Background and AimsAutoimmune hepatitis(AIH)frequently coexists with extrahepatic autoimmune diseases(EADs),but their prevalence,characteristics,progression,and treatment effect in the Han Chinese population remain un...Background and AimsAutoimmune hepatitis(AIH)frequently coexists with extrahepatic autoimmune diseases(EADs),but their prevalence,characteristics,progression,and treatment effect in the Han Chinese population remain unclear.This study aimed to evaluate the prevalence and spectrum of EADs and to assess their clinical features,disease course,and treatment outcomes in Han Chinese patients with AIH.MethodsMedical records of 371 Han Chinese patients with AIH(diagnosed from March 2016 to October 2023)were retrospectively analyzed.ResultsAmong the 371 AIH patients,304(81.94%)were female,with a median age of 52.5 years(interquartile range,46.0-61.0).A total of 23.98%(89/371)had at least one EAD,including 27.06%(82/303)in type 1 AIH,11.11%(7/63)in antibody-negative AIH,and none in type 2.A single EAD was the most common(20.21%,75/371).The most frequent EADs were Sjogren’s syndrome(8.63%)and autoimmune thyroid disease(8.36%).Compared with patients without EADs,those with EADs had lower alanine aminotransferase,red blood cell,and hemoglobin levels,but higher aspartate aminotransferase/alanine aminotransferase ratio and antinuclear antibody(ANA)positivity(all P<0.05).ANA positivity was independently associated with EADs(odds ratio=2.209,95%confidence interval=1.242-3.927,P=0.007).After three months of treatment,the complete biochemical response rate was lower in the EADs group than in the non-EADs group(40.0%vs.55.3%,P=0.024),whereas no significant differences were observed at 6,12,24,or 36 months(all P>0.05).ConclusionsIn the Han Chinese population,23.98%of AIH patients had EADs,with Sjogren’s syndrome and autoimmune thyroid disease being the most common.ANA positivity was a significant risk factor for EADs.EAD patients had a poorer initial treatment response at three months,but comparable long-term biochemical response from six months.展开更多
Background:In patients with autoimmune hemolytic anemia(AIHA),the risk of relapse is high owing to persistent autoreactive B-cell activity.Multirefractory AIHA is a more advanced stage of disease that is defined by a ...Background:In patients with autoimmune hemolytic anemia(AIHA),the risk of relapse is high owing to persistent autoreactive B-cell activity.Multirefractory AIHA is a more advanced stage of disease that is defined by a lack of response to at least three lines of therapy.CD19-directed chimeric antigen receptor(CAR)T-cell therapy results in profound B-cell depletion and may be a useful approach to achieving drug-free remission in multirefractory AIHA.展开更多
Autoimmune enteropathy(AIE)is a rare immune mediated disorder primarily affecting children,characterized by chronic diarrhea,malabsorption,vomiting,weight loss and villous atrophy.It has also been observed in adults p...Autoimmune enteropathy(AIE)is a rare immune mediated disorder primarily affecting children,characterized by chronic diarrhea,malabsorption,vomiting,weight loss and villous atrophy.It has also been observed in adults presenting diagnostic and treatment challenges due to its overlap with other gastrointestinal disorders such as celiac disease.Initial diagnostic criteria for AIE include small bowel villous atrophy,lack of response to dietary restrictions,presence of anti-enterocyte antibodies,and predisposition to autoimmunity without severe immu-nodeficiency.Refined criteria emphasize characteristic histological findings and exclusion of other causes of villous atrophy.AIE is associated with various autoimmune disorders and can present with overlapping features with Celiac disease,including villous atrophy but without significant intraepithelial lympho-cytosis.Treatment primarily involves immunosuppression using corticosteroids,calcineurin inhibitors,and anti-tumor necrosis factor therapy,alongside nutri-tional support.Despite the challenges,understanding AIE’s diverse manifest-ations and improving diagnostic criteria are essential for effective management and improved patient outcome.Further research is needed to elucidate the pathogenesis,disease progression and long-term outcomes of AIE.展开更多
Parkinson's disease is a neurodegenerative disorder marked by the degeneration of dopaminergic neurons and clinical symptoms such as tremors,rigidity,and slowed movements.A key feature of Parkinson's disease i...Parkinson's disease is a neurodegenerative disorder marked by the degeneration of dopaminergic neurons and clinical symptoms such as tremors,rigidity,and slowed movements.A key feature of Parkinson's disease is the accumulation of misfoldedα-synuclein,forming insoluble Lewy bodies in the substantia nigra pars compacta,which contributes to neurodegeneration.Theseα-synuclein aggregates may act as autoantigens,leading to T-cell-mediated neuroinflammation and contributing to dopaminergic cell death.Our perspective explores the hypothesis that Parkinson's disease may have an autoimmune component,highlighting research that connects peripheral immune responses with neurodegeneration.T cells derived from Parkinson's disease patients appear to have the potential to initiate an autoimmune response againstα-synuclein and its modified peptides,possibly leading to the formation of neo-epitopes.Recent evidence associates Parkinson's disease with abnormal immune responses,as indicated by