related diseases.

• #4 Autoimmune pancreatitis » Global Autoimmune Institute

  https://www.autoimmuneinstitute.org/autoimmune-resources/autoimmune-diseases-list/autoimmune-pancreatitis/

  Also known as immunoglobulin G4-related disease, AIP is caused by an immune system attack on the pancreas which causes chronic inflammation, enlargement, or a mass in the pancreas. […] The subtypes of AIP are known as type 1 and type 2, and affect different parts of the body. […] AIP Type 1: […] AIP Type 2: […] The frequency of both types varies according to geographical location.

• #5 Autoimmune pancreatitis – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/autoimmune-pancreatitis/

  Autoimmune pancreatitis (AIP) is a chronic inflammation that is thought to be caused by the body’s immune system attacking the pancreas and that responds to steroid therapy. […] Doctors don’t know what causes autoimmune pancreatitis, but as in other autoimmune diseases, it is thought to be caused by the body’s immune system attacking healthy body tissue.

• #6 Autoimmune pancreatitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/autoimmune-pancreatitis?lang=us

  Autoimmune pancreatitis is a rare, benign pancreatic disease associated with autoimmune manifestations on clinical, histological, and laboratory grounds 1. Three types (AIP-1, AIP-2, AIP-3) have been described, differing in presentation, pathogenesis, and natural history 23. […] Three types of autoimmune pancreatitis have been described 22,23: […] type 1 autoimmune pancreatitis (AIP-1) […] IgG4-related disease […] more common in Asian countries […] type 2 autoimmune pancreatitis (AIP-2) […] also known as idiopathic ductal centric pancreatitis (IDCP) […] more common in Western countries […] type 3 autoimmune pancreatitis (AIP-3) […] caused by immune checkpoint inhibitor therapy.

• #7 Autoimmune Pancreatitis – Pancreatic Diseases – Gastroenterology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.5.5.

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis due to an autoimmune etiology. […] Three types of AIP are distinguished: 1) Type 1 is a pancreatic manifestation of a systemic autoimmune disease called IgG4-related disease. […] 2) Type 2 is characterized by neutrophilic infiltration in the epithelium of the pancreatic duct. […] 3) Type 3 is a recently recognized form of AIP caused by pancreatic injury due to the use of immune checkpoint inhibitors for a broad spectrum of malignancies. […] An increased concentration of IgG4 has the greatest diagnostic significance as a single parameter; a value of 2-fold the upper limit of normal may indicate type 1 AIP. However, IgG4 is not specific to AIP. […] Type 1 AIP typically shows lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. Type 2 AIP shows idiopathic duct-centric pancreatitis with granulocytic epithelial lesions. Type 3 AIP shows CD8+ T-cell infiltration.

• #8 Autoimmune Pancreatitis – Practical Gastro

  https://practicalgastro.com/2015/08/08/autoimmune-pancreatitis/

  Autoimmune pancreatitis represents a small percentage of all forms of recurrent and chronic pancreatitis. It occurs due to a primary pancreatic autoimmune process or as a secondary component of a systemic autoimmune disorder. This condition accounts for approximately six percent of chronic pancreatitis cases in the United States. […] There are two subtypes of autoimmune pancreatitis. Type 1 is typically found in elderly men and is associated with systemic disease affecting extra-pancreatic organs such as the bile duct, kidneys, lymph nodes, and salivary glands. Elevated serum immunoglobulin four (IgG4) is a defining feature. Type II autoimmune pancreatitis is typically seen one decade before type I. It is not predominant to one gender, does not involve other organs with the exception of inflammatory bowel disease and is not linked with increased IgG4 levels. Both subtypes demonstrate lymphoplasmacytic infiltrate upon histologic examination.

• #8 Autoimmune Pancreatitis – Practical Gastro

  https://practicalgastro.com/2015/08/08/autoimmune-pancreatitis/

  The diagnosis of autoimmune pancreatitis requires a high index of suspicion and a multidisciplinary approach involving serologic tests, radiologic imaging, endoscopic imaging with tissue sampling, and sometimes surgical biopsies. The Mayo Clinic proposed the HISORt criteria to help better define and confirm the presence of this disease. […] Increased serum IgG4 is the best serologic diagnostic marker for autoimmune pancreatitis; however, one study reports that only 44% of patients initially presenting with this disease had elevated levels. Serum IgG4 can also be elevated in pancreatic cancer. Guidelines therefore endorse a level above two times the upper limit of normal as being highly suggestive of autoimmune pancreatitis. […] Core biopsy is considered the best mode of obtaining specimens to assess for autoimmune pancreatitis while fine needle aspiration is preferred for evaluating for pancreatic carcinoma. In the absence of malignant histology, biopsy samples demonstrating lymphocytic and plasma cell infiltrates along with fibrosis support the diagnosis of autoimmune pancreatitis especially when plasma cells are positive for abundant IgG4.

