Background:A high prevalence of diabetes mellitus(DM)coexisting with autoimmune pancreatitis(AIP)is observed.However,evidence on the circumstances under which corticosteroid therapy(CST)for AIP improves or worsens DM ...Background:A high prevalence of diabetes mellitus(DM)coexisting with autoimmune pancreatitis(AIP)is observed.However,evidence on the circumstances under which corticosteroid therapy(CST)for AIP improves or worsens DM is scarce.This study aimed to demonstrate and identify predictors of DM control under the influence of CST.Methods:Patients diagnosed with type 1 AIP were enrolled from a prospectively maintained cohort and were classified into three groups according to the chronology in which AIP and DM were diagnosed:pre-existing DM(pDM),concurrent DM(cDM),and non-DM(nDM).The responses of DM to CST were assessed when corticosteroid was ceased or tapered to a maintenance dose and classified as‘improvement’and‘non-improvement’(including‘no change’and‘exacerbation’).Results:Among 101 patients with type 1 AIP,52(51.5%)patients were complicated with DM at the time of AIP diagnosis,with 36 patients in the cDM group and 16 patients in the pDM group.The incidences of diffuse pancreatic swelling(72.2%)and pancreatic body/tail involvement(91.7%)were significantly higher in the cDM group than in both the pDM and nDM groups.Of the 52 patients with DM,CST was administered in 48 cases.Multivariate logistic analysis identified that elevated serum gamma-glutamyl transferase(GGT)level at AIP diagnosis[odds ratio(OR)=0.032,95%confidence interval(CI):0.003-0.412,P=0.008]and pancreatic atrophy after CST(OR=0.027,95%CI:0.003-0.295,P=0.003)were negatively associated with DM control improvement.Conclusions:Patients with diffuse pancreatic swelling and pancreatic body/tail involvement in pancreatitis tended to be complicated with cDM at AIP diagnosis.CST exerted a beneficial effect on the clinical course of DM in nearly half of the AIP patients complicated with DM at diagnosis,particularly in those without elevated serum GGT levels at diagnosis and who did not experience pancreatic atrophy after CST.展开更多
BACKGROUND Autoimmune pancreatitis(AIP)is a chronic form of pancreatitis characterized by diffused enlargement of the pancreas and irregular stenosis of the main pancreatic duct.Some studies have reported that AIP can...BACKGROUND Autoimmune pancreatitis(AIP)is a chronic form of pancreatitis characterized by diffused enlargement of the pancreas and irregular stenosis of the main pancreatic duct.Some studies have reported that AIP can cause hemorrhage of gastric varices(GV)related to portal hypertension(PH).However,such cases are rare.In addition,the association of PH with AIP is unclear.At the same time,the efficacy and duration of glucocorticoid therapy is also controversial.CASE SUMMARY In this case,we reported a case of GV in pancreatic PH associated with AIP.Enhanced abdominal computed tomography(CT)suggested splenic vein(SV)and superior mesenteric vein(SMV)thromboses.The patient received a long-term glucocorticoid therapy,that the initial dose of 40 mg is reduced weekly by 5 mg,and then reduced to 5 mg for long-term maintenance.CT and gastroscopic examination after 8 mo of treatment indicated that SV and SMV were recanalized,pancreatic stiffness and swelling were ameliorated,and the GV almost completely disappeared.CONCLUSION Long-term glucocorticoid therapy can alleviate the development of GV in patients with AIP and has potential reversibility.展开更多
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 ...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.展开更多
In this editorial we comment on the article by Jaber et al.Autoimmune pancreatitis(AIP)represents a distinct form of pancreatitis,categorized into AIP-1 and AIP-2,characterized by obstructive jaundice,lymphoplasmacyti...In this editorial we comment on the article by Jaber et al.Autoimmune pancreatitis(AIP)represents a distinct form of pancreatitis,categorized into AIP-1 and AIP-2,characterized by obstructive jaundice,lymphoplasmacytic infiltrate,and fibrosis.AIP-1,associated with elevated immunoglobulin G4(IgG4)levels,exhibits higher relapse rates,affecting older males,while AIP-2 is less common and linked to inflammatory bowel disease.AIP is considered a manifestation of IgG4-related systemic disease,sharing characteristic histological findings.Steroids are the primary treatment,with emerging biomarkers like interferon alpha and inter-leukin-33.AIP poses an increased risk of various malignancies,and the assoc-iation with pancreatic cancer is debated.Surgery is reserved for severe cases,necessitating careful evaluation due to diagnostic challenges.AIP patients may have concurrent PanINs but display favorable long-term outcomes compared to pancreatic cancer patients.Thorough diagnostic assessment,including biopsy and steroid response,is crucial for informed surgical decisions in AIP.展开更多
We are writing in response to the paper published in the World Journal of Gastroenterology by Zhou et al.The authors identified higher serum immunoglobulin(Ig)G4 levels and age over 55 years as independent risk factor...We are writing in response to the paper published in the World Journal of Gastroenterology by Zhou et al.The authors identified higher serum immunoglobulin(Ig)G4 levels and age over 55 years as independent risk factors for disease relapse.Despite notable strengths,it is crucial to address potential biases.Firstly,the cohort study included 189 patients with autoimmune pancreatitis(AIP)type 1(with higher IgG4 seropositivity and higher relapse)and 24 with type 2(with lower IgG4 seropositivity and lower relapse).Consequently,most,if not all,AIP type 2 patients were assigned to the normal group,possibly inflating the association of higher serum IgG4 levels with relapse and potentially exaggerating the association of older age with relapse.Secondly,the authors did not provide sufficient details regarding AIP diagnosis,such as the ratio of definitive vs probable cases and the proportion of biopsies.In cases where histological evidence is unavailable or indeterminate,AIP type 2 may be misdiagnosed as definitive type 1,and type 1 may also be misdiagnosed as probable type 2,particularly in cases with normal or mildly elevated serum IgG4 levels.Lastly,in this retrospective study,approximately one-third of the consecutive patients initially collected were excluded for various reasons.Accordingly,the impact of nonrandom exclusion on relapse outcomes should be carefully considered.In conclusion,the paper by Zhou et al offers plausible,though not entirely compelling,evidence suggesting a predictive role of elevated serum IgG4 levels and advanced age in AIP relapse.The foundation for future investigations lies in ensuring a reliable diagnosis and accurate disease subtyping,heavily dependent on obtaining histological specimens.In this regard,endoscopic ultrasound-guided fine-needle biopsy emerges as a pivotal component of the diagnostic process,contributing to mitigating biases in future explorations of the disease.展开更多
Autoimmune pancreatitis(AIP)is a rare chronic autoimmune disorder.The diagnosis of AIP mainly depends on histopathology,imaging and response to treatment.Serum immunoglobulin 4(IgG4)is used only as collateral evidence...Autoimmune pancreatitis(AIP)is a rare chronic autoimmune disorder.The diagnosis of AIP mainly depends on histopathology,imaging and response to treatment.Serum immunoglobulin 4(IgG4)is used only as collateral evidence in diagnostic criteria for AIP because of its moderate sensitivity.Serum IgG4 levels are normal in 15%-37%of type 1 AIP and most of type 2 AIP patients.In these patients,the indeterminate imaging and histopathology may lead to the difficulty in definitive diagnosis of AIP.Therefore,discovery of new biomarkers is impor-tant for AIP diagnosis.Here,we provide some views on the progression and challenges in identifying novel serological biomarkers in AIP diagnosis.展开更多