increased levels of immune cells,such as CD4^(+)and CD8^(+)T cells,observed in both patients and mouse models.The convergence of T cells filtration increasing major histocompatibility complex molecules,and the susceptibility of dopaminergic neurons supports the hypothesis that Parkinson's disease may exhibit autoimmune characteristics.Understanding the immune mechanisms involved in Parkinson's disease will be crucial for developing therapeutic strategies that target the autoimmune aspects of the disease.Novel approaches,including precision medicine based on major histocompatibility complex/human leukocyte antigen typing and early biomarker identification,could pave the way for immune-based treatments aimed at slowing or halting disease progression.This perspective explores the relationship between autoimmunity and Parkinson's disease,suggesting that further research could deepen understanding and offer new therapeutic avenues.In this paper,it is organized to provide a comprehensive perspective on the autoimmune aspects of Parkinson's disease.It investigates critical areas such as the autoimmune response observed in Parkinson's disease patients and the role of autoimmune mechanisms targetingα-synuclein in Parkinson's disease.The paper also examines the impact of CD4~+T cells,specifically Th1 and Th17,on neurons through in vitro and ex vivo studies.Additionally,it explores howα-synuclein influences glia-induced neuroinflammation in Parkinson's disease.The discussion extends to the clinical implications and therapeutic landscape,offering insights into potential treatments.Consequently,we aim to provide a comprehensive perspective on the autoimmune aspects of Parkinson's disease,incorporating both supportive and opposing views on its classification as an autoimmune disorder and exploring implications for clinical applications.展开更多
OBJECTIVE:To elucidate the therapeutic efficacy and mechanism of action of Chaihu Guizhi Ganjiang decoction(柴胡桂枝干姜汤,CGGD)in autoimmune hepatitis.METHODS:CGGD components and potential target genes were extracted...OBJECTIVE:To elucidate the therapeutic efficacy and mechanism of action of Chaihu Guizhi Ganjiang decoction(柴胡桂枝干姜汤,CGGD)in autoimmune hepatitis.METHODS:CGGD components and potential target genes were extracted from previously published databases.The autoimmune hepatitis(AIH)-related regulatory genes were obtained from the Dis Ge NET database.Intersections were taken,and enrichment analyses were performed on the extracted data.Concanavalin A(Con A)-induced AIH model mice were treated with CGGD via gavage.The results of network pharmacological analysis were experimentally validated.RESULTS:Network pharmacology revealed 228 genes at the intersection of AIH and CGGD.Kyoto Encyclopedia of Genes and Genomes analysis revealed that CGGD primarily regulates the phosphoinositide 3-kinase(PI3K)/protein kinase B(AKT)signaling pathway and cellular metabolism in AIH.Gene Ontology enrichment analysis revealed that CGGD modulates inflammation through transcription factor-mediated signaling pathways.As predicted,CGGD attenuated Con A-induced AIH in a dose-dependent manner by activating the PI3K/AKT signaling pathway.Histopathological assessment confirmed the protective effects of CGGD against Con Ainduced AIH.Further investigation revealed that CGGD regulated the T helper cell 17(Th17)/regulatory T cell(Treg)balance by modulating the PI3K/Akt/nuclear factor kappa-B(NF-κB)pathway.CONCLUSIONS:This study demonstrated the therapeutic effect of CGGD on AIH through a combination of network pharmacological prediction and experimental validation.Its mechanism of action involves PI3K/Akt/NF-κB-mediated regulation of Th17/Treg cells.展开更多
This letter addresses the study by Jayabalan et al,which underscores the liver outcome score(LOS)and hemoglobin(Hb)as key prognostic markers for patients with autoimmune liver disease overlap syndromes(AILDOS),with pa...This letter addresses the study by Jayabalan et al,which underscores the liver outcome score(LOS)and hemoglobin(Hb)as key prognostic markers for patients with autoimmune liver disease overlap syndromes(AILDOS),with particular relevance to the autoimmune hepatitis-primary biliary cholangitis(AIH-PBC)subgroup.The findings indicate that an LOS threshold of 6 achieves high sensitivity and specificity in predicting liver-related mortality among AIH-PBC patients.Moreover,low Hb levels emerge as a significant mortality predictor across all AILDOS cases.These results contribute valuable perspectives on risk stratification in AILDOS,highlighting the promise of non-invasive prognostic tools.Future studies with larger cohorts are needed to substantiate LOS and Hb as robust markers for clinical application.展开更多
Autoimmune liver disease overlap syndrome(OS)is a rare and clinically significant condition that has received limited attention in microbiome research.In their recent study,Wang et al combined 16S rRNA sequencing with...Autoimmune liver disease overlap syndrome(OS)is a rare and clinically significant condition that has received limited attention in microbiome research.In their recent study,Wang et al combined 16S rRNA sequencing with untargeted metabolomics to characterize the gut-liver axis in OS,identifying shared features of dysbiosis in autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC),and unique signatures,including enrichment of Klebsiella and Escherichia and depletion of aromatic amino acids.In this letter,we critically appraise these findings,emphasizing that OS should be considered a distinct immunometabolic phenotype rather than a simple mixture of AIH and PBC.We discuss the potential mechanistic relevance of the Fusicatenibacter-tyrosine relationship,highlight the clinical implications of integrating microbiota-metabolite analyses,and outline the limitations that future studies must address.展开更多