• #9 What Autoimmune Disease Causes Pancreatitis | Maggie Yu MD, IFMCP

  https://drmaggieyu.com/blog/what-autoimmune-disease-causes-pancreatitis/

  Autoimmune pancreatitis (AIP) is a unique form of pancreatic disease that can be divided into two main types: Type 1 and Type 2, with steroids being a common treatment. Each type affects the pancreas differently. […] Type 1 AIP, also known as IgG4-related pancreatitis, involves the body’s immune system attacking its own pancreatic tissue, affecting people. This attack leads to chronic inflammation and eventually fibrosis in the pancreas. Patients often have elevated levels of IgG4 antibodies in their blood. […] Type 2 AIP is less common and not associated with increased IgG4 levels. Instead, it features granulocytic epithelial lesions which indicate an immune response directly targeting the pancreatic ducts. Unlike Type 1, Type 2 often occurs without affecting other organs. […] The hallmark of autoimmune pancreatitis is chronic inflammation leading to fibrosis within the pancreas. Over time, this process can cause severe damage to pancreatic tissue.

• #9 What Autoimmune Disease Causes Pancreatitis | Maggie Yu MD, IFMCP

  https://drmaggieyu.com/blog/what-autoimmune-disease-causes-pancreatitis/

  Genetic predisposition plays a crucial role in autoimmune diseases. Research shows that certain genes can make individuals more susceptible to these conditions. This is especially true for autoimmune pancreatitis. […] Environmental factors also contribute significantly. They can activate the immune system against the pancreas. […] Some known triggers include viral infections, certain medications, smoking, and heavy alcohol use. […] The immune system uses T-cells to fight off invaders like viruses and bacteria. In autoimmune pancreatitis, however, these cells mistakenly attack pancreatic tissues. […] Another key factor involves IgG4 antibodies specific to this condition. These antibodies are produced by plasma cells in response to perceived threats. […] In normal situations, IgG4 helps protect against disease. But in autoimmune pancreatitis, they target organs such as the pancreas and salivary glands.

• #9 What Autoimmune Disease Causes Pancreatitis | Maggie Yu MD, IFMCP

  https://drmaggieyu.com/blog/what-autoimmune-disease-causes-pancreatitis/

  Middle-aged males often face a higher risk of developing Type 1 autoimmune pancreatitis. This fact is crucial in understanding the disease’s etiology. […] Studies show that men over forty are more susceptible to this condition than their younger or female counterparts. […] Autoimmune pancreatitis incidence rates vary across different regions of the world. Some areas report higher numbers of cases than others. […] A significant risk factor for autoimmune pancreatitis is family history. If someone in your family has had the disease, your chances of developing it increase.

• #10 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Autoimmune pancreatitis (AIP) is a rare disease. The diagnosis of AIP is difficult and should be made by a comprehensive evaluation of clinical, radiological, serological, and pathological findings. Two different types of AIP have been identified: autoimmune pancreatitis type 1 (AIP-1), which is considered a pancreatic manifestation of multiorgan disease related to IgG4, and autoimmune pancreatitis type 2 (AIP-2), which is considered a pancreas-specific disease not related to IgG4. […] AIP-1 is the pancreatic manifestation of IgG4-related disease (IgG4-RD) characterized by lymphoplasmacytic infiltration with more than ten IgG4-positive plasma cells per high-power field (HPF), storiform fibrosis, and obliterative phlebitis. […] In contrast, AIP-2 is not a systemic disease, and the pancreas is the only organ affected. The fibro-inflammatory process mainly involves the pancreatic ducts with neutrophilic infiltration of the medium and small ducts.

• #10 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  The pathogenesis of AIP-1 is not yet fully understood, and many details are still unclear. The innate and adaptive immune systems are involved in the development of IgG4-RD. […] The role of IgG4 in the development of AIP-1 and IgG4-RD is still unclear. […] AIP-2 is characterized by neutrophil-rich inflammation. In particular, granulocytic epithelial lesions (GELs), consisting of neutrophilic ductal infiltration are the major histologic finding of the disease. A possible role of Th-17 in the development of the disease was suggested. […] The cornerstone of AIP treatment is steroid therapy. High doses of methylprednisolone lead to remission in most cases of AIP. However, relapses are quite common, especially in subtype 1; for this reason, the duration of steroid treatment is still debated and alternative therapies are sought to avoid the side effects of steroids.