BACKGROUND Autoimmune pancreatitis(AIP)is a rare form of autoimmune-mediated pancrea-titis,which is easily misdiagnosed as pancreatic cancer and thus treated surgi-cally.We studied the diagnosis and treatment of a pat...BACKGROUND Autoimmune pancreatitis(AIP)is a rare form of autoimmune-mediated pancrea-titis,which is easily misdiagnosed as pancreatic cancer and thus treated surgi-cally.We studied the diagnosis and treatment of a patient with type 1 AIP recent-ly admitted to our hospital,and reviewed the literature to provide a reference for clinical diagnosis of AIP.CASE SUMMARY The chief complaint was yellowing of the body,eyes and urine for 21 d.The pa-tient's clinical presentation was obstructive jaundice and imaging suggested pan-creatic swelling.It was difficult to distinguish between inflammation and tumor.Serum immunoglobulin G4(IgG4)was markedly elevated.IgG4 is an important serological marker for type 1 AIP.The patient was diagnosed with AIP,IgG4-related cholangitis,acute cholecystitis and hepatic impairment.After applying hormonal therapy,the patient's symptoms improved significantly.At the same time,imaging suggested that pancreatic swelling subsided,and liver function and other biochemical indicators decreased.The treatment was effective.CONCLUSION In patients with pancreatic swelling,the possibility of AIP should be considered.展开更多
BACKGROUND Autoimmune pancreatitis(AIP)has been linked with elevated immunoglobulin(Ig)G4 levels.The characteristics and outcomes of AIP based on serum markers have not been fully evaluated.AIM To compare clinical fea...BACKGROUND Autoimmune pancreatitis(AIP)has been linked with elevated immunoglobulin(Ig)G4 levels.The characteristics and outcomes of AIP based on serum markers have not been fully evaluated.AIM To compare clinical features,treatment efficacy,and outcome of AIP based on serum IgG4 levels and analyze predictors of relapse.METHODS A total of 213 patients with AIP were consecutively reviewed in our hospital from 2006 to 2021.According to the serum IgG4 level,all patients were divided into two groups,the abnormal group(n=148)with a high level of IgG4[>2×upper limit of normal(ULN)]and the normal group(n=65).The t-test or Mann-Whitney U test was used to compare continuous variables.Categorical parameters were compared by theχ^(2) test or Fisher’s exact test.Kaplan-Meier curves Zhou GZ et al.Clinical characteristics and outcome of AIP WJG https://www.wjgnet.com 5126 September 21,2023 Volume 29 Issue 35 and log-rank tests were established to assess the cumulative relapse rates.Univariate and multivariate analyses were used to investigate potential risk factors of AIP relapse.RESULTS Compared with the normal group,the abnormal group had a higher average male age(60.3±10.4 vs 56.5±12.9 years,P=0.047);higher level of serum total protein(72.5±7.9 g/L vs 67.2±7.5 g/L,P<0.001),IgG4(1420.5±1110.9 mg/dL vs 252.7±106.6 mg/dL,P<0.001),and IgE(635.6±958.1 IU/mL vs 231.7±352.5 IU/mL,P=0.002);and a lower level of serum complement C3(100.6±36.2 mg/dL vs 119.0±45.7 mg/dL,P=0.050).In addition,a lower number of cases with abnormal pancreatic duct and pancreatic atrophy(23.6%vs 37.9%,P=0.045;1.6%vs 8.6%,P=0.020,respectively)and a higher rate of relapse(17.6%vs 6.2%,P=0.030)were seen in the abnormal group.Multivariate analyses revealed that serum IgG4[(>2×ULN),hazard ratio(HR):3.583;95%confidence interval(CI):1.218–10.545;P=0.020]and IgA(>1×ULN;HR:5.908;95%CI:1.199–29.120;P=0.029)and age>55 years(HR:2.383;95%CI:1.056–5.378;P=0.036)were independent risk factors of relapse.CONCLUSION AIP patients with high IgG4 levels have clinical features including a more active immune system and higher relapse rate.Several factors,such as IgG4 and IgA,are associated with relapse.展开更多
Autoimmune pancreatitis(AIP),a chronic inflammation caused by the immune system attacking the pancreas,usually presents imaging and clinical features that overlap with those of pancreatic ductal adenocarcinoma(PDAC).S...Autoimmune pancreatitis(AIP),a chronic inflammation caused by the immune system attacking the pancreas,usually presents imaging and clinical features that overlap with those of pancreatic ductal adenocarcinoma(PDAC).Serum biomarkers,substances that quantitatively change in sera during disease development,are a promising non-invasive tool with high utility for differentiating between these diseases.In this way,the presence of AIP is currently suspected when serum concentrations of immunoglobulin G4(IgG4)antibody are elevated.However,this approach has some drawbacks.Notably,IgG4 antibody concentrations are also elevated in sera from some patients with PDAC.This review focuses on the most recent and relevant serum biomarkers proposed to differentiate between AIP and PDAC,evaluating the usefulness of immunoglobulins,autoantibodies,chemokines,and cytokines.The proposed serum biomarkers have proven useful,although most studies had a small sample size,did not examine their presence in patients with PDAC,or did not test them in humans.In addition,current evidence suggests that a single serum biomarker is unlikely to accurately differentiate these diseases and that a set of biomarkers will be needed to achieve adequate specificity and sensitivity,either alone or in combination with clinical data and/or radiological images.展开更多
Autoimmune pancreatitis(AIP)is a complex,poorly understood disease gaining increasing attention."Clinical Characteristics and Outcome of AIP Based on Serum IgG4 levels,"investigated AIP with a focus on serum...Autoimmune pancreatitis(AIP)is a complex,poorly understood disease gaining increasing attention."Clinical Characteristics and Outcome of AIP Based on Serum IgG4 levels,"investigated AIP with a focus on serum immunoglobulin(Ig)G4 levels.The 213 patients with AIP were classified according to serum IgG4 levels:Abnormal(elevated)and normal.Patients with higher IgG4 levels exhibited a more active immune system and increased relapse rates.Beyond IgG4,the IgA levels and age independently contributed to relapse risk,guiding risk assessment and tailored treatments for better outcomes.However,limitations persist,such as no IgA correlation with IgG4 levels,absent data on autoantibodypositive AIP cases critical for Asian diagnostic criteria,and unexplored relapse rates in high serum IgG AIP by subtype.Genetic factors and family histories were not addressed.As the understanding and referral of seronegative AIPs increase,there's a growing need for commercially available,highly sensitive,and specific autoantibodies to aid in diagnosing individuals with low or absent serum IgG4 levels.展开更多
Pancreatic ductal adenocarcinoma(PDAC)is one of the most lethal malignancies and is developing into the 2nd leading cause of cancer-related death.Often,the clinical and radiological presentation of PDAC may be mirrore...Pancreatic ductal adenocarcinoma(PDAC)is one of the most lethal malignancies and is developing into the 2nd leading cause of cancer-related death.Often,the clinical and radiological presentation of PDAC may be mirrored by other inflammatory pancreatic masses,such as autoimmune pancreatitis(AIP)and massforming chronic pancreatitis(MFCP),making its diagnosis challenging.Differentiating AIP and MFCP from PDAC is vital due to significant therapeutic and prognostic implications.Current diagnostic criteria and tools allow the precise differentiation of benign from malignant masses;however,the diagnostic accuracy is imperfect.Major pancreatic resections have been performed in AIP cases under initial suspicion of PDAC after a diagnostic approach failed to provide an accurate diagnosis.It is not unusual that after a thorough diagnostic evaluation,the clinician is confronted with a pancreatic mass with uncertain diagnosis.In those cases,a re-evaluation must be entertained,preferably by an experienced multispecialty team including radiologists,pathologists,gastroenterologists,and surgeons,looking for disease-specific clinical,imaging,and histological hallmarks or collateral evidence that could favor a specific diagnosis.Our aim is to describe current diagnostic limitations that hinder our ability to reach an accurate diagnosis among AIP,PDAC,and MFCP and to highlight those disease-specific clinical,radiological,serological,and histological characteristics that could support the presence of any of these three disorders when facing a pancreatic mass with uncertain diagnosis after an initial diagnostic approach has been unsuccessful.展开更多
