Treatment Of Autoimmune Pancreatitis Research Articles

Dexamethasone’s Clinical Efficacy in Experimental Autoimmune Pancreatitis Correlates with a Unique Transcriptomic Signature, Whilst Kinase Inhibitors Are Not Effective

(1) Background: Autoimmune pancreatitis (AIP) is mainly treated with steroids. Using an AIP mouse model, we investigated two potential alternatives, the transforming growth factor-β-activated kinase 1 inhibitor, takinib, and the Janus kinase inhibitor, tofacitinib. (2) Methods: In a multicenter preclinical study, MRL/MpJ mice were injected with polyinosinic/polycytidylic acid (poly I:C) for two weeks to induce AIP. They were then treated for four weeks with either takinib (25, 50, or 75 mg/kg body weight), tofacitinib (5, 10 or 15 mg/kg), dexamethasone (1 mg/kg), or solvent, while the poly I:C injections were continued. The severity of AIP was assessed histopathologically. Flow cytometry was used to examine lymphocyte subtypes in the spleen and mesenteric lymph nodes. The pancreatic gene expression profiles were analyzed by RNA sequencing. (3) Results: Poly I:C-treated mice developed severe AIP with inflammation, destruction of acinar tissue, and fibrosis. Dexamethasone significantly attenuated the disease, while takinib or tofacitinib had no effects. Dexamethasone also antagonized the effects of poly I:C on the relative frequencies of the AIP-associated lymphocyte subtypes CD4/CD69, CD8/CD44high, and CD4/CD25/FoxP3 in the spleen. In the principal component analysis of pancreatic transcriptomics, poly I:C-injected mice treated with tofacitinib, takinib, or solvent clustered together, while untreated and dexamethasone-treated mice formed separate, unique clusters. (4) Conclusions: Dexamethasone effectively reduced AIP severity, while takinib and tofacitinib were ineffective. The unique gene expression profile in dexamethasone-treated mice may provide a basis for identifying new drug targets for AIP treatment.

Autoimmune pancreatitis: Cornerstones and future perspectives.

Autoimmune pancreatitis (AIP) is an autoimmune subtype of chronic pancreatitis resulting from the aberrant immune response against the pancreas, leading to inflammation and fibrosis. Although AIP is rare, its incidence is increasing and is often misdiagnosed as other pancreatic diseases. AIP is commonly classified into two types. Type 1 AIP (AIP-1) is typically associated with elevated serum immunoglobulin G4 (IgG4) levels and systemic manifestations, while type 2 AIP is typically a more localized form of the disease, and may coexist with other autoimmune disorders, especially inflammatory bowel diseases. Additionally, there is emerging recognition of a third type (type 3 AIP), which refers to immunotherapy-triggered AIP, although this classification is still gaining acceptance in medical literature. The clinical manifestations of AIP mainly include painless jaundice and weight loss. Elevated serum IgG4 levels are particularly characteristic of AIP-1. Diagnosis relies on a combination of clinical, laboratory, radiological, and histological findings, given the similarity of AIP symptoms to other pancreatic disorders. The mainstay of treatment for AIP is steroid therapy, which is effective in most cases. Severe cases might require additional imm-unosuppressive agents. This review aims to summarize the current knowledge of AIP, encompassing its epidemiology, etiology, clinical presentation, diagnosis, and treatment options. We also address the challenges and controversies in diagnosing and treating AIP, such as distinguishing it from pancreatic cancer and managing long-term treatment, highlighting the need for increased awareness and knowledge of this complex disease.

Treatment approaches in autoimmune pancreatitis (Review).

Autoimmune pancreatitis (AIP) is a rare disease. There are two distinct types of AIP: AIP type 1 (AIP-1), a pancreatic manifestation of a multi-organ disease linked to immunoglobulin (Ig)G4, and AIP type 2 (AIP-2), a pancreas-specific disease unrelated to IgG4. The usual course of treatment for AIP is oral corticosteroid medication. Rituximab has also been recommended for recurrent AIP-1 in order to initiate remission and provide ongoing treatment. Immunomodulators such as azathioprine are used to keep certain patients in remission. Evaluation also takes into account a number of pharmacological alternatives, including biologic drugs like anti-tumor necrosis factor therapy, a safe and efficient second-line treatment for AIP-2 relapse or steroid dependence. Corticosteroids and immunosuppressants, which are poorly tolerated due to considerable side effects, are being replaced by other biologic drugs, which may offer a beneficial therapeutic alternative.