• #11 Type 2 Autoimmune Pancreatitis (Idiopathic Duct-Centric Pancreatitis) Highlighting Patients Presenting as Clinical Acute Pancreatitis: A Single-Center Experience

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl18429

  Type 2 autoimmune pancreatitis (AIP) has been considered extremely rare in East Asia. This study aimed to clarify the prevalence, clinical characteristics and radiological findings of type 2 AIP highlighting patients presenting as acute pancreatitis in a single center. […] Among 244 patients with AIP, 27 (11.1%) had type 2 AIP (definite, 15 [55.5%] and probable 12 [44.5%]). The median age of patients with type 2 AIP was 29 years (interquartile range, 20 to 39 years). Acute pancreatitis was the most common initial presentation (n=17, 63%) while obstructive jaundice was present in only one patient. Ulcerative colitis (UC) was associated with type 2 AIP in 44.4% (12/27) of patients. […] In South Korea, type 2 AIP is not as rare as previously thought. Overall, the clinical profile of type 2 AIP was similar to that of Western countries. Type 2 AIP should be considered in young UC patients with acute pancreatitis of uncertain etiology.

• #12 American College of Gastroenterology Annual Meeting

  https://www.healio.com/news/gastroenterology/20221026/rare-type-3-autoimmune-pancreatitis-results-in-rapid-pancreatic-atrophy

  Type 3 autoimmune pancreatitis led to rapid pancreatic atrophy and functional endocrine failure despite steroid therapy or immune checkpoint inhibitor withdrawal, a researcher reported at ACG Annual Scientific Meeting. […] A third form, type 3, is rare with an estimated incidence of about 0.6% to 4% and is characterized by pancreatic injury induced by checkpoint inhibitors, she said. […] Type 3 AIP is a novel, drug-induced chronic inflammatory disease of the pancreas that is predominantly asymptomatic and mild in severity but causes a rapid pancreatic atrophy, Thomas concluded. It is more pronounced in those receiving combination immunotherapy and occurs despite steroid therapy or holding immunotherapy.

• #13 Autoimmune pancreatitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/autoimmune-pancreatitis?embed_domain=hackmd.io%25252f%252540yipuafecsl2jsu8smr5njq%25252fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.pngfavicon.icofavicon.ico&lang=gb

  Autoimmune pancreatitis is a rare, benign pancreatic disease associated with autoimmune manifestations on clinical, histological, and laboratory grounds 1. Three types (AIP-1, AIP-2, AIP-3) have been described, differing in presentation, pathogenesis, and natural history 23. […] Three types of autoimmune pancreatitis have been described 22,23: […] type 1 autoimmune pancreatitis (AIP-1) […] IgG4-related disease […] more common in Asian countries […] type 2 autoimmune pancreatitis (AIP-2) […] more common in Western countries […] type 3 autoimmune pancreatitis (AIP-3) […] caused by immune checkpoint inhibitor therapy.

• #14 Pathophysiology of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4024516/

  Autoimmune pancreatitis (AIP) is a recently discovered form of pancreatitis and represents one of the diseases of the pancreas which can be cured and healed medically. […] The categorization of AIP as an autoimmune disorder is based on the observation that the disease is associated with the infiltration of immune cells into pancreatic tissue, and that the disease dramatically responds to steroid therapy. […] The pancreas of a patient with AIP is often infiltrated by various types of immune cells, including cluster of differentiation (CD) 4-positive T cells and granulocytes in type 2 AIP or IgG4-producing plasma cells and B-lymphocyte antigen CD20 in type 1 AIP. […] It has also been reported that DRB1*0405 and DQB1*0401 mutations are significantly more frequent in patients with AIP when compared to those with chronic calcifying pancreatitis.

• #14 Pathophysiology of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4024516/

  Furthermore, Park et al found that only DQB1*0302 had a significant association with the relapse of AIP. […] Finally, it has been found that the polymorphic genes (CTLA-4 49A polymorphism and -318C/+ 49A/CT60G haplotype) encoding cytotoxic T lymphocyte-associated antigen 4, a key negative regulator of the T-cell immune response, are associated with AIP in a Chinese population. […] Even if these data are not concordant, it is possible that physiological IgG4 responses are induced by prolonged antigen exposure and controlled by type 2 helper T cells.

• #15 Autoimmune Pancreatitis: Etiology, Pathogenesis, Clinical Finding

  https://www.primescholars.com/articles/autoimmune-pancreatitis-etiology-pathogenesis-clinical-findings-and-treatment-the-japanese-experience-98724.html

  The occasional coexistence of pancreatitis with other lesions suggests that there may be common target antigens in the pancreas and other exocrine organs such as the salivary gland, biliary tract and renal tubules. […] We identified several autoantibodies such as antinuclear antibody (ANA), anti-lactoferrin (LF) antibody (ALF), and anti-carbonic anhydrase-II (CA-II) antibody (ACA-II) by using the patients serum and expression vector encoding human pancreatic cDNA. […] Although the majority of patients with AIP show increased levels of IgG, especially the IgG4 subtype, the roles of IgG4 in AIP are still unclear. […] The activated CD4+ and CD8+ T-cells bearing HLA-DR and CD45RO were elevated in the peripheral blood lymphocytes (PBLs) as well as in the pancreas in AIP as compared to other causes of pancreatitis such as alcoholic or gallstone-related pancreatitis.