Autoimmune pancreatitis(AIP)is a rare disease clinically characterized by obstructive jaundice,unintentional weight loss,acute pancreatitis,focal pancreatic mass,and diabetes.AIP is classified into two subtypes-type 1...Autoimmune pancreatitis(AIP)is a rare disease clinically characterized by obstructive jaundice,unintentional weight loss,acute pancreatitis,focal pancreatic mass,and diabetes.AIP is classified into two subtypes-type 1 and type 2-according to pathological findings,clinical features,and serology test results,but some cases may be defined as type not otherwise in the absence of pathological findings and inflammatory bowel disease.To address the differences in diagnostic criteria by country,standard diagnostic criteria for AIP were proposed in 2011 by an international consensus of expert opinions.Differential diagnosis of AIP from pancreatic ductal adenocarcinoma is important but remains challenging for clinicians.Fortunately,all subtypes of AIP show dramatic response to steroid treatment.This review discusses the current perspectives on the diagnosis and management of AIP in clinical practice.展开更多
AIM:To explore the optimal steroid therapeutic strategy for autoimmune pancreatitis(AIP).METHODS:This study was conducted retrospectively in two large institutions in China.Patients with clinically,radiologically and ...AIM:To explore the optimal steroid therapeutic strategy for autoimmune pancreatitis(AIP).METHODS:This study was conducted retrospectively in two large institutions in China.Patients with clinically,radiologically and biochemically diagnosed AIP were enrolled.The performed radiological investigations and biochemical tests,the regimen of the given steroid treatment,remission and relapse whether with and without steroid therapy were analyzed.RESULTS:Twenty-eight patients with AIP received steroid treatment,while 40 patients were treated surgically by pancreatoduodenectomy,distal pancreatectomy and choledochojejunostomy,radiofrequency ablation for the enlarged pancreatic head,percutaneous transhepatic biliary drainage and endoscopic biliary drainage.The starting oral prednisolone dose was 30 mg/d in 18(64.3%) patients and 40 mg/d in 10(35.7%) patients administered for 3 wk.The remission rate of AIP patients with steroid treatment(96.4%) was significantly higher than in those without steroid treatment(75%).Maintenance therapy(oral prednisolone dose 5 mg/d) was performed after remission for at least 6-12 mo to complete the treatment course.Similarly,the relapse rate was significantly lower in AIP patients with steroid treatment(28.6%) than in those without steroid treatment(42.5%).Steroid re-treatment was effective in all relapsed patients with or without steroid therapy.CONCLUSION:Steroid therapy should be considered in all patients with active inflammatory phase of AIP.However,the optimal regimen still should be trailed in larger numbers of patients with AIP.展开更多
Helicobacter pylori(H.pylori)is an infectious agent influencing as much as 50%of the world’s population.It is the causative agent for several diseases,most especially gastric and duodenal peptic ulcer,gastric adenoca...Helicobacter pylori(H.pylori)is an infectious agent influencing as much as 50%of the world’s population.It is the causative agent for several diseases,most especially gastric and duodenal peptic ulcer,gastric adenocarcinoma and mucosaassociated lymphoid tissue lymphoma of the stomach.A number of other,extragastric manifestations also are associated with H.pylori infection.These include neurological disorders,such as Alzheimer’s disease,demyelinating multiple sclerosis and Parkinson’s disease.There is also evidence for a relationship between H.pylori infection and such dermatological diseases as psoriasis and rosacea as well as a connection with infection and open-angle glaucoma.Generally little is known about the relationship between H.pylori infection and diseases of the pancreas.Most evidence about H.pylori and its potential role in the development of pancreatic diseases concerns pancreatic adenocarcinoma and autoimmune forms of chronic pancreatitis.There is data(albeit not fully consistent)indicating modestly increased pancreatic cancer risk in H.pylori-positive patients.The pathogenetic mechanism of this increase is not yet fully elucidated,but several theories have been proposed.Reduction of antral Dcells in H.pylori-positive patients causes a suppression of somatostatin secretion that,in turn,stimulates increased secretin secretion.That stimulates pancreatic growth and thus increases the risk of carcinogenesis.Alternatively,H.pylori,as a part of microbiome dysbiosis and the so-called oncobiome,is proven to be associated with pancreatic adenocarcinoma development via the promotion of cellular proliferation.The role of H.pylori in the inflammation characteristic of autoimmune pancreatitis seems to be explained by a mechanism of molecular mimicry among several proteins(mostly enzymes)of H.pylori and pancreatic tissue.Patients with autoimmune pancreatitis often show positivity for antibodies against H.pylori proteins.H.pylori,as a part of microbiome dysbiosis,also is viewed as a potential trigger of autoimmune inflammation of the pancreas.It is precisely these relationships(and associated equivocal conclusions)that constitute a center of attention among pancreatologists,immunologists and pathologists.In order to obtain clear and valid results,more studies on sufficiently large cohorts of patients are needed.The topic is itself sufficiently significant to draw the interest of clinicians and inspire further systematic research.Next-generation sequencing could play an important role in investigating the microbiome as a potential diagnostic and prognostic biomarker for pancreatic cancer.展开更多
Background:Autoimmune pancreatitis(AIP)is a well-recognized fibroinflammatory disease of the pancreas.Despite the significant number of studies published on AIP type 1 and 2,no studies have been focused on AIP type no...Background:Autoimmune pancreatitis(AIP)is a well-recognized fibroinflammatory disease of the pancreas.Despite the significant number of studies published on AIP type 1 and 2,no studies have been focused on AIP type not otherwise specified(NOS)and therefore very little is known about clinical features and long-term outcomes of these patients.The aim of this study was to investigate clinical and radiological features of AIP type NOS-patients.Methods:Patients classified as AIP type NOS at clinical onset included in our database prospectively maintained since 1995 were evaluated.Epidemiological,clinical data were collected and analyzed.Results:Forty-six patients were included in the study.The clinical onset was mainly characterized by weight loss,jaundice and acute pancreatitis.Eight patients(17.4%)were reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis.Seven patients(15.2%)experienced relapse after steroid treatment but only one(2.2%)needed immunosuppressive drugs because of recurrent relapses.Conclusions:AIP type NOS shares clinical features similar to AIP type 2 and a relevant proportion of patients was reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis.The risk of relapse is low but not irrelevant.展开更多