Recently,Jayabalan et al published an important study.The authors defined the liver outcome score as a novel biomarker for predicting liver-related mortality in patients with autoimmune hepatitis-primary biliary chola...Recently,Jayabalan et al published an important study.The authors defined the liver outcome score as a novel biomarker for predicting liver-related mortality in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome.After thoroughly reviewing their work,we offer insights that primarily relate to their study design to enhance the medical community’s understanding of this complex disease.展开更多
Autoimmune hepatitis(AIH)is a rare cause of chronic liver disease.The exact pa-thophysiology of AIH is unknown.Breakdown of self-tolerance against hepatic antigens and molecular mimicry are often implicated in the pat...Autoimmune hepatitis(AIH)is a rare cause of chronic liver disease.The exact pa-thophysiology of AIH is unknown.Breakdown of self-tolerance against hepatic antigens and molecular mimicry are often implicated in the pathogenesis of AIH.Immunosuppressive therapy is the mainstay of treatment;however,10%–25%of patients with AIH may not respond to primary therapy.Those patients are often salvaged with second-and third-line immunosuppressive therapy.Workup for other concomitant diseases should be done for patients who fail to respond to primary immunosuppressive therapy.Concurrent metabolic dysfunction-asso-ciated steatotic liver disease,alcohol-related liver disease,overlap syndrome(AIH with primary biliary cholangitis or sclerosing cholangitis),chronic hepatitis B virus,hepatitis C virus,and human immunodeficiency virus infection should be ruled out in such cases.Targeting the concomitant etiology may lead to resolution of the clinical symptoms and induce biochemical and histological remission.Isolated AIH without other etiologies for liver injury should be managed with a higher dose of steroids,azathioprine,or other immunosuppressive agents.Second-and third-line immunosuppressive agents include mycophenolate mofetil,cyclosporine,tacrolimus,infliximab,and rituximab.Patients with AIH may present with acute severe AIH(AS-AIH)and AIH-related acute on chronic liver failure,and they often require liver transplantation.The terms refractory or difficult-to-treat AIH have been used interchangeably and have no distinct definition.Difficult-to-treat AIH includes patients with intolerable side effects,fulminant disease(AIH with acute on chronic liver failure and AS-AIH),AIH in pregnancy,and HIV infection.Patients who fail to respond to standard first-line immunosuppressive therapy should be classified as refractory AIH.This review addresses the issues in the management of difficult-to-treat AIH with recent advances in pharmacological management.展开更多
BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High...BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High levels of anti-ganglionic nicotinic acetylcholine receptor(gAChR)serum antibodies are detected in approximately 50%of AAG cases,confirming the diagnosis.CASE SUMMARY We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period.Recently,the patient was unable to stand upright for more than a few seconds before fainting.Additionally,he presented with decreased sweating,dry mouth,urinary retention,early satiety,weight loss,bloating,constipation,and erectile dysfunction.Neurological examination revealed dilated pupils that were unresponsive to light.Deep tendon reflexes were absent or diminished.Serologic evaluation revealed the presence of gAChR autoantibodies.An orthostatic hypotension test yielded a positive result.The patient did not respond to symptomatic therapy,including midodrine,fludrocortisone and atomoxetine.Second-line therapy with immunoadsorption produced a noticeable clinical improvement;however,orthostatic hypotension persisted.Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.CONCLUSION Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.展开更多
Drug-induced autoimmune-like hepatitis(DI-ALH)is an increasingly recognized phenotype within the spectrum of drug-induced liver injury.Several drugs,including nitrofurantoin,minocycline,hydralazine,methyldopa and infl...Drug-induced autoimmune-like hepatitis(DI-ALH)is an increasingly recognized phenotype within the spectrum of drug-induced liver injury.Several drugs,including nitrofurantoin,minocycline,hydralazine,methyldopa and infliximab,have a well-documented capacity to induce DI-ALH.Distinguishing DI-ALH from classic de novo autoimmune hepatitis(AIH)can be challenging due to overlapping clinical,biochemical,and serological features.Accurate distinction from classic AIH is crucial,as management and prognosis differ.While some DIALH cases resolve spontaneously after drug withdrawal,others show persistent or worsening liver injury.Histological studies have shown that fibrosis and cirrhosis are more prevalent in classic AH.Unfortunately,there are no pathognomic clinical,biochemical or immunological features that reliably distinguish DI-ALH from classic AIH.However,most patients with DI-ALH do not relapse after corticosteroid withdrawal,in contrast to the high relapse rate observed in classic AIH.Most patients respond well to corticosteroids,and once liver tests normalize,biochemical parameters should be monitored,and long-term immunosuppression should not be indicated.However,DI-ALH is not exempt from risk of relapse,underscoring the need for long-term follow-up.Most patients with DIALH have a favorable prognosis;however,although rare,cases of cirrhosis and,in exceptional instances,acute liver failure have been reported.International collaborative studies are needed to further characterize DI-ALH.In this review,we update current controversies,present emerging concepts,and outline future challenges in the diagnosis and management of this complex condition learned so far.展开更多