Лечение аутоиммунного панкреатита у английского кокер-спаниеля

Autoimmune pancreatitis (AIP) is a poorly studied pathology in veterinary medicine, which is due to the low prevalence of the English Cocker Spaniel breed in the Russian Federation, as well as the lack of specific diagnosis of the disease. In human medicine, the incidence of the disease is also low. The principal difference between the treatment of AIP and the treatment of pancreatitis of a non–immune nature is the use of immunosuppressive drugs. The main guideline in evaluating the effectiveness of the chosen therapy is a positive clinical response to the use of drugs of this group.

Characteristics and outcomes of patients with autoimmune pancreatitis after pancreatectomy: a retrospective study

Objective: Autoimmune pancreatitis (AIP) is a rare and special type of pancreatitis, which is often difficult to distinguish from pancreatic cancer. We aimed to discuss the clinical characteristics, operation characteristics, and therapeutic effects of AIP patients who received surgical treatment based on retrospective analysis. Methods: The medical records of patients who underwent surgery at Peking Union Medical College Hospital from January 2012 to July 2021 and whose postoperative pathology was AIP were collected. Clinical symptoms, laboratory examinations, imaging features, surgical information, and prognostic data were analyzed. Results: Nineteen patients with AIP (median age 60 years old, 14 males) underwent surgery, whose main indication for surgery was suspected of a malignant lesion (n = 18, 94.7%). Ten patients underwent pancreaticoduodenectomy, 8 underwent distal pancreatectomy, and 1 underwent total pancreatectomy, the pathological result of whom were all AIP. The main postoperative complication was pancreatic fistula (n = 11, 57.9%), and one patient (5.3%) underwent a second operation due to gastrointestinal anastomotic bleeding. One patient (5.6%) had a recurrence of AIP, 3 patients (16.7%) were treated with glucocorticoid after surgery, and one patient (5.6%) was treated with immunosuppressors. Conclusions: Differential diagnosis of AIP from pancreatic cancer is complex. Operation difficulty and complication incidence for patients with AIP were similar to pancreatic cancer patients. Although glucocorticoid is the first-line treatment for AIP, surgery may still be necessary for patients who are strongly suspected of a malignant lesion.

Characteristics of mass-forming autoimmune pancreatitis commonly misdiagnosed as a malignant tumor.

This study aimed to explore the clinical characteristics and differential diagnosis of patients with autoimmune pancreatitis (AIP) and pancreatic cancer to prevent misdiagnosis and mistreatment. The clinical data of patients with AIP with suspected pancreatic or bile duct malignancy and pancreatic cancer were retrospectively analyzed. The risk factors and the diagnostic value of IgG4 and Tbil levels before treatment for AIP was investigated. Moreover, the imaging features and response to hormone therapy were analyzed. AIP was commonly observed in men. Compared to patients with pancreatic cancer, the incidence of poor appetite and weight loss and carbohydrate antigen 19-9 (CA19-9) level was lower in patients with AIP, while the immunoglobulin G4 (IgG4) level was higher (p < 0.05). After treatment, the IgG4 and CA19-9 levels in patients with AIP were decreased (p < 0.001). IgG4 level before treatment (OR = 2.452, 95%CI: 1.180-5.096, P = 0.016) and total bilirubin (Tbil) level before treatment (OR = 0.992, 95%CI: 0.985-0.998, P = 0.013) were independent risk factors of AIP. Furthermore, the diagnostic value of IgG4 level before treatment, Tbil level before treatment, IgG4/Tbil before treatment, and a combination of these indicators was high. Moreover, 15 (68.18%) patients with AIP had space-occupying lesions of the pancreas, and 16 (72.73%) had autoimmune cholangitis. Most patients with AIP were sensitive to hormone therapy. The Tbil and IgG4 levels, imaging findings, and hormone therapy reactivity could differentiate AIP from pancreatic cancer. A combination of IgG4, Tbil, and IgG4/Tbil before treatment might be a promising diagnostic biomarker for AIP.