Autoimmune pancreatitis, a recently recognized type of chronic pancreatitis, is not rare in Japan, but reports of it elsewhere are relatively uncommon. We report the first preoperatively diagnosed case of autoimmune p...Autoimmune pancreatitis, a recently recognized type of chronic pancreatitis, is not rare in Japan, but reports of it elsewhere are relatively uncommon. We report the first preoperatively diagnosed case of autoimmune pancreatitis in Hungary, which responded well to steroid treatment and provided radiographic and functional evidence of this improvement. A 62-year-old female presented with a 4-month history of recurrent epigastric pain and a 5-kg weight loss. The oral glucose tolerance test (OGTT) indicated diabetes mellitus and the result of the fecal elastase test was abnormal. Ultrasonography (US) and the CT scan demonstrated a diffusely enlarged pancreas, and endoscopic retrograde cholangiopancreatography (ERCP) an irregular main pancreatic duct with long strictures in the head and tail. Autoimmune pancreatitis was diagnosed. The patient was started on 32 mg prednisolone daily, After 4 wk, the OGTT and faecal elastase test results had normalized. The repeated US and CT scan revealed a marked improvement of the diffuse pancreatic swelling, while on repeated ERCP, the main pancreatic duct narrowing was seen to be ameliorated. It is important to be aware of this disease and its diagnosis, because AIP can clinically resemble pancreatobiliary malignancies, or chronic or acute pancreatitis, However, in contrast with chronic pancreatitis, its symptoms and morphologic and laboratory alterations are completely reversed by oral steroid therapy.展开更多
Autoimmune pancreatitis is a distinct form of chronic pancreatitis. It is a chronic pancreatitis distinct from alcohol, genetic or idiopathic impairment and involves possible autoimmune mechanisms. The diagnosis of au...Autoimmune pancreatitis is a distinct form of chronic pancreatitis. It is a chronic pancreatitis distinct from alcohol, genetic or idiopathic impairment and involves possible autoimmune mechanisms. The diagnosis of autoimmune pancreatitis is based on a body of biological, histological and radiological arguments. Also called sclerosing lymphoplasmacytic pancreatitis, it is one of the causes of chronic pancreatitis. The risk factor for the onset of cancer that it represents and its sensitivity to corticosteroids make it a pathology requiring special management. We report a case of autoimmune pancreatitis in a 35-year-old patient.展开更多
Diagnostic criteria for autoimmune pancreatitis (AIP) have been proposed and used clinically because, despite its unique clinicopathological features, AIP does not have disease-specific serological tests for confirm...Diagnostic criteria for autoimmune pancreatitis (AIP) have been proposed and used clinically because, despite its unique clinicopathological features, AIP does not have disease-specific serological tests for confirmation. However, diagnosis of a patient with pancreatic lesions mimicking cancer who deviates from these diagnostic criteria is still difficult. We present herein a patient with a variant form of AIP successfully diagnosed by fine-needle biopsy, whose response to steroid therapy was excellent. A 55-year-old Japanese man was admitted to hospital because of jaundice and pancreatic head mass. AIP was considered as one of the differential diagnoses; however, as the patient showed neither pancreatic duct narrowing nor immunological abnormalities, he did not meet the Japanese diagnostic criteria for AIP. Histopathology of the pancreatic mass demonstrated abundant infiltration by lymphocytes and interstitial fibrosis, which suggested AIP. Immunoreaction to IgG4, which is supposed to be specific to AIP, was not observed; however, response to subsequent prednisolone therapy was good, with dramatic pancreatic head mass regression. Aside from the pancreatic head mass, diffusely spreading small lesions were observed throughout the liver. The likelihood of a potential association with extrapancreatic lesions of AIP was considered and led us to carry out a liver biopsy, which revealed biliary hamartoma, also called yon Meyenburg complex (VMC). As IgG4-positive plasma cell infiltration was not demonstrated in the hamartomatous regions, the hepatic condition was thought to have occurred incidentally; however, to the best of our knowledge, this is the first report in which the association between AIP and VMC was investigated and discussed.展开更多
Interleukin-35(IL-35)is a novel protein comprising IL-12αand IL-27βchains.The IL12A and EBI3 genes are responsible for its production.The study of IL-35 has experienced a substantial increase in interest in recent y...Interleukin-35(IL-35)is a novel protein comprising IL-12αand IL-27βchains.The IL12A and EBI3 genes are responsible for its production.The study of IL-35 has experienced a substantial increase in interest in recent years,as demonstrated by many research papers.Recent clinical studies have shown that individuals who do not have a C-peptide have notably reduced amounts of IL-35 in their blood serum.This is accompanied by a drop in the percentage of IL-35+Treg cells,regulatory B cells,and CD8+FOXP3+cells that produce IL-35.This article em-phasizes the potential significance of IL-35 expression in governing the immune response and its involvement in chronic inflammatory autoimmune diabetes in pancreatic inflammation.It demonstrates IL-35's ability to regulate cytokine proportions,modulate B cells,and protect against autoimmune diabetes.However,further investigation is necessary to ascertain the precise mechanism of IL-35,and meticulous planning is essential for clinical studies.展开更多
AIM: To identify the radiological characteristics of focal autoimmune pancreatitis (f-AIP) useful for differentiation from pancreatic cancer (PC). METHODS: Magnetic resonance imaging (MRI) and triple-phase computed to...AIM: To identify the radiological characteristics of focal autoimmune pancreatitis (f-AIP) useful for differentiation from pancreatic cancer (PC). METHODS: Magnetic resonance imaging (MRI) and triple-phase computed tomography (CT) scans of 79 patients (19 with f-AIP, 30 with PC, and 30 with a normal pancreas) were evaluated retrospectively. A radiologist measured the CT attenuation of the pancreatic parenchyma, the f-AIP and PC lesions in triple phases. The mean CT attenuation values of the f-AIP lesions were compared with those of PC, and the mean CT attenuation values of pancreatic parenchyma in the three groups were compared. The diagnostic performance of CT attenuation changes from arterial phase to hepatic phase in the differentiation between f-AIP and PC was evaluated using receiver operating characteristic (ROC) curve analysis. We also investigated the incidence of previously reported radiological findings for differentiation between f-AIP and PC. RESULTS: The mean CT attenuation values of f-AIP lesions in enhanced phases were significantly higher than those of PC (arterial phase: 60 ± 7 vs 48 ± 10, P < 0.05; pancreatic phase: 85 ± 6 vs 63 ± 15, P < 0.05; hepatic phase: 95 ± 7 vs 63 ± 13, P < 0.05). The mean CT attenuation values of f-AIP lesions were significantly lower those of uninvolved pancreas and normal pancreas in the arterial and pancreatic phase of CT (P < 0.001, P < 0.001), with no significant difference at the hepatic phase or unenhanced scanning (P = 0.4, P = 0.1). When the attenuation value increase was equal or more than 28 HU this was considered diagnostic for f-AIP, and a sensitivity of 87.5%, specificity of 100% and an area under the ROC curve of 0.974 (95%CI: 0.928-1.021) were achieved. Five findings were more frequently observed in f-AIP patients: (1) sausageshaped enlargement; (2) delayed homogeneous enhancement; (3) hypoattenuating capsule-like rim; (4) irregular narrowing of the main pancreatic duct (MPD) and/or stricture of the common bile duct (CBD); and (5) MPD upstream dilation ≤ 5 mm. CONCLUSION: Analysis of a combination of CT and MRI findings could improve the diagnostic accuracy of differentiating f-AIP from PC.展开更多
基金from CAMS Innovation Fund for Medical Sciences(CIFMS)(2021-I2M-1-002)National Key Clinical Specialty Construction Project(ZK108000)+1 种基金National High-Level Hospital Clinical Research Funding(2022-PUMCH-B-024)National Natural Science Foundation of China,Joint Fund Project(U20A600).