• #16 Type 1 autoimmune pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-6-82

  A Th2-dominant immune reaction and the activation of regulatory T-cells are assumed to be involved in the underlying immune reaction. […] However, the exact role of IgG4 in this disease still remains to be clarified. […] The entity „autoimmune pancreatitis (AIP)” was first proposed by Yoshida et al in 1995, who described a case of steroid-responsive pancreatitis. […] The presence of pancreatitis, features of autoimmune disease, and responsiveness to immunosuppression led to the connotation of AIP. […] This form of pancreatitis was recognized as lymphoplasmacytic sclerosing pancreatitis or non-alcoholic duct destructive chronic pancreatitis based on distinct histological features in the 1990s. […] Another landmark paper was published in the New England Journal of Medicine in 2001, where Hamano et al reported that serum IgG4 levels are specifically elevated in Japanese patients with AIP.

• #16 Type 1 autoimmune pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-6-82

  Few reports regarded IgG4-type autoantibodies as pathogenic in type 1 AIP. […] A T-helper (Th) 1 immune response has been considered to be predominant in type 1 AIP. […] However, recent studies provided data in support of a Th2-predominant immune response in IgG4-related disease. […] Immune complex deposition is identifiable in the basement membrane of pancreatic ducts and acini. […] These complexes consist of predominantly IgG4 and complement C3, with minor components of other IgG subclasses. […] Given that IgG4 cannot form large immune complexes because of Fab-arm exchange, it remains to be clarified what is the role of IgG4 in immune complex formation, and how these depositions are involved in the pathogenesis of type 1 AIP.

• #16 Type 1 autoimmune pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-6-82

  Before the concept of autoimmune pancreatitis (AIP) was established, this form of pancreatitis had been recognized as lymphoplasmacytic sclerosing pancreatitis or non-alcoholic duct destructive chronic pancreatitis based on unique histological features. […] With the discovery in 2001 that serum IgG4 concentrations are specifically elevated in AIP patients, this emerging entity has been more widely accepted. […] Type 1 AIP is estimated as a pancreatic manifestation of systemic IgG4-related disease based on the fact that synchronous or metachronous lesions can develop in multiple organs (e.g. bile duct, salivary/lacrimal glands, retroperitoneum, artery, lung, and kidney) and those lesions are histologically identical irrespective of the organ of origin. […] Several potential autoantigens have been identified so far.

• #17 Pathogenesis of autoimmune and hereditary pancreatitis with a focus on neutrophil granulocytes and neutrophil serine proteases

  https://www.oaepublish.com/articles/rdodj.2022.17

  The best-characterized variants are localized in the cationic trypsinogen gene (PRSS1) and implicate different biochemical reactions: while some mutations are linked to an increase in autocatalytic activation and enhanced stability of active trypsin, others are connected to increased transactivation of anionic trypsinogen, the second major trypsinogen isoform, or even protein misfolding, which results in chronic pancreatitis mediated by increased endoplasmic reticulum stress. […] Autoimmune pancreatitis (AIP) is another rare type of pancreatitis that exhibits features of both acute and chronic pancreatitis. […] AIP is classified into two subtypes: type 1, also known as lymphoplasmacytic sclerosing pancreatitis (LPSP); and type 2, idiopathic duct-centric chronic pancreatitis (IDCP). […] Each type of AIP has its own characteristics. Hallmarks of type 1 AIP are infiltration by IgG4-positive plasma cells, increased IgG4 titer in the serum, and histological abnormalities such as storiform fibrosis and lymphoplasmacytic infiltration of the pancreatic ducts.

• #17 Pathogenesis of autoimmune and hereditary pancreatitis with a focus on neutrophil granulocytes and neutrophil serine proteases

  https://www.oaepublish.com/articles/rdodj.2022.17

  In comparison, type 2 AIP is characterized by the infiltration of neutrophils into the pancreatic duct and the surrounding tissue, especially the interlobular duct, with local destruction of the ductal epithelium at what are called granulocytic epithelial lesions (GELs). […] Both types of AIP are associated with different antibodies targeting the plasminogen-binding protein of Helicobacter pylori, the ubiquitin-protein ligase E3 component n-recognin, SPINK1, PRSS1, and PRSS2. […] The knowledge of the role of neutrophils in general and NSPs in particular in the pathogenesis and course of hereditary and autoimmune pancreatitis remains very limited. […] To the best of our knowledge, no currently available literature has specifically focused on the role of NSPs in hereditary pancreatitis, although the organ-injuring effects of neutrophils and elastase in acute pancreatitis are clear in general. […] Further knowledge of the role of neutrophils and NSPs in these two rare forms of pancreatitis remains missing.