S1483 A 20-Year-OId Female With Obstructive Jaundice and a Pancreatic Head Mass: A Case Report of Type 2 Autoimmune Pancreatitis

Introduction: Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that may be associated with IgG4 laden lymphoplasmacytic infiltration and fibrosis in multiple organs. It accounts for only a small percentage of all patients with chronic pancreatitis. It can present with abdominal pain, jaundice, recurrent acute pancreatitis, or clinical findings similar to pancreatic cancer. Radiologic findings can also be similar to the above. Therefore, it is important to fully understand the radiologic and histologic nuances of AIP. We review a case of a 20-year-old female who presented with abdominal pain and jaundice who was subsequently diagnosed with Type 2 AIP. Case Description/Methods: A patient with no known past medical history presented with one week of abdominal pain. Imaging demonstrated a normal appearing pancreas with a nodular area of soft tissue fullness at the junction of the pancreatic head and body. Patient returned a few weeks later with jaundice. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a significant biliary stricture which was stented. EUS with biopsies demonstrated mild acute on chronic inflammation of the ampulla and mild acute inflammation of the pancreatic head as well as benign pancreatic parenchyma with edema, very focal fibrosis, and focal neutrophilic infiltrate in acini and intralobular small ducts. Steroids were prescribed leading to resolution of signs and symptoms. Discussion: Initial presentation of AIP can mimic that of other pancreatic pathology so further imaging with biopsy is necessary to make a final diagnosis. Type 1 and Type 2 AIP both have histologic findings of periductal infiltration of plasma cells and lymphocytes, peculiar storiform fibrosis, and venulitis. Type 1 typically has a high number of IgG4 positive plasma cells and can be associated with various systemic lesions related to IgG4 positive cell infiltrates. Type 2 is known for intraluminal and intraepithelial neutrophils in small to medium ducts and acini causing destruction of the duct lumen and is considered a pancreatic specific disease. In both types imaging tends to show diffuse parenchymal enlargement with delayed enhancement and ductal strictures without significant upstream dilatation. Treatment of AIP consists of steroids with Type 1 known to have relapses requiring re-treatment. Based on the histologic and radiologic findings a presumptive diagnosis of Type 2 AIP was made. Response to steroid treatment and no evidence of relapse confirmed the diagnosis.Figure 1.: A single severe stenosis seen in the lower third of the common bile duct.

Pancreatitis autoinmune. Serie de casos y actualización bibliográfica

The concept and diagnostic criteria for autoimmune pancreatitis have evolved since the first case reports published in the 1960s. The advances in knowledge during the past two decades have resulted in the recognition of several pancreatic conditions that show, on histological evaluation, variable combinations of inflammation and fibrosis. In fact, autoimmune pancreatitis comprises two distinct diseases: type 1 and type 2. Type 2 autoimmune pancreatitis is a particular pancreatic disease about which there is limited data; it differs from the ‘classic’ IgG4-related type 1 autoimmune pancreatitis in terms of epidemiology, pathological characteristics and natural evolution. There are patients with complex pancreatic conditions where an autoimmune component and good response to steroids are demonstrated, but they do not meet the suggested criteria for the diagnosis of type 1 or type 2 autoimmune pancreatitis. Although the term steroid-responsive pancreatitis may be interpreted as a synonym to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that improve with this type of treatment. Here, we present three clinical cases and a literature review related to the diagnosis and treatment of autoimmune pancreatitis and other less frequent pancreatic disorders that present a good response to steroid therapy.