文摘Background:A high prevalence of diabetes mellitus(DM)coexisting with autoimmune pancreatitis(AIP)is observed.However,evidence on the circumstances under which corticosteroid therapy(CST)for AIP improves or worsens DM is scarce.This study aimed to demonstrate and identify predictors of DM control under the influence of CST.Methods:Patients diagnosed with type 1 AIP were enrolled from a prospectively maintained cohort and were classified into three groups according to the chronology in which AIP and DM were diagnosed:pre-existing DM(pDM),concurrent DM(cDM),and non-DM(nDM).The responses of DM to CST were assessed when corticosteroid was ceased or tapered to a maintenance dose and classified as‘improvement’and‘non-improvement’(including‘no change’and‘exacerbation’).Results:Among 101 patients with type 1 AIP,52(51.5%)patients were complicated with DM at the time of AIP diagnosis,with 36 patients in the cDM group and 16 patients in the pDM group.The incidences of diffuse pancreatic swelling(72.2%)and pancreatic body/tail involvement(91.7%)were significantly higher in the cDM group than in both the pDM and nDM groups.Of the 52 patients with DM,CST was administered in 48 cases.Multivariate logistic analysis identified that elevated serum gamma-glutamyl transferase(GGT)level at AIP diagnosis[odds ratio(OR)=0.032,95%confidence interval(CI):0.003-0.412,P=0.008]and pancreatic atrophy after CST(OR=0.027,95%CI:0.003-0.295,P=0.003)were negatively associated with DM control improvement.Conclusions:Patients with diffuse pancreatic swelling and pancreatic body/tail involvement in pancreatitis tended to be complicated with cDM at AIP diagnosis.CST exerted a beneficial effect on the clinical course of DM in nearly half of the AIP patients complicated with DM at diagnosis,particularly in those without elevated serum GGT levels at diagnosis and who did not experience pancreatic atrophy after CST.
基金Supported by Sichuan Science and Technology Program,China,No.MZGC20230031.
文摘BACKGROUND Autoimmune pancreatitis(AIP)is a chronic form of pancreatitis characterized by diffused enlargement of the pancreas and irregular stenosis of the main pancreatic duct.Some studies have reported that AIP can cause hemorrhage of gastric varices(GV)related to portal hypertension(PH).However,such cases are rare.In addition,the association of PH with AIP is unclear.At the same time,the efficacy and duration of glucocorticoid therapy is also controversial.CASE SUMMARY In this case,we reported a case of GV in pancreatic PH associated with AIP.Enhanced abdominal computed tomography(CT)suggested splenic vein(SV)and superior mesenteric vein(SMV)thromboses.The patient received a long-term glucocorticoid therapy,that the initial dose of 40 mg is reduced weekly by 5 mg,and then reduced to 5 mg for long-term maintenance.CT and gastroscopic examination after 8 mo of treatment indicated that SV and SMV were recanalized,pancreatic stiffness and swelling were ameliorated,and the GV almost completely disappeared.CONCLUSION Long-term glucocorticoid therapy can alleviate the development of GV in patients with AIP and has potential reversibility.
文摘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.
文摘In this editorial we comment on the article by Jaber et al.Autoimmune pancreatitis(AIP)represents a distinct form of pancreatitis,categorized into AIP-1 and AIP-2,characterized by obstructive jaundice,lymphoplasmacytic infiltrate,and fibrosis.AIP-1,associated with elevated immunoglobulin G4(IgG4)levels,exhibits higher relapse rates,affecting older males,while AIP-2 is less common and linked to inflammatory bowel disease.AIP is considered a manifestation of IgG4-related systemic disease,sharing characteristic histological findings.Steroids are the primary treatment,with emerging biomarkers like interferon alpha and inter-leukin-33.AIP poses an increased risk of various malignancies,and the assoc-iation with pancreatic cancer is debated.Surgery is reserved for severe cases,necessitating careful evaluation due to diagnostic challenges.AIP patients may have concurrent PanINs but display favorable long-term outcomes compared to pancreatic cancer patients.Thorough diagnostic assessment,including biopsy and steroid response,is crucial for informed surgical decisions in AIP.
文摘We are writing in response to the paper published in the World Journal of Gastroenterology by Zhou et al.The authors identified higher serum immunoglobulin(Ig)G4 levels and age over 55 years as independent risk factors for disease relapse.Despite notable strengths,it is crucial to address potential biases.Firstly,the cohort study included 189 patients with autoimmune pancreatitis(AIP)type 1(with higher IgG4 seropositivity and higher relapse)and 24 with type 2(with lower IgG4 seropositivity and lower relapse).Consequently,most,if not all,AIP type 2 patients were assigned to the normal group,possibly inflating the association of higher serum IgG4 levels with relapse and potentially exaggerating the association of older age with relapse.Secondly,the authors did not provide sufficient details regarding AIP diagnosis,such as the ratio of definitive vs probable cases and the proportion of biopsies.In cases where histological evidence is unavailable or indeterminate,AIP type 2 may be misdiagnosed as definitive type 1,and type 1 may also be misdiagnosed as probable type 2,particularly in cases with normal or mildly elevated serum IgG4 levels.Lastly,in this retrospective study,approximately one-third of the consecutive patients initially collected were excluded for various reasons.Accordingly,the impact of nonrandom exclusion on relapse outcomes should be carefully considered.In conclusion,the paper by Zhou et al offers plausible,though not entirely compelling,evidence suggesting a predictive role of elevated serum IgG4 levels and advanced age in AIP relapse.The foundation for future investigations lies in ensuring a reliable diagnosis and accurate disease subtyping,heavily dependent on obtaining histological specimens.In this regard,endoscopic ultrasound-guided fine-needle biopsy emerges as a pivotal component of the diagnostic process,contributing to mitigating biases in future explorations of the disease.