BACKGROUND Autoimmune hepatitis(AIH)is typically treated with immunomodulators and steroids.However,some patients are refractory to these treatments,necessitating alternative approaches.Biological therapies have recen...BACKGROUND Autoimmune hepatitis(AIH)is typically treated with immunomodulators and steroids.However,some patients are refractory to these treatments,necessitating alternative approaches.Biological therapies have recently been explored for these difficult cases.AIM To assess the efficacy and safety of biologics in AIH,focusing on patients unresponsive to standard treatments and evaluating outcomes such as serological markers and histological remission.METHODS A case-based systematic review was performed following the PRISMA protocol to evaluate the efficacy and safety of biological therapies in AIH.The primary focus was on serological improvement and histological remission.The secondary focus was on assessing therapy safety and additional outcomes.A standardized search command was applied to MEDLINE,EMBASE,and Cochrane Library databases to identify relevant studies.Inclusion criteria encompassed adult AIH patients treated with biologics.Data were analyzed based on demographics,prior treatments,and therapy-related outcomes.A narrative synthesis was employed to address biases and provide a comprehensive overview of the evidence.RESULTS A total of 352 studies were reviewed,with 30 selected for detailed analysis.Key findings revealed that Belimumab led to a favourable response in five out of eight AIH patients across two studies.Rituximab demonstrated high efficacy,with 41 out of 45 patients showing significant improvement across six studies.Basiliximab was assessed in a single study,where the sole patient treated experienced a beneficial outcome.Additionally,a notable number of AIH cases were induced by anti-tumor necrosis factor(TNF)medications,including 16 cases associated with infliximab and four cases with adalimumab.All these cases showed improvement upon withdrawal of the biologic agent.CONCLUSION Belimumab and Rituximab show promise as effective alternatives for managing refractory AIH,demonstrating significant improvements in clinical outcomes and liver function.However,the variability in patient responses to different therapies highlights the need for personalized treatment strategies.The risk of AIH induced by anti-TNF therapies underscores the need for vigilant monitoring and prompt symptom recognition.These findings support the incorporation of biologic agents into AIH treatment protocols,particularly for patients who do not respond to conventional therapies.展开更多
Chloroquine(CQ)and hydroxychloroquine(HCQ),originally developed as anti-malarial drugs,have found a new purpose in treating various autoimmune dis-eases due to their immunomodulatory properties.These drugs work throug...Chloroquine(CQ)and hydroxychloroquine(HCQ),originally developed as anti-malarial drugs,have found a new purpose in treating various autoimmune dis-eases due to their immunomodulatory properties.These drugs work through mu-ltiple mechanisms,including inhibiting Toll-like receptor signaling,suppressing antigen presentation,and modulating autophagy.This review article provides a comprehensive analysis of the immunomodulatory effects of CQ and HCQ in several autoimmune diseases such as systemic lupus erythematosus,rheumatoid arthritis,systemic sclerosis,and others.We delve into the intricate mechanisms of action,highlighting the key immune cells involved and discussing the clinical implications of these drugs in managing autoimmune conditions.Our review covers the latest research and clinical trials,offering a comprehensive under-standing of the therapeutic potential of CQ and HCQ in autoimmune diseases.We also discuss the challenges and controversies surrounding the use of these drugs,such as their long-term side effects and the need for personalized treatment approaches.By synthesizing current knowledge and identifying areas for future research,this review aims to provide a valuable resource for healthcare profes-sionals and researchers involved in the management of autoimmune diseases.展开更多