Autoimmune Pancreatitis and IgG4-related Cholangitis: A Single-center Experience

Background: Autoimmune pancreatitis (AIP) and IgG4-related cholangitis (IRC) are newly-recognized diseases. Reports in Thailand are scarce. Objective: To evaluate clinical presentations, other organ involvement (OOI), serology, radiologic, endoscopic features and treatment of AIP and IRC in one institute. Materials and Methods: Patients with AIP and IRC, who were diagnosed and followed-up at Siriraj Hospital during 2005 to 2016 were retrospectively reviewed. Results: There were 15 patients (75%) with AIP, 7 patients (37%) with IRC (4 isolated IRC and 3 IRC with AIP) and 1 (5%) with IgG4-related disease without AIP or IRC (sialadenitis and retroperitoneal fibrosis). Male to female ratio was 1.9: 1. Median age of onset was 64 years. Among the 15 AIP, all were type 1. Initial presentations were jaundice (60%), abdominal pain (40%), OOI (20%; sialadenitis, orbital pseudotumor), and weight loss (7%). Clinical manifestations during the course were jaundice (80%), abdominal pain (67%), weight loss (47%), OOI (47%; retroperitoneal fibrosis, IRC, sialadenitis, orbital pseudotumor, periurethral mass, lung nodules) and steatorrhea (7%). Serum IgG4 was elevated in all patients. Radiography showed diffuse type in 8 cases (53%), focal type in 5 (33%), and unknown in 2 (13%). More than half were suspicious of AIP before diagnosis. Corticosteroid was required in 10 patients (67%). Maintenance therapy was offered in 5 patients (33%) with steroid and azathioprine. Surgery was performed before diagnosis in 7 patients (47%). Spontaneous remission occurred in 4 patients (27%). Among the 7 IRC, initial presentations were jaundice (57%), weight loss (29%), abdominal pain (14%), prolonged fever (14%), and cholangitis (14%). Clinical manifestations during the course were jaundice (57%), abdominal pain (57%), weight loss (29%), recurrent cholangitis (14%) and retroperitoneal fibrosis (14%). Serum IgG4 was elevated in 5 patients (71%). Prednisolone was prescribed in 6 cases (86%). Five patients (71%) required azathioprine. Surgery was performed before diagnosis in 4 patients (57%). Conclusion: AIP and IRC were uncommon. The most common presentation was jaundice. All AIP were type 1 and more than half were diffuse type. OOI were common. Half of IRC had AIP. Approximately half of AIP/IRC patients underwent surgery before diagnosis. Corticosteroid was mainstay treatment. Keywords: Autoimmune pancreatitis, IgG4, Immunoglobulin G4, Sclerosing cholangitis, Thailand &nbsp;

Amelioration of Murine Autoimmune Pancreatitis by Targeted LTβR Inhibition and Anti-CD20 Treatment.

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis, for which treatment options, especially the long-term management, are limited. The only therapy that has been established and accepted so far is corticosteroids, but the relapse rate is significant. In the current study, we discern the effector mechanisms of targeted LTβR pathway inhibition using LTβR-Ig. Furthermore, the efficacy of LTβR-Ig therapy is compared with the depletion of immune cell subsets (CD4+ and CD20+), which are suggested to play a pathological role in AIP development. Three well-established mouse models of AIP were used to examine treatment efficacies and mechanisms. Tg(Ela1-Lta,b) mice represent a genetic model, in which AIP develops spontaneously. In MRL/Mp and IL-10-/- mice, AIP is induced by repeated polyinosinic:polycytidylic acid injection. Mice with AIP were treated with anti-CD20, anti-CD4 mAbs, or targeted LTβR-Ig. LTβR-Ig and anti-CD20 treatment led to significant improvement of AIP, including a decrease in autoantibody production and pancreatic inflammation in Tg(Ela1-Lta,b) and IL-10-/- mice. The molecular mechanism of this beneficial effect possibly involves the downregulation of Stat3 and noncanonical NF-κb activation. Anti-CD4 treatment reduced Th1 and Th2 signature but did not alleviate AIP. Additionally, in contrast to anti-CD20 or anti-CD4 treatments, blocking LTβR signaling disrupted tertiary lymphoid organs in all three models. We demonstrate that treatment with LTβR-Ig or anti-CD20 Ab alleviated murine AIP. LTβR-Ig treatment for AIP was effective in both lymphotoxin-dependent and lymphotoxin-independent AIP models, possibly because of its dual anti-inflammatory and antiautoimmune mechanisms.

PrescrAIP: A Pan-European Study on Current Treatment Regimens of Auto-Immune Pancreatitis.