文摘Autoimmune pancreatitis(AIP)is a rare chronic autoimmune disorder.The diagnosis of AIP mainly depends on histopathology,imaging and response to treatment.Serum immunoglobulin 4(IgG4)is used only as collateral evidence in diagnostic criteria for AIP because of its moderate sensitivity.Serum IgG4 levels are normal in 15%-37%of type 1 AIP and most of type 2 AIP patients.In these patients,the indeterminate imaging and histopathology may lead to the difficulty in definitive diagnosis of AIP.Therefore,discovery of new biomarkers is impor-tant for AIP diagnosis.Here,we provide some views on the progression and challenges in identifying novel serological biomarkers in AIP diagnosis.
文摘BACKGROUND Autoimmune pancreatitis(AIP)is a rare form of autoimmune-mediated pancrea-titis,which is easily misdiagnosed as pancreatic cancer and thus treated surgi-cally.We studied the diagnosis and treatment of a patient with type 1 AIP recent-ly admitted to our hospital,and reviewed the literature to provide a reference for clinical diagnosis of AIP.CASE SUMMARY The chief complaint was yellowing of the body,eyes and urine for 21 d.The pa-tient's clinical presentation was obstructive jaundice and imaging suggested pan-creatic swelling.It was difficult to distinguish between inflammation and tumor.Serum immunoglobulin G4(IgG4)was markedly elevated.IgG4 is an important serological marker for type 1 AIP.The patient was diagnosed with AIP,IgG4-related cholangitis,acute cholecystitis and hepatic impairment.After applying hormonal therapy,the patient's symptoms improved significantly.At the same time,imaging suggested that pancreatic swelling subsided,and liver function and other biochemical indicators decreased.The treatment was effective.CONCLUSION In patients with pancreatic swelling,the possibility of AIP should be considered.
基金Young Scholar Independent Innovation Science Fund of Chinese PLA General Hospital,No.22QNCZ020National Key Research and Development Program,No.2022YFC2504003.
文摘BACKGROUND Autoimmune pancreatitis(AIP)has been linked with elevated immunoglobulin(Ig)G4 levels.The characteristics and outcomes of AIP based on serum markers have not been fully evaluated.AIM To compare clinical features,treatment efficacy,and outcome of AIP based on serum IgG4 levels and analyze predictors of relapse.METHODS A total of 213 patients with AIP were consecutively reviewed in our hospital from 2006 to 2021.According to the serum IgG4 level,all patients were divided into two groups,the abnormal group(n=148)with a high level of IgG4[>2×upper limit of normal(ULN)]and the normal group(n=65).The t-test or Mann-Whitney U test was used to compare continuous variables.Categorical parameters were compared by theχ^(2) test or Fisher’s exact test.Kaplan-Meier curves Zhou GZ et al.Clinical characteristics and outcome of AIP WJG https://www.wjgnet.com 5126 September 21,2023 Volume 29 Issue 35 and log-rank tests were established to assess the cumulative relapse rates.Univariate and multivariate analyses were used to investigate potential risk factors of AIP relapse.RESULTS Compared with the normal group,the abnormal group had a higher average male age(60.3±10.4 vs 56.5±12.9 years,P=0.047);higher level of serum total protein(72.5±7.9 g/L vs 67.2±7.5 g/L,P<0.001),IgG4(1420.5±1110.9 mg/dL vs 252.7±106.6 mg/dL,P<0.001),and IgE(635.6±958.1 IU/mL vs 231.7±352.5 IU/mL,P=0.002);and a lower level of serum complement C3(100.6±36.2 mg/dL vs 119.0±45.7 mg/dL,P=0.050).In addition,a lower number of cases with abnormal pancreatic duct and pancreatic atrophy(23.6%vs 37.9%,P=0.045;1.6%vs 8.6%,P=0.020,respectively)and a higher rate of relapse(17.6%vs 6.2%,P=0.030)were seen in the abnormal group.Multivariate analyses revealed that serum IgG4[(>2×ULN),hazard ratio(HR):3.583;95%confidence interval(CI):1.218–10.545;P=0.020]and IgA(>1×ULN;HR:5.908;95%CI:1.199–29.120;P=0.029)and age>55 years(HR:2.383;95%CI:1.056–5.378;P=0.036)were independent risk factors of relapse.CONCLUSION AIP patients with high IgG4 levels have clinical features including a more active immune system and higher relapse rate.Several factors,such as IgG4 and IgA,are associated with relapse.
文摘Autoimmune pancreatitis(AIP),a chronic inflammation caused by the immune system attacking the pancreas,usually presents imaging and clinical features that overlap with those of pancreatic ductal adenocarcinoma(PDAC).Serum biomarkers,substances that quantitatively change in sera during disease development,are a promising non-invasive tool with high utility for differentiating between these diseases.In this way,the presence of AIP is currently suspected when serum concentrations of immunoglobulin G4(IgG4)antibody are elevated.However,this approach has some drawbacks.Notably,IgG4 antibody concentrations are also elevated in sera from some patients with PDAC.This review focuses on the most recent and relevant serum biomarkers proposed to differentiate between AIP and PDAC,evaluating the usefulness of immunoglobulins,autoantibodies,chemokines,and cytokines.The proposed serum biomarkers have proven useful,although most studies had a small sample size,did not examine their presence in patients with PDAC,or did not test them in humans.In addition,current evidence suggests that a single serum biomarker is unlikely to accurately differentiate these diseases and that a set of biomarkers will be needed to achieve adequate specificity and sensitivity,either alone or in combination with clinical data and/or radiological images.
文摘Autoimmune pancreatitis(AIP)is a complex,poorly understood disease gaining increasing attention."Clinical Characteristics and Outcome of AIP Based on Serum IgG4 levels,"investigated AIP with a focus on serum immunoglobulin(Ig)G4 levels.The 213 patients with AIP were classified according to serum IgG4 levels:Abnormal(elevated)and normal.Patients with higher IgG4 levels exhibited a more active immune system and increased relapse rates.Beyond IgG4,the IgA levels and age independently contributed to relapse risk,guiding risk assessment and tailored treatments for better outcomes.However,limitations persist,such as no IgA correlation with IgG4 levels,absent data on autoantibodypositive AIP cases critical for Asian diagnostic criteria,and unexplored relapse rates in high serum IgG AIP by subtype.Genetic factors and family histories were not addressed.As the understanding and referral of seronegative AIPs increase,there's a growing need for commercially available,highly sensitive,and specific autoantibodies to aid in diagnosing individuals with low or absent serum IgG4 levels.