Background:Previous studies have highlighted the frequent occurrence of sarcopenia in patients with pancreatic diseases,including chronic pancreatitis.We aimed to clarify the prevalence of skeletal muscle(SM)loss and ...Background:Previous studies have highlighted the frequent occurrence of sarcopenia in patients with pancreatic diseases,including chronic pancreatitis.We aimed to clarify the prevalence of skeletal muscle(SM)loss and sarcopenia,and their associations with clinical characteristics,bone mineral density,and pancreatic imaging findings in patients with autoimmune pancreatitis(AIP).Methods:This study included 114 patients with AIP treated at Tohoku University Hospital.The SM index was assessed using a bioelectrical impedance analysis device,grip strength was measured using a hand dynamometer,and bone mineral density was evaluated using dual-energy X-ray absorptiometry.Univari-ate and multivariate logistic regression analyses were used to analyze factors associated with SM loss and sarcopenia.Results:Among 114 patients,57(50.0%)had SM loss,31(27.2%)had reduced grip strength,and 27(23.7%)had both.Patients with SM loss were older and had a lower body mass index,weaker grip strength,higher Controlling Nutritional Status scores,and lower serum lipase and albumin levels compared to those without SM loss.Computed tomography scans revealed a higher prevalence of pancreatic parenchy-mal atrophy in patients with SM loss.Similar differences were observed between patients with sarcopenia and those without.Osteopathy was observed in 35.6%of patients with SM loss and 38.1%of those with sarcopenia,whereas only 4.1%of patients without SM loss had osteopathy.Low BMI(<21.0 kg/m^(2))was also found to be an independent risk factor for SM loss in multivariate analysis.Age>72 years,low BMI(<20.0 kg/m^(2)),and low serum lipase levels(<13 U/L)were independent risk factors for sarcopenia in multivariate analysis.Conclusions:SM loss and sarcopenia are prevalent in patients with AIP and are associated with aging,poor nutritional status,low serum lipase levels,and pancreatic parenchymal atrophy.In addition to the high risk of osteopathy,careful attention should be paid to maintain muscle health in AIP patients.展开更多
BACKGROUND Hepatitis D virus-hepatitis B virus(HDV-HBV)co-infection accelerates liver disease progression and increases the risk of hepatocellular carcinoma,but the immunopathogenic mechanism of its combination with a...BACKGROUND Hepatitis D virus-hepatitis B virus(HDV-HBV)co-infection accelerates liver disease progression and increases the risk of hepatocellular carcinoma,but the immunopathogenic mechanism of its combination with autoimmune hepatitis(AIH)has not been clarified.This study reveals for the first time that HDV may induce AIH through abnormalities in immunoregulation in two specific cases.This is the first report of HDV-HBV co-infected patients who did not receive interferon therapy and achieved serological conversion and histological remission by combining antiviral(entecavir)with immunosuppression(prednisone+azathioprine)therapy,providing new evidence of the mechanism of this complex disease.CASE SUMMARY A 40-year-old female developed malaise and jaundice with an alanine aminotransferase/aspartate aminotransferase>20 upper limit of normal(ULN),total bilirubin:97.20μmol/L,immunoglobulin G(IgG)47.1 g/L(>3×ULN),HDV RNA 1.6×10^(7)copies/mL and liver biopsy showed G3S4.Tenofovir alafenamide combined with prednisone and azathioprine was administered,and three months later the Child-Turcotte-Pugh class C was reduced to class B and IgG decreased to 13.62 g/L.Another 58-year-old male complained of pain in the liver area,antinuclear antibody was 1:320,IgG 22.6 g/L(>1.3×ULN),and liver biopsy showed G2S3.Entecavir was administered in combination with prednisone and azathioprine,and after 3 months,liver function returned to normal,and IgG reduced to 14.22 g/L.CONCLUSION Patients with HDV-HBV co-infection combined with AIH can achieve clinical remission following combination therapy,and the study of immunomodulatory mechanisms should be emphasized.展开更多
BACKGROUND The relationship between autoimmune gastritis(AIG)and gastric polyps(GPs)is not well understood.AIM To explore the clinical characteristics and risk factors of AIG with GPs in patients.METHODS This double c...BACKGROUND The relationship between autoimmune gastritis(AIG)and gastric polyps(GPs)is not well understood.AIM To explore the clinical characteristics and risk factors of AIG with GPs in patients.METHODS This double center retrospective study included 530 patients diagnosed with AIG from July 2019 to July 2023.We collected clinical,biochemical,serological,and demographic data were of each patient.Logistic regression analyses,both multivariate and univariate,were conducted to pinpoint independent risk factors for GPs in patients with AIG patients.Receiver operating characteristic curves were utilized to establish the optimal cutoff values,sensitivity,and specificity of these risk factors for predicting GPs in patients with AIG.RESULTS Patients with GPs had a higher median age than those without GPs[61(52.25-69)years vs 58(47-66)years,P=0.006].The gastrin-17 levels were significantly elevated in patients with GPs compared with those without GPs[91.9(34.2-138.9)pmol/mL vs 60.9(12.6-98.4)pmol/mL,P<0.001].Additionally,the positive rate of parietal cell antibody(PCA)antibody was higher in these patients than in those without GPs(88.6%vs 73.6%,P<0.001).Multivariate and univariate analyses revealed that PCA positivity[odds ratio(OR)=2.003,P=0.017],pepsinogen II(OR=1.053,P=0.015),and enterochromaffin like cells hyperplasia(OR=3.116,P<0.001)were significant risk factors for GPs,while pepsinogen I was identified as a protective factor.CONCLUSION PCA positivity and enterochromaffin like cells hyperplasia are significant risk factor for the development of GPs in patients with AIG.Elevated gastrin-17 levels may also play a role in this process.These findings suggest potential targets for further research and therapeutic intervention in managing GPs in patients with AIG.展开更多