Introduction: Treatment of autoimmune pancreatitis (AIP) is based solely on consensus and has yet to become standardized. Consequently, therapeutic regimens vary greatly between countries and centers, and largely depend on the experience of the physician. At this moment, the optimal regimen for inducing disease remission and preventing relapse is unknown.Objectives: The primary objective of this study is to describe current treatment regimens used in Europe, and to compare their effectiveness in inducing remission and preventing and treating relapse. The secondary objectives are: to identify risk factors for relapse; to assess the diagnostic accuracy of the Unified-AIP criteria; to assess the performance of the M-ANNHEIM score for predicting relapse; and to assess long-term outcomes including pancreatic exocrine insufficiency and pancreatic cancer.Methods: This is an international, retrospective, observational cohort study, performed in over 40 centers from 16 European countries. Eligible are all patients diagnosed with AIP from 2005 onwards, regardless of the used diagnostic criteria. Data on study subjects will be retrieved from the hospital's electronic medical records and registered with a standardized, web-based, electronic case report form (eCRF). To compare the effectiveness of treatment regimens in inducing remission, preventing relapse, and treating relapse, subjects will be stratified in groups based on: type of therapy; initial therapy dose; cumulative therapy dose; therapy tapering speed and duration; and having received maintenance therapy or not.Ethics and Dissemination: Ethical and/or institutional review board approvals are obtained by all participating centers according to local regulations. The study complies with the General Data Protection Regulation (GDPR). All manuscripts resulting from the study will be submitted to peer-reviewed journals.Conclusion: This is the first pan-European retrospective registry for AIP. It will produce the first large-scale data on treatment of European patients with AIP, providing answers on the use and effectiveness of treatment regimens. In the future, this collaboration may provide a network for continuation into a prospective European registry.

Steroid Therapy and Steroid Response in Autoimmune Pancreatitis.

Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. AIP is classified into two distinct subtypes on the basis of the histological subtype: immunoglobulin G4 (IgG4)-related lymphoplasmacytic sclerosing pancreatitis (type 1) and idiopathic duct-centric pancreatitis (type 2). Type 1 AIP is often accompanied by systemic lesions, biliary strictures, hepatic inflammatory pseudotumors, interstitial pneumonia and nephritis, dacryoadenitis, and sialadenitis. Type 2 AIP is associated with inflammatory bowel diseases in approximately 30% of cases. Standard therapy for AIP is oral corticosteroid administration. Steroid treatment is generally indicated for symptomatic cases and is exceptionally applied for cases with diagnostic difficulty (diagnostic steroid trial) after a negative workup for malignancy. More than 90% of patients respond to steroid treatment within 1 month, and most within 2 weeks. The steroid response can be confirmed on clinical images (computed tomography, ultrasonography, endoscopic ultrasonography, magnetic resonance imaging, and 18F-fluorodeoxyglucose-positron emission tomography). Hence, the steroid response is included as an optional diagnostic item of AIP. Steroid treatment results in normalization of serological markers, including IgG4. Short- and long-term corticosteroid treatment may induce adverse events, including chronic glycometabolism, obesity, an immunocompromised status against infection, cataracts, glaucoma, osteoporosis, and myopathy. AIP is common in old age and is often associated with diabetes mellitus (33–78%). Thus, there is an argument for corticosteroid therapy in diabetes patients with no symptoms. With low-dose steroid treatment or treatment withdrawal, there is a high incidence of AIP recurrence (24–52%). Therefore, there is a need for long-term steroid maintenance therapy and/or steroid-sparing agents (immunomodulators and rituximab). Corticosteroids play a critical role in the diagnosis and treatment of AIP.

Autoimmune pancreatitis: an update.

Since the time of inception of autoimmune pancreatitis (AIP), our knowledge of autoimmune pancreatitis has expanded significantly. The aim of this review is to provide an update on clinical manifestations, diagnosis, imaging features, and treatment of AIP. Type 1 AIP is the pancreatic manifestation of IgG4-related systemic disease, which can be diagnosed using a combination of clinical, histopathological, pancreatic imaging findings in conjunction with manifestation in other organs, as well of responsiveness to steroid treatment. It is vital to differentiate AIP from pancreatic cancer since both can mimic each other clinically and radiologically. Type 2 AIP is a rare but distinct subtype of AIP which occurs mostly in the younger patient. AIP is steroid-responsive chronic pancreatitis with distinct manifestations on imaging.