文摘Pancreatic ductal adenocarcinoma(PDAC)is one of the most lethal malignancies and is developing into the 2nd leading cause of cancer-related death.Often,the clinical and radiological presentation of PDAC may be mirrored by other inflammatory pancreatic masses,such as autoimmune pancreatitis(AIP)and massforming chronic pancreatitis(MFCP),making its diagnosis challenging.Differentiating AIP and MFCP from PDAC is vital due to significant therapeutic and prognostic implications.Current diagnostic criteria and tools allow the precise differentiation of benign from malignant masses;however,the diagnostic accuracy is imperfect.Major pancreatic resections have been performed in AIP cases under initial suspicion of PDAC after a diagnostic approach failed to provide an accurate diagnosis.It is not unusual that after a thorough diagnostic evaluation,the clinician is confronted with a pancreatic mass with uncertain diagnosis.In those cases,a re-evaluation must be entertained,preferably by an experienced multispecialty team including radiologists,pathologists,gastroenterologists,and surgeons,looking for disease-specific clinical,imaging,and histological hallmarks or collateral evidence that could favor a specific diagnosis.Our aim is to describe current diagnostic limitations that hinder our ability to reach an accurate diagnosis among AIP,PDAC,and MFCP and to highlight those disease-specific clinical,radiological,serological,and histological characteristics that could support the presence of any of these three disorders when facing a pancreatic mass with uncertain diagnosis after an initial diagnostic approach has been unsuccessful.
文摘Autoimmune pancreatitis(AIP)is a rare disease clinically characterized by obstructive jaundice,unintentional weight loss,acute pancreatitis,focal pancreatic mass,and diabetes.AIP is classified into two subtypes-type 1 and type 2-according to pathological findings,clinical features,and serology test results,but some cases may be defined as type not otherwise in the absence of pathological findings and inflammatory bowel disease.To address the differences in diagnostic criteria by country,standard diagnostic criteria for AIP were proposed in 2011 by an international consensus of expert opinions.Differential diagnosis of AIP from pancreatic ductal adenocarcinoma is important but remains challenging for clinicians.Fortunately,all subtypes of AIP show dramatic response to steroid treatment.This review discusses the current perspectives on the diagnosis and management of AIP in clinical practice.
基金Supported by National Natural Science Foundation of China,No. 81170453
文摘AIM:To explore the optimal steroid therapeutic strategy for autoimmune pancreatitis(AIP).METHODS:This study was conducted retrospectively in two large institutions in China.Patients with clinically,radiologically and biochemically diagnosed AIP were enrolled.The performed radiological investigations and biochemical tests,the regimen of the given steroid treatment,remission and relapse whether with and without steroid therapy were analyzed.RESULTS:Twenty-eight patients with AIP received steroid treatment,while 40 patients were treated surgically by pancreatoduodenectomy,distal pancreatectomy and choledochojejunostomy,radiofrequency ablation for the enlarged pancreatic head,percutaneous transhepatic biliary drainage and endoscopic biliary drainage.The starting oral prednisolone dose was 30 mg/d in 18(64.3%) patients and 40 mg/d in 10(35.7%) patients administered for 3 wk.The remission rate of AIP patients with steroid treatment(96.4%) was significantly higher than in those without steroid treatment(75%).Maintenance therapy(oral prednisolone dose 5 mg/d) was performed after remission for at least 6-12 mo to complete the treatment course.Similarly,the relapse rate was significantly lower in AIP patients with steroid treatment(28.6%) than in those without steroid treatment(42.5%).Steroid re-treatment was effective in all relapsed patients with or without steroid therapy.CONCLUSION:Steroid therapy should be considered in all patients with active inflammatory phase of AIP.However,the optimal regimen still should be trailed in larger numbers of patients with AIP.
基金Supported by Ministry of Health of the Czech Republic,No.NU20-03-00126Ministry of Health of the Czech Republic-conceptual development of research organization,No.FNBr,65269705,SUp 3/21.
文摘Helicobacter pylori(H.pylori)is an infectious agent influencing as much as 50%of the world’s population.It is the causative agent for several diseases,most especially gastric and duodenal peptic ulcer,gastric adenocarcinoma and mucosaassociated lymphoid tissue lymphoma of the stomach.A number of other,extragastric manifestations also are associated with H.pylori infection.These include neurological disorders,such as Alzheimer’s disease,demyelinating multiple sclerosis and Parkinson’s disease.There is also evidence for a relationship between H.pylori infection and such dermatological diseases as psoriasis and rosacea as well as a connection with infection and open-angle glaucoma.Generally little is known about the relationship between H.pylori infection and diseases of the pancreas.Most evidence about H.pylori and its potential role in the development of pancreatic diseases concerns pancreatic adenocarcinoma and autoimmune forms of chronic pancreatitis.There is data(albeit not fully consistent)indicating modestly increased pancreatic cancer risk in H.pylori-positive patients.The pathogenetic mechanism of this increase is not yet fully elucidated,but several theories have been proposed.Reduction of antral Dcells in H.pylori-positive patients causes a suppression of somatostatin secretion that,in turn,stimulates increased secretin secretion.That stimulates pancreatic growth and thus increases the risk of carcinogenesis.Alternatively,H.pylori,as a part of microbiome dysbiosis and the so-called oncobiome,is proven to be associated with pancreatic adenocarcinoma development via the promotion of cellular proliferation.The role of H.pylori in the inflammation characteristic of autoimmune pancreatitis seems to be explained by a mechanism of molecular mimicry among several proteins(mostly enzymes)of H.pylori and pancreatic tissue.Patients with autoimmune pancreatitis often show positivity for antibodies against H.pylori proteins.H.pylori,as a part of microbiome dysbiosis,also is viewed as a potential trigger of autoimmune inflammation of the pancreas.It is precisely these relationships(and associated equivocal conclusions)that constitute a center of attention among pancreatologists,immunologists and pathologists.In order to obtain clear and valid results,more studies on sufficiently large cohorts of patients are needed.The topic is itself sufficiently significant to draw the interest of clinicians and inspire further systematic research.Next-generation sequencing could play an important role in investigating the microbiome as a potential diagnostic and prognostic biomarker for pancreatic cancer.
文摘Background:Autoimmune pancreatitis(AIP)is a well-recognized fibroinflammatory disease of the pancreas.Despite the significant number of studies published on AIP type 1 and 2,no studies have been focused on AIP type not otherwise specified(NOS)and therefore very little is known about clinical features and long-term outcomes of these patients.The aim of this study was to investigate clinical and radiological features of AIP type NOS-patients.Methods:Patients classified as AIP type NOS at clinical onset included in our database prospectively maintained since 1995 were evaluated.Epidemiological,clinical data were collected and analyzed.Results:Forty-six patients were included in the study.The clinical onset was mainly characterized by weight loss,jaundice and acute pancreatitis.Eight patients(17.4%)were reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis.Seven patients(15.2%)experienced relapse after steroid treatment but only one(2.2%)needed immunosuppressive drugs because of recurrent relapses.Conclusions:AIP type NOS shares clinical features similar to AIP type 2 and a relevant proportion of patients was reclassified as AIP type 2 during follow-up because of the development of ulcerative colitis.The risk of relapse is low but not irrelevant.