Foot reflexology(FR)is a Chinese-originated and non-invasive complementary therapy increasingly used by functional,alternative and para-medical professionals.Enhance attempts are made to study FR in non-functional org...Foot reflexology(FR)is a Chinese-originated and non-invasive complementary therapy increasingly used by functional,alternative and para-medical professionals.Enhance attempts are made to study FR in non-functional organic conditions.The present invited Editorial discusses the application of FR in autoimmune diseases(AD),highlighting a few successful studies demonstrating symptomatic relief and objective improvements.Despite promising results,the FR domain remains under-investigated and an urgent need to confirm and understand the effect of FR in chronic diseases,including AD,is highly recommended.展开更多
BACKGROUND Autoimmune gastritis(AIG)is recognized endoscopically by the presence of antrum-sparing corpus-dominant atrophy,known as reverse atrophy.However,a past Helicobacter pylori(H.pylori)infection can obscure thi...BACKGROUND Autoimmune gastritis(AIG)is recognized endoscopically by the presence of antrum-sparing corpus-dominant atrophy,known as reverse atrophy.However,a past Helicobacter pylori(H.pylori)infection can obscure this classic pattern.We present two cases of AIG with past H.pylori infection and highlight a novel endoscopic sign that may aid AIG recognition when typical features are absent.CASE SUMMARY One patient reported postprandial fullness,while the other was asymptomatic.Neither had a history of H.pylori eradication therapy.Both tested negative on a urea breath test and positive for anti-parietal cell antibodies.In both patients,endoscopy revealed mucosal atrophy involving both the corpus and antrum,which was counter to the characteristic reverse atrophy pattern typically seen in AIG.Beyond the atrophic border,we observed a distinct pattern of gyrus-like changes,manifesting as elevated mucosa between deep fissures.Histologically,targeted biopsies from these gyrus-like areas revealed parietal cell degeneration,lymphocytic infiltration,and hyperplasia of enterochromaffin-like cells,consistent with early histopathologic changes seen in AIG.These results supported diagnoses of AIG with past H.pylori infection.CONCLUSION Gyrus-like changes may serve as a novel endoscopic clue of AIG with past H.pylori infection.展开更多
Autoimmune diseases frequently present with ophthalmological manifestations,posing significant diagnostic and therapeutic challenges.This review delved into the complex interplay between autoimmunity and ocular health...Autoimmune diseases frequently present with ophthalmological manifestations,posing significant diagnostic and therapeutic challenges.This review delved into the complex interplay between autoimmunity and ocular health,highlighting common manifestations such as uveitis,keratitis,and optic neuritis.We explored advanced diagnostic tools and techniques to improve early detection and accurate diagnosis.Additionally,the review addressed current therapeutic strategies,emphasizing the need for tailored treatments to manage ocular symptoms effectively while minimizing systemic side effects.By overcoming these challenges we aimed to enhance patient outcomes and quality of life for those affected by autoimmune-related eye diseases.展开更多
BACKGROUND Noninvasive tests are crucial for the management and follow-up of patients with autoimmune hepatitis,but their validation is limited because of insufficient data.AIM To investigate the diagnostic performanc...BACKGROUND Noninvasive tests are crucial for the management and follow-up of patients with autoimmune hepatitis,but their validation is limited because of insufficient data.AIM To investigate the diagnostic performance of three fibrosis noninvasive tests[FibroTest,vibration-controlled transient elastography(VCTE),and the fibrosis-4 index(FIB-4)and two activity biomarkers(alanine aminotransferase(ALT)and ActiTest].METHODS This study enrolled 103 patients for whom liver biopsy,hepatic elastography results,and laboratory markers were available.Diagnostic performance was assessed with receiver operating characteristic(ROC)curves,the Obuchowski measure(OM),and the Bayesian latent class model.RESULTS FibroTest and VCTE outperformed FIB-4 in cases of significant fibrosis(≥F2),with areas under the ROC curve of 0.83[95%confidence interval(CI):0.73-0.90],0.86(95%CI:0.77-0.92),and 0.71(95%CI:0.60-0.80),respectively.The mean(standard error)OM values were 0.92(0.01),0.93(0.01),and 0.88(0.02)for FibroTest,VCTE,and FIB-4,respectively;FibroTest and VCTE performed comparably,and both were superior to FIB-4(P=0.03 and P=0.005).The areas under the ROC curve values for activity biomarkers were 0.86(95%CI:0.76-0.92)for ActiTest and 0.84(95%CI:0.73-0.90)for ALT(P=0.06).The OM values for ActiTest and ALT were 0.92(0.02)and 0.90(0.02),respectively(P=0.005).CONCLUSION FibroTest and VCTE outperformed FIB-4 according to the OM.FibroTest-ActiTest facilitated the evaluation of both fibrosis and activity.展开更多
基金Jiangsu Province Traditional Chinese Medicine Science and Technology Development Program(YB2020037)Nanjing Infectious Disease Clinical Medical Center,Innovation Center for Infectious Disease of Jiangsu Province(NO.CXZX202232)Nanjing Health Science and Technology Development Special Fund Project(YKK22127).