• #18 Autoimmune Pancreatitis: Case Series and Review of the Literature.

  http://www.annclinlabsci.org/content/39/2/167.full?ck=nck

  Autoimmune pancreatitis (AuP) is a chronic pancreatic inflammation secondary to an underlying autoimmune mechanism. […] In 1995 the term autoimmune pancreatitis was used by Yoshida et al, stating that it was a distinct disease. […] The pathogenesis of AuP remains a mystery. The occasional coexistence of AuP with other auto-immune diseases suggests that there may be common target antigens in the pancreas and the other exocrine organs, such as the salivary glands, biliary tract, and renal tubules. […] A number of autoantibodies such as rheumatoid factor (RF), p-ANCA, ANA, ASMA, AMA, antithyroglobulin, and anti-microsomal antibodies have been reported. […] Elevated IgG4 levels in the presence of radiologic findings may warrant initiation of steroid therapy.

• #19 Autoimmune pancreatitis – Wikipedia

  https://en.wikipedia.org/wiki/Autoimmune_pancreatitis

  Autoimmune Pancreatitis (AIP) 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. […] Type 1 AIP is now regarded as a manifestation of IgG4-related disease, and those affected have tended to be older and to have a high relapse rate. […] Although the exact mechanism explaining the clinical manifestations of autoimmune pancreatitis remain for an important part obscure, most professionals would agree that the development of IgG4 antibodies, recognizing an epitiope on the membrane of pancreatic ancinar cells is an important factor in the pathophysiology of the disease. These antibodies are postulated to provoke an immune response against these ancinar cells resulting in pancreatic inflammation and destruction. […] Hence improved diagnosis, understanding and treatment of autoimmune pancreatitis awaits the identification of the auto-antigens involved.

• #20 Autoimmune Pancreatitis: Differential Diagnosis With Pancreatic Adenocarcinoma | Cirugía Española (English Edition)

  https://www.elsevier.es/en-revista-cirugia-espanola-english-edition–436-articulo-autoimmune-pancreatitis-differential-diagnosis-with-S2173507717301667

  Autoimmune pancreatitis (AIP) is an unusual etiology of chronic pancreatitis, involved in approximately 6% of all cases. […] AIP is divided into 2 clinical-pathological forms: type I, or lymphoplasmocytic sclerosing pancreatitis (LPSP); and type II, or idiopathic duct-centric pancreatitis (IDCP). […] AIP is a disease that responds to treatment with corticosteroids, and accurate diagnosis is crucial to avoid unnecessary major surgeries. […] Serum elevation of IgG4 (immunoglobulin G4) above normal levels (140mg/dL) is another of the basic diagnostic criteria. […] Treatment with corticosteroids should only be initiated after adenocarcinoma has been ruled out.

• #21 Chronic pancreatitis – Guts UK

  https://gutscharity.org.uk/advice-and-information/conditions/chronic-pancreatitis/

  Chronic pancreatitis is a long-standing inflammation of the pancreas. […] The causes of chronic pancreatitis vary greatly. In most people with chronic pancreatitis there is probably a combination of family (genetic) and environmental factors. […] Autoimmune pancreatitis occurs when the body’s own immune system attacks the pancreas. This disorder may be linked to other autoimmune diseases, such as primary biliary cirrhosis and inflammatory bowel disease. But it is also likely to have a genetic background.

• #22 Autoimmune Pancreatitis: Symptoms, Types, Treatment, More

  https://www.healthline.com/health/autoimmune-pancreatitis

  Autoimmune pancreatitis happens when your immune system mistakenly attacks your pancreas. […] The exact cause of autoimmune pancreatitis is unknown. Its possible that its caused by an initiating event, such as a bacterial infection, in people who are genetically predisposed to autoimmune pancreatitis or other autoimmune conditions. […] Autoimmune pancreatitis can relapse following treatment. Relapses are more common in type 1 disease, occurring in up to 50 percent of people with this type of autoimmune pancreatitis. Having bile duct involvement increases the risk of relapse. […] If its not treated, autoimmune pancreatitis can cause complications.

• #23 Autoimmune Pancreatitis | Digestive Health | Loyola Medicine

  https://www.loyolamedicine.org/services/digestive-health-program/digestive-health-conditions/autoimmune-pancreatitis

  Autoimmune pancreatitis is a rare disease that is characterized by chronic or long-term inflammation of the pancreas. […] There are two types of autoimmune pancreatitis (type 1 and type 2). Type 1 is the most common form. Type 2 is distinct from type 1 in that type 2 is primarily confined to the pancreas, while type 1 can promote symptoms or disease inside and outside of the pancreas. Moreover, type 1 autoimmune pancreatitis is an IgG4-related disease with unique immune system causes, while type 2 autoimmune pancreatitis is not. […] Individuals who are prone to inflammation or inflammatory diseases, such as lupus, are at risk of developing autoimmune pancreatitis. Moreover, type 1 autoimmune pancreatitis is more common in men over the age of 60. […] Other risk factors include: Alcohol abuse, Genetics or family history of autoimmune pancreatitis, H. pylori infection in individuals with a family history of autoimmune pancreatitis.