基金Supported by ETT (5 K.503), OTKA (5 K507) and the Hungarian Academy of Sciences (BO 5/2003)
文摘Autoimmune pancreatitis, a recently recognized type of chronic pancreatitis, is not rare in Japan, but reports of it elsewhere are relatively uncommon. We report the first preoperatively diagnosed case of autoimmune pancreatitis in Hungary, which responded well to steroid treatment and provided radiographic and functional evidence of this improvement. A 62-year-old female presented with a 4-month history of recurrent epigastric pain and a 5-kg weight loss. The oral glucose tolerance test (OGTT) indicated diabetes mellitus and the result of the fecal elastase test was abnormal. Ultrasonography (US) and the CT scan demonstrated a diffusely enlarged pancreas, and endoscopic retrograde cholangiopancreatography (ERCP) an irregular main pancreatic duct with long strictures in the head and tail. Autoimmune pancreatitis was diagnosed. The patient was started on 32 mg prednisolone daily, After 4 wk, the OGTT and faecal elastase test results had normalized. The repeated US and CT scan revealed a marked improvement of the diffuse pancreatic swelling, while on repeated ERCP, the main pancreatic duct narrowing was seen to be ameliorated. It is important to be aware of this disease and its diagnosis, because AIP can clinically resemble pancreatobiliary malignancies, or chronic or acute pancreatitis, However, in contrast with chronic pancreatitis, its symptoms and morphologic and laboratory alterations are completely reversed by oral steroid therapy.
文摘Autoimmune pancreatitis is a distinct form of chronic pancreatitis. It is a chronic pancreatitis distinct from alcohol, genetic or idiopathic impairment and involves possible autoimmune mechanisms. The diagnosis of autoimmune pancreatitis is based on a body of biological, histological and radiological arguments. Also called sclerosing lymphoplasmacytic pancreatitis, it is one of the causes of chronic pancreatitis. The risk factor for the onset of cancer that it represents and its sensitivity to corticosteroids make it a pathology requiring special management. We report a case of autoimmune pancreatitis in a 35-year-old patient.
文摘Diagnostic criteria for autoimmune pancreatitis (AIP) have been proposed and used clinically because, despite its unique clinicopathological features, AIP does not have disease-specific serological tests for confirmation. However, diagnosis of a patient with pancreatic lesions mimicking cancer who deviates from these diagnostic criteria is still difficult. We present herein a patient with a variant form of AIP successfully diagnosed by fine-needle biopsy, whose response to steroid therapy was excellent. A 55-year-old Japanese man was admitted to hospital because of jaundice and pancreatic head mass. AIP was considered as one of the differential diagnoses; however, as the patient showed neither pancreatic duct narrowing nor immunological abnormalities, he did not meet the Japanese diagnostic criteria for AIP. Histopathology of the pancreatic mass demonstrated abundant infiltration by lymphocytes and interstitial fibrosis, which suggested AIP. Immunoreaction to IgG4, which is supposed to be specific to AIP, was not observed; however, response to subsequent prednisolone therapy was good, with dramatic pancreatic head mass regression. Aside from the pancreatic head mass, diffusely spreading small lesions were observed throughout the liver. The likelihood of a potential association with extrapancreatic lesions of AIP was considered and led us to carry out a liver biopsy, which revealed biliary hamartoma, also called yon Meyenburg complex (VMC). As IgG4-positive plasma cell infiltration was not demonstrated in the hamartomatous regions, the hepatic condition was thought to have occurred incidentally; however, to the best of our knowledge, this is the first report in which the association between AIP and VMC was investigated and discussed.
文摘Interleukin-35(IL-35)is a novel protein comprising IL-12αand IL-27βchains.The IL12A and EBI3 genes are responsible for its production.The study of IL-35 has experienced a substantial increase in interest in recent years,as demonstrated by many research papers.Recent clinical studies have shown that individuals who do not have a C-peptide have notably reduced amounts of IL-35 in their blood serum.This is accompanied by a drop in the percentage of IL-35+Treg cells,regulatory B cells,and CD8+FOXP3+cells that produce IL-35.This article em-phasizes the potential significance of IL-35 expression in governing the immune response and its involvement in chronic inflammatory autoimmune diabetes in pancreatic inflammation.It demonstrates IL-35's ability to regulate cytokine proportions,modulate B cells,and protect against autoimmune diabetes.However,further investigation is necessary to ascertain the precise mechanism of IL-35,and meticulous planning is essential for clinical studies.
基金Supported by National Nature Science Foundation of China No.30970801National Nature Science Foundation of China,No.81170435+1 种基金the China Post-doctoral Science Foundation,No.20100480545the Shanghai Leading Talent Team Construction Special Funds,No.2011-036
文摘AIM: To identify the radiological characteristics of focal autoimmune pancreatitis (f-AIP) useful for differentiation from pancreatic cancer (PC). METHODS: Magnetic resonance imaging (MRI) and triple-phase computed tomography (CT) scans of 79 patients (19 with f-AIP, 30 with PC, and 30 with a normal pancreas) were evaluated retrospectively. A radiologist measured the CT attenuation of the pancreatic parenchyma, the f-AIP and PC lesions in triple phases. The mean CT attenuation values of the f-AIP lesions were compared with those of PC, and the mean CT attenuation values of pancreatic parenchyma in the three groups were compared. The diagnostic performance of CT attenuation changes from arterial phase to hepatic phase in the differentiation between f-AIP and PC was evaluated using receiver operating characteristic (ROC) curve analysis. We also investigated the incidence of previously reported radiological findings for differentiation between f-AIP and PC. RESULTS: The mean CT attenuation values of f-AIP lesions in enhanced phases were significantly higher than those of PC (arterial phase: 60 ± 7 vs 48 ± 10, P < 0.05; pancreatic phase: 85 ± 6 vs 63 ± 15, P < 0.05; hepatic phase: 95 ± 7 vs 63 ± 13, P < 0.05). The mean CT attenuation values of f-AIP lesions were significantly lower those of uninvolved pancreas and normal pancreas in the arterial and pancreatic phase of CT (P < 0.001, P < 0.001), with no significant difference at the hepatic phase or unenhanced scanning (P = 0.4, P = 0.1). When the attenuation value increase was equal or more than 28 HU this was considered diagnostic for f-AIP, and a sensitivity of 87.5%, specificity of 100% and an area under the ROC curve of 0.974 (95%CI: 0.928-1.021) were achieved. Five findings were more frequently observed in f-AIP patients: (1) sausageshaped enlargement; (2) delayed homogeneous enhancement; (3) hypoattenuating capsule-like rim; (4) irregular narrowing of the main pancreatic duct (MPD) and/or stricture of the common bile duct (CBD); and (5) MPD upstream dilation ≤ 5 mm. CONCLUSION: Analysis of a combination of CT and MRI findings could improve the diagnostic accuracy of differentiating f-AIP from PC.