文摘Background and AimsAutoimmune hepatitis(AIH)frequently coexists with extrahepatic autoimmune diseases(EADs),but their prevalence,characteristics,progression,and treatment effect in the Han Chinese population remain unclear.This study aimed to evaluate the prevalence and spectrum of EADs and to assess their clinical features,disease course,and treatment outcomes in Han Chinese patients with AIH.MethodsMedical records of 371 Han Chinese patients with AIH(diagnosed from March 2016 to October 2023)were retrospectively analyzed.ResultsAmong the 371 AIH patients,304(81.94%)were female,with a median age of 52.5 years(interquartile range,46.0-61.0).A total of 23.98%(89/371)had at least one EAD,including 27.06%(82/303)in type 1 AIH,11.11%(7/63)in antibody-negative AIH,and none in type 2.A single EAD was the most common(20.21%,75/371).The most frequent EADs were Sjogren’s syndrome(8.63%)and autoimmune thyroid disease(8.36%).Compared with patients without EADs,those with EADs had lower alanine aminotransferase,red blood cell,and hemoglobin levels,but higher aspartate aminotransferase/alanine aminotransferase ratio and antinuclear antibody(ANA)positivity(all P<0.05).ANA positivity was independently associated with EADs(odds ratio=2.209,95%confidence interval=1.242-3.927,P=0.007).After three months of treatment,the complete biochemical response rate was lower in the EADs group than in the non-EADs group(40.0%vs.55.3%,P=0.024),whereas no significant differences were observed at 6,12,24,or 36 months(all P>0.05).ConclusionsIn the Han Chinese population,23.98%of AIH patients had EADs,with Sjogren’s syndrome and autoimmune thyroid disease being the most common.ANA positivity was a significant risk factor for EADs.EAD patients had a poorer initial treatment response at three months,but comparable long-term biochemical response from six months.
文摘Background:In patients with autoimmune hemolytic anemia(AIHA),the risk of relapse is high owing to persistent autoreactive B-cell activity.Multirefractory AIHA is a more advanced stage of disease that is defined by a lack of response to at least three lines of therapy.CD19-directed chimeric antigen receptor(CAR)T-cell therapy results in profound B-cell depletion and may be a useful approach to achieving drug-free remission in multirefractory AIHA.
文摘Autoimmune enteropathy(AIE)is a rare immune mediated disorder primarily affecting children,characterized by chronic diarrhea,malabsorption,vomiting,weight loss and villous atrophy.It has also been observed in adults presenting diagnostic and treatment challenges due to its overlap with other gastrointestinal disorders such as celiac disease.Initial diagnostic criteria for AIE include small bowel villous atrophy,lack of response to dietary restrictions,presence of anti-enterocyte antibodies,and predisposition to autoimmunity without severe immu-nodeficiency.Refined criteria emphasize characteristic histological findings and exclusion of other causes of villous atrophy.AIE is associated with various autoimmune disorders and can present with overlapping features with Celiac disease,including villous atrophy but without significant intraepithelial lympho-cytosis.Treatment primarily involves immunosuppression using corticosteroids,calcineurin inhibitors,and anti-tumor necrosis factor therapy,alongside nutri-tional support.Despite the challenges,understanding AIE’s diverse manifest-ations and improving diagnostic criteria are essential for effective management and improved patient outcome.Further research is needed to elucidate the pathogenesis,disease progression and long-term outcomes of AIE.
基金supported by the National Research Foundation of South Korea(2023R1A2C2004516,RS-2023-00219399 to SPY,and 2022R1I1A1A01063513 to MGJ)。
文摘Parkinson's disease is a neurodegenerative disorder marked by the degeneration of dopaminergic neurons and clinical symptoms such as tremors,rigidity,and slowed movements.A key feature of Parkinson's disease is the accumulation of misfoldedα-synuclein,forming insoluble Lewy bodies in the substantia nigra pars compacta,which contributes to neurodegeneration.Theseα-synuclein aggregates may act as autoantigens,leading to T-cell-mediated neuroinflammation and contributing to dopaminergic cell death.Our perspective explores the hypothesis that Parkinson's disease may have an autoimmune component,highlighting research that connects peripheral immune responses with neurodegeneration.T cells derived from Parkinson's disease patients appear to have the potential to initiate an autoimmune response againstα-synuclein and its modified peptides,possibly leading to the formation of neo-epitopes.Recent evidence associates Parkinson's disease with abnormal immune responses,as indicated by increased levels of immune cells,such as CD4^(+)and CD8^(+)T cells,observed in both patients and mouse models.The convergence of T cells filtration increasing major histocompatibility complex molecules,and the susceptibility of dopaminergic neurons supports the hypothesis that Parkinson's disease may exhibit autoimmune characteristics.Understanding the immune mechanisms involved in Parkinson's disease will be crucial for developing therapeutic strategies that target the autoimmune aspects of the disease.Novel approaches,including precision medicine based on major histocompatibility complex/human leukocyte antigen typing and early biomarker identification,could pave the way for immune-based treatments aimed at slowing or halting disease progression.This perspective explores the relationship between autoimmunity and Parkinson's disease,suggesting that further research could deepen understanding and offer new therapeutic avenues.In this paper,it is organized to provide a comprehensive perspective on the autoimmune aspects of Parkinson's disease.It investigates critical areas such as the autoimmune response observed in Parkinson's disease patients and the role of autoimmune mechanisms targetingα-synuclein in Parkinson's disease.The paper also examines the impact of CD4~+T cells,specifically Th1 and Th17,on neurons through in vitro and ex vivo studies.Additionally,it explores howα-synuclein influences glia-induced neuroinflammation in Parkinson's disease.The discussion extends to the clinical implications and therapeutic landscape,offering insights into potential treatments.Consequently,we aim to provide a comprehensive perspective on the autoimmune aspects of Parkinson's disease,incorporating both supportive and opposing views on its classification as an autoimmune disorder and exploring implications for clinical applications.