• #24 Treatment approaches in autoimmune pancreatitis (Review)

  https://www.spandidos-publications.com/10.3892/br.2023.1714

  On the other hand, in the case of AIP-2, risk factors for recurrence include initial use of steroids and tobacco, and the presence of chronic pancreatitis. […] The epidemiology, histological pattern, pathogenesis and natural history of AIP-1 and -2 are distinct from one another. […] The cornerstone in the development of AIP is the aberrant immune response. […] The development of AIP-1 involves both innate and adaptive immunity. The involvement of IgG4 in disease progression is still unknown, and it is unclear if specific triggers, including changes in the gut flora, can hasten the development of AIP-1. The pathophysiology of AIP-2 is less understood.

• #25 Autoimmune Pancreatitis – MD Searchlight

  https://mdsearchlight.com/health/autoimmune-pancreatitis/

  Autoimmune pancreatitis is believed to be caused by an autoimmune reaction that is, the body’s immune system attacking its own cells. This can be seen through the presence of immune cells called lymphocytes in the pancreas. Autoimmune pancreatitis belongs to a group of diseases related to a particular type of antibody known as IgG4. […] Various theories have been proposed on how the inflammation starts in autoimmune pancreatitis. Potential triggers could include bacterial infection and a phenomenon known as molecular mimicry, which occurs when the immune system mistakes its own cells for invaders due to their similar appearance. This usually happens in individuals who already have genetic risk factors and an autoimmune tendency. […] Other potential contributors could be certain immune cells (T-regulatory cells) and a shift in the body’s immune response towards an increased activity of a type of immune cell called T helper 2. These changes can lead to an increased release of immune system proteins like cytokines and interleukins, which may cause inflammation and scarring (fibrosis) of tissues. […] The IgG4 antibodies are thought to cause damage to tissues, both in the pancreas and throughout the body. However, our understanding of why and how IgG4-related diseases develop is still incomplete.

• #26 Causes of Pancreatitis | Columbia Surgery

  https://columbiasurgery.org/pancreas/causes-pancreatitis

  In approximately 5-6% of patients with chronic pancreatitis, the disease is caused by autoimmune inflammation (in which the immune system attacks the pancreas). […] Other indicators of autoimmune pancreatitis include narrowing of the main pancreatic duct, scarring of the pancreatic tissue, and infiltration with inflammatory cells. […] AIP can occur by itself or in association with other autoimmune diseases such as primary sclerosing cholangitis (PSC), primary biliary cirrhosis, retroperitoneal fibrosis, rheumatoid arthritis, sarcoidosis, and Sjgren’s syndrome.

• #27

  https://journals.lww.com/joms/fulltext/2020/40010/increased_risk_of_acute_pancreatitis_in_patients.2.aspx

  Sjgren’s syndrome (SS) is a chronic autoimmune disease with lymphocytic exocrine gland infiltration causing dry mouth and eyes. […] PSS has been suggested to increase the acute pancreatitis risk. […] The SS cohort had a higher acute pancreatitis risk than the non-SS cohort after covariate adjustments (adjusted hazard ratio [HR], 3.374; 95% confidence interval [CI], 2.8693.969). […] Patients with PSS exhibited a 2.872-fold risk (95% CI, 2.6113.901) and patients with SSS a 4.121-fold risk (95% CI, 3.7525.124) for acute pancreatitis. […] Patients with SS presented increased acute pancreatitis risks than patients without SS, and patients with SSS had higher acute pancreatitis risks than patients with PSS. […] The pathogenesis of SS includes a genetic predisposition, innate and adaptive immunity abnormalities, and hormonal and environmental factors.

• #27

  https://journals.lww.com/joms/fulltext/2020/40010/increased_risk_of_acute_pancreatitis_in_patients.2.aspx

  SS is an autoimmune disease involving exocrine glands. Chronic pancreatitis has been found in association with other autoimmune diseases such as SS, primary biliary cirrhosis, and sclerosing cholangitis. […] Our study showed a 2.872-fold higher acute pancreatitis risk in patients with PSS and a 4.121-fold higher risk in patients with SSS than the risk in the non-SS cohort. […] The exact mechanism of PSS-induced acute pancreatitis remains unknown. […] Autoimmune pancreatitis (AIP) was first described by Yoshida et al. in 1995, and the term was adopted for pancreatic disease of an autoimmune origin. […] AIP is classified into two subtypes. Type 1 AIP is a systemic disease affecting the pancreas, salivary glands, and kidneys, while type 2 AIP affects only the pancreas. […] Some studies have shown raised IgG4 serum levels and IgG4-positive plasma cell infiltration in labial salivary glands of patients with SS.