Insulin secretion improvement during steroid therapy for autoimmune pancreatitis according to the onset of diabetes mellitus.

Autoimmune pancreatitis (AIP) is frequently complicated by diabetes mellitus (DM), but DM associated with AIP is reported to improve after steroid therapy. The aim of this study is to investigate glucose intolerance during steroid therapy according to the onset of DM. Sixty-one patients who underwent steroid therapy for AIP were included into this study. We evaluated C peptide index (CPI), homeostasis model assessment for insulin resistance (HOMA-R), and the pancreatic diameter at AIP diagnosis and after 4weeks, 1year, and 2years of steroid therapy. Patients were categorized into three groups according to DM onset: Pre-existing DM (pDM), concurrent DM (cDM), and non-DM (nDM). Forty-three patients (71%) had DM: 15 pDM and 28 cDM. At AIP diagnosis, CPI was lower in patients with pDM (0.7, P = 0.007) and cDM (0.9, P = 0.018) than nDM (1.3). After 4weeks of steroid therapy, CPI improved in cDM (P < 0.001) and in nDM (P = 0.021). After 2years of steroid therapy, HOMA-R increased (2.1-3.0, P = 0.007) but CPI gradually improved (1.0-2.1, P = 0.004). DM improved in 23% of cDM, and 55% of insulin users in cDM discontinued using insulin. Pancreatic atrophy was seen in 30%, and was associated with DM. DM in patients with AIP was associated with impaired insulin secretion rather than insulin resistance. Insulin secretion improved during steroid therapy for AIP in patients with concurrent DM. Thus, glucose intolerance can be an indication for AIP treatment.

Su1453 – Diagnosis and Treatment of Autoimmune Pancreatitis: A Chilean Experience

Su1453 – Diagnosis and Treatment of Autoimmune Pancreatitis: A Chilean Experience

Feasibility and usefulness of endoscopic ultrasonography-guided shear-wave measurement for assessment of autoimmune pancreatitis activity: a prospective exploratory study

PurposeTo assess the feasibility and the clinical usefulness of a newly developed endoscopic ultrasonography (EUS) shear-wave elastography technique (EUS shear-wave measurement: EUS-SWM) in the diagnosis and treatment of autoimmune pancreatitis (AIP).MethodsTissue elasticity was measured in the pancreas in 160 patients. The success rate of EUS-SWMs, the velocity of the shear wave (Vs, m/s), and the reliability index of the Vs measurement (VsN) were evaluated, and the elasticity (median Vs) was compared between AIP patients (n = 14) and normal controls.ResultsA total of 3837 EUS-SWMs were performed without adverse events. Overall, 97.6% (3743/3837) were successful. The median VsN was 74%. The median Vs values of the pancreas were as follows: 2.22 m/s in the pancreatic head (push position), 2.36 m/s in the head (pull position), 1.99 m/s in the body, and 2.25 m/s in the tail. The median Vs of the AIP group (2.57 m/s) was significantly higher than that of the normal controls (1.89 m/s) (P = 0.0185). The mean Vs significantly decreased from 3.32 m/s to 2.46 m/s after steroid therapy (n = 6) (P = 0.0234).ConclusionEUS-SWM is feasible and generates credible results. EUS-SWM was a useful method for assessment of the effect of steroid therapy in AIP patients.