基金Supported by the Nanjing Health Science and Technology Key Medical Science and Technology Development Program:Mechanism of Action of the Modified Si-Miao Powder with Sanguisorba Carbonisata in Regulating Microbiota and Microecology for the Treatment of Recurrent Vulvovaginal Candidiasis(ZKX22039)。
文摘OBJECTIVE:To elucidate the therapeutic efficacy and mechanism of action of Chaihu Guizhi Ganjiang decoction(柴胡桂枝干姜汤,CGGD)in autoimmune hepatitis.METHODS:CGGD components and potential target genes were extracted from previously published databases.The autoimmune hepatitis(AIH)-related regulatory genes were obtained from the Dis Ge NET database.Intersections were taken,and enrichment analyses were performed on the extracted data.Concanavalin A(Con A)-induced AIH model mice were treated with CGGD via gavage.The results of network pharmacological analysis were experimentally validated.RESULTS:Network pharmacology revealed 228 genes at the intersection of AIH and CGGD.Kyoto Encyclopedia of Genes and Genomes analysis revealed that CGGD primarily regulates the phosphoinositide 3-kinase(PI3K)/protein kinase B(AKT)signaling pathway and cellular metabolism in AIH.Gene Ontology enrichment analysis revealed that CGGD modulates inflammation through transcription factor-mediated signaling pathways.As predicted,CGGD attenuated Con A-induced AIH in a dose-dependent manner by activating the PI3K/AKT signaling pathway.Histopathological assessment confirmed the protective effects of CGGD against Con Ainduced AIH.Further investigation revealed that CGGD regulated the T helper cell 17(Th17)/regulatory T cell(Treg)balance by modulating the PI3K/Akt/nuclear factor kappa-B(NF-κB)pathway.CONCLUSIONS:This study demonstrated the therapeutic effect of CGGD on AIH through a combination of network pharmacological prediction and experimental validation.Its mechanism of action involves PI3K/Akt/NF-κB-mediated regulation of Th17/Treg cells.
文摘This letter addresses the study by Jayabalan et al,which underscores the liver outcome score(LOS)and hemoglobin(Hb)as key prognostic markers for patients with autoimmune liver disease overlap syndromes(AILDOS),with particular relevance to the autoimmune hepatitis-primary biliary cholangitis(AIH-PBC)subgroup.The findings indicate that an LOS threshold of 6 achieves high sensitivity and specificity in predicting liver-related mortality among AIH-PBC patients.Moreover,low Hb levels emerge as a significant mortality predictor across all AILDOS cases.These results contribute valuable perspectives on risk stratification in AILDOS,highlighting the promise of non-invasive prognostic tools.Future studies with larger cohorts are needed to substantiate LOS and Hb as robust markers for clinical application.
基金Supported by the Basic Science Research Program through the National Research Foundation of Korea funded by the Ministry of Education,No.RS-2023-00237287.
文摘Autoimmune liver disease overlap syndrome(OS)is a rare and clinically significant condition that has received limited attention in microbiome research.In their recent study,Wang et al combined 16S rRNA sequencing with untargeted metabolomics to characterize the gut-liver axis in OS,identifying shared features of dysbiosis in autoimmune hepatitis(AIH)and primary biliary cholangitis(PBC),and unique signatures,including enrichment of Klebsiella and Escherichia and depletion of aromatic amino acids.In this letter,we critically appraise these findings,emphasizing that OS should be considered a distinct immunometabolic phenotype rather than a simple mixture of AIH and PBC.We discuss the potential mechanistic relevance of the Fusicatenibacter-tyrosine relationship,highlight the clinical implications of integrating microbiota-metabolite analyses,and outline the limitations that future studies must address.
基金Supported by The Key Research and Development Project of the Science and Technology Department of Sichuan Province,China,No.2023YFS0280The High-Level Research Initiation Fund of The First Affiliated Hospital of Chengdu Medical College,China,No.CYFY-GQ43.
文摘Recently,Jayabalan et al published an important study.The authors defined the liver outcome score as a novel biomarker for predicting liver-related mortality in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome.After thoroughly reviewing their work,we offer insights that primarily relate to their study design to enhance the medical community’s understanding of this complex disease.