• #28 Imaging autoimmune pancreatitis

  https://www.openaccessjournals.com/articles/imaging-autoimmune-pancreatitis-8204.html

  Autoimmune pancreatitis (AIP) is characterized by diffuse or segmental enlargement of the pancreas with irregular narrowing of the main pancreatic duct, and fibrotic change with lymphocyte infiltration histopathologically. […] According to the results, we classified AIP into three types: diffuse, segmental and multifocal. […] AIP can be classified into three types: diffuse, segmental and multifocal. Differentiation between AIP and other pancreatic disorders on CT and MRI may be easier, considering those types of AIP. […] In patients with AIP, serum IgG4 levels are frequently and significantly elevated, and various extrapancreatic lesions are present. Dense infiltration of IgG4-positive plasma cells as well as fibrosis have been found in the peripancreatic retroperitoneal tissue, bile duct, gallbladder wall, periportal area of the liver, salivary glands and the pancreas. Furthermore, all of the extrapancreatic lesions associated with AIP, such as sclerosing cholangitis, sclerosing sialadenitis and retroperitoneal fibrosis, show infiltration of abundant IgG4-positive plasma cells.

• #29 Clinical characteristics and long-term prognosis of autoimmune pancreatitis with renal lesions | Scientific Reports

  https://www.nature.com/articles/s41598-020-79899-3

  Autoimmune pancreatitis (AIP) is recognized as the pancreatic manifestation of a systemic IgG4-related disease that can involve various organs, including the kidney. […] The renal lesions associated with AIP are considered to be a specific pathologic feature of IgG4-RD, a disease termed IgG4-related kidney disease (IgG4-RKD). […] However, the way in which these renal lesions affect the treatment and long-term prognosis of AIP is still not well understood. […] AIP has been recognized as a pancreatic manifestation of IgG4-related systemic diseases. […] Approximately one-fourth to one-third of AIP patients are reported to have IgG4-RKD. […] One of the reasons for this is that although progression of IgG4-RKD may lead to renal dysfunction and renal failure, in most patients diagnosed with AIP, renal function remains normal or mildly compromised, even if renal involvement is present.

• #29 Clinical characteristics and long-term prognosis of autoimmune pancreatitis with renal lesions | Scientific Reports

  https://www.nature.com/articles/s41598-020-79899-3

  Therefore, when diagnosing AIP, it is important to assess images for the presence of renal lesions. […] The response of IgG4-RKD to steroids is considered to be as good as that of other IgG4-related diseases. […] In the present study, the cumulative relapse rates were significantly higher in the KD group than in the non-KD group (22.1% vs. 7.5% in one year and 61% vs. 21.9% in 3 years, respectively, P<0.001). [...] The presence of renal lesions might be another risk factor for relapse in AIP patients. [...] Interestingly, all 14 patients belonged to the non-KD group, and none of the patients in the KD group developed a malignancy during the study period, although this difference was not statistically significant (P=0.178).

• #30 Autoimmune Pancreatitis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/autoimmune-pancreatitis-causes-symptoms-and-treatment?srsltid=AfmBOoqIrFUfQhvFmny0AVGIKw7cLJ_Q6KuulikJOueTdxEqrDjmBodE

  Autoimmune Pancreatitis, abbreviated as AIP, is a chronic inflammatory condition in which the internal defence mechanism of the body identifies the pancreatic cells as foreign bodies and destroys the living elements. […] The nature of AIP being autoimmune, medical professionals, doctors and researchers have confirmed the abnormal activity of the bodys own internal immune system, in attacking the pancreas. Although the causative factors behind AIP are yet to be determined, healthcare experts have identified certain risk factors that make people more prone to acquiring AIP. […] Most people with confirmed cases of Type 1 AIP were reported to be men over the age of 60, making the older male population more inclined to contracting IgG4-related pancreatitis. […] Since the majority of instances of Type 2 AIP were observed in both men and women above the age of 40, alongside underlying gut complications of IBD, middle-aged individuals have higher chances of developing idiopathic duct-centric pancreatitis.

• #31 Review of Diagnostic Biomarkers in Autoimmune Pancreatitis: Where Are We Now?

  https://www.mdpi.com/2075-4418/11/5/770

  AIP, similar to other forms of IgG4-RD, shows a remarkable response to steroid therapy, which suggests that this disease may be associated with an autoimmune mechanism. […] Elevation of serum IgG4 levels is not specific for the diagnosis of AIP because this happens in a variety of diseases, including cancers, infections, and other autoimmune diseases. […] AIP often relapses, even after successful remission following steroid therapy. Therefore, maintenance steroid therapy (MST) could be useful for preventing AIP relapse. […] We previously identified laminin 511-E8 as a pathogenic antigen for AIP and suggested that anti-laminin 511-E8 may be a useful biomarker for AIP. However, various studies, including ours, could not identify specific biomarkers for the diagnosis of AIP. […] The presence of diffuse pancreatic swelling and IAC-complicated AIP are considered clinical predictors of AIP relapse; however, objective evaluation using biomarkers is desirable. […] IgG4 may be useful, not only in diagnosis but also for predicting relapse of AIP. […] Fukiage et al. suggested serum autotaxin (ATX) as a possible biomarker for predicting relapse of AIP.