Development of Pancreatic Adenocarcinoma in Autoimmune Pancreatitis: A Systematic Review

Introduction: Presentation of autoimmune pancreatitis (AIP) can be similar to pancreatic adenocarcinoma (PAC) with a pancreatic mass that may cause obstructive jaundice. Chronic pancreatitis predisposes patients to PAC. The relationship between AIP and PAC is unknown. The aim of this systematic review is to determine the frequency of incident PAC in AIP, and secondarily to assess for prevalent PAC and AIP. Methods: A systematic search using PubMed, Embase, Scopus & Cochrane was performed. Search criteria included “autoimmune pancreatitis,” “pancreatic cancer,” “adenocarcinoma,” & “carcinoma” with all permutations. AIP was defined by international guidelines. Our inclusion criterion for incident PAC cases was a minimum of 1 year between AIP and PAC diagnoses to exclude prevalent cancers. The inclusion criterion for prevalent PAC cases was defined by presence of AIP and PAC within 1 year. The search yielded 636 results reviewed by 2 authors. 8 case reports and 4 retrospective studies with a total of 19 patients were included in the combined analyses. Results: There were 7 cases of incident PAC, with 4/7 (57.1%) men, mean age 67±14.1yrs (Range 39-80yrs) (Table 1). Mean time from AIP to PAC diagnosis was 78.1±60.9months (Range 12-186 months). 6/7 (85.7%) patients received prednisone therapy for AIP. 12 total cases of prevalent PAC with AIP were identified, all of which were men, mean age 61.4±5yrs (Range 53-70yrs) (Table 2). All prevalent cases of PAC with AIP were diagnosed simultaneously, with pathologic confirmation of both entities present on histology in 11/12 cases. Medical treatment of AIP was not reported for any prevalent PAC cases. Stage of PAC at diagnosis when reported was similar between incident & prevalent cases with most cases being unresectable (60% vs. 66.7%). Conclusion: Development of PAC in underlying AIP is exceedingly rare with only 7 total incident cases reported in the literature. The absolute risk remains unknown and was unable to be calculated based on included study types in this analysis. The number of prevalent cases of PAC concomitantly diagnosed with AIP should prompt a comprehensive investigation to exclude the presence of underlying malignancy and should be performed in cases of newly diagnosed AIP. Further prospective studies are warranted to determine overall risk of PAC in AIP.2733_A Figure 1. Study Characteristics of Autoimmune Pancreatitis Patients who Developed Incident Pancreatic Adenocarcinoma2733_B Figure 2. Study Characteristics of Autoimmune Pancreatitis Patients who had Prevalent Pancreatic Adenocarcinoma

Diagnosis and treatment of autoimmune pancreatitis.

Clinical management of autoimmune pancreatitis changed over the last years. The lack of high-quality studies, probably due to the relative rarity of the disease, has not fully clarified many diagnostic and therapeutic aspects. Aim of this review is to overview the literature from a clinical point of view, focusing on diagnostic and therapeutic approach to this complicated disease. They have been focused mainly on the risk of misdiagnosing a cancer. Many significant articles have been published on the treatment strategies of autoimmune pancreatitis, considering both induction and maintenance therapy. Diagnosis of autoimmune pancreatitis remains challenging, particularly in focal pancreatic involvement, despite recent advances on imaging modalities. Treatment is based on induction and maintenance phases. Steroid treatment remains the best option to induce remission. Maintenance therapy may be used to prevent disease relapses, and low-dose steroids, azathioprine or rituximab are the therapeutic options. However, it remains unclear which patient needs to be treated.

The cutting-edge of endoscopic diagnosis and treatment for autoimmune pancreatitis

The cutting-edge of endoscopic diagnosis and treatment for autoimmune pancreatitis

DIAGNOSIS AND TREATMENT OF AUTOIMMUNE PANCREATITIS. (REVIEW)

The autoimmune pancreatitis was described in 1961 as “a primary inflammatory sclerosis of the pancreas”. Subsequent reports have described this disease as lymphoplasmacytic sclerosing pancreatitis, chronic sclerosing pancreatitis, nonalcoholic duct-destructive pancreatitis, inflammatory pseudotumor of pancreas, etc. The concept of autoimmune pancreatitis was proposed by Yoshida et al. in 1995. Numerous clinical, serological, radiological and pathological studies were conducted. Diagnostic criteria, serological markers and pathological features were introduced to facilitate autoimmune pancreatitis patients management. Also data were obtained indicating autoimmune pancreatitis as a part of new clinicopathological entity - IgG4-related sclerosing disease. Important issue is surgical treatment of autoimmune pancreatitis which is performed in case of suspected pancreatic cancer as a rule. Thus, autoimmune pancreatitis is an actual problem of modern medicine and requires further study.