• #32 Autoimmune pancreatitis can develop into chronic pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-9-77

  Autoimmune pancreatitis (AIP) has been recognized as a distinct type of pancreatitis that is possibly caused by autoimmune mechanisms. […] We have proposed a working hypothesis that AIP can develop into ordinary chronic pancreatitis resembling alcoholic pancreatitis over a long-term course based on several clinical findings, most notably frequent pancreatic stone formation. […] In this review article, we describe a series of study results to confirm our hypothesis and clarify that: 1) pancreatic calcification in AIP is closely associated with disease recurrence; […] approximately 40% of AIP patients experience pancreatic stone formation over a long-term course, for which a primary risk factor is narrowing of both Wirsungs and Santorinis ducts; and 4) nearly 20% of AIP patients progress to confirmed chronic pancreatitis according to the revised Japanese Clinical Diagnostic Criteria, with independent risk factors being pancreatic head swelling and non-narrowing of the pancreatic body duct.

• #32 Autoimmune pancreatitis can develop into chronic pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-9-77

  Some patients with AIP exhibit severe pancreatic calcification over a long-term course. Pancreatic stones are a primary characteristic of ordinary chronic pancreatitis, which suggests that AIP can progress to a form of chronic pancreatitis that meets the diagnostic criteria for ordinary chronic pancreatitis. […] This indicated that advanced stage AIP might have formerly been included in the diagnostic criteria for ordinary chronic pancreatitis, and especially alcoholic pancreatitis. […] We could therefore recommend careful follow-up of patients with AIP having these 2 risk factors to prevent progression to chronic pancreatitis. […] Finally, AIP can lead to severe pancreatic stone formation and progress to confirmed chronic pancreatitis over a long-term period, which may be most presumably caused by disease recurrence and pancreatic juice stasis.

• #33 Pancreatitis: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/8103-pancreatitis

  Most of the time, pancreatitis is acute and temporary. But causes that are chronic and don’t go away, such as inherited disorders, can cause chronic pancreatitis. Repeat episodes of acute pancreatitis can also lead to chronic pancreatitis. If your pancreas becomes inflamed too many times from repeated stress and injury, your body may learn to keep it constantly inflamed, even after the injury has stopped.

• #34 Autoimmune Pancreatitis | Padmavathi Gastro and Liver Hospital | Best liver transplant hospital in Hyderabad

  https://padmavathigastro.com/pancreas/autoimmune-pancreatitis.html

  Autoimmune pancreatitis, also called AIP, is a chronic inflammation that is thought to be caused by the bodys immune system attacking the pancreas and that responds to steroid therapy. […] Doctors dont know what causes autoimmune pancreatitis, but as in other autoimmune diseases, the bodys immune system attacks healthy body tissue. […] There is no established association between AIP and pancreatic cancer.

• #35 Prognosis and Long Term Outcomes of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/prognosis-and-long-term-outcomes-of-autoimmune-pancreatitis

  A Japanese survey indicated that the average life expectancies of male and female patients with chronic pancreatitis were 11 and 17 years shorter than those of the general population, respectively. The major cause of the death was malignancy, indicating that the standard death rates for bile duct and pancreatic cancer were very high (3.44 and 7.84, respectively). It is possible that immunodeficiency due to corticosteroid therapy and chronic inflammation of the pancreas may contribute to the occurrence of malignancy. […] Since AIP occurs predominantly in elderly patients, deficiency of the immunosurveillance system may be associated with its pathogenesis, which in turn may be associated with the occurrence of various malignancies other than pancreatic cancer (37). In addition to AIP, IgG4-related disease was reported to be highly complicated with malignancies (45).

• #36 Short Note on Etiology and Pathophysiology of Autoimmune Pancreat

  https://www.longdom.org/open-access/short-note-on-etiology-and-pathophysiology-of-autoimmune-pancreatitis-101067.html

  Autoimmune Pancreatitis (AIP) is a relatively rare form of chronic pancreatitis that is characterized by inflammation of the pancreas caused by an autoimmune response. […] In autoimmune pancreatitis, the body’s immune system mistakenly identifies the pancreatic tissue as a foreign invader and launches an inflammatory response. […] Autoimmune pancreatitis is a complex and relatively rare condition characterized by chronic inflammation of the pancreas due to an autoimmune response. […] Ongoing research continues to explore the underlying mechanisms of autoimmune pancreatitis, refine diagnostic criteria, and develop more targeted therapies.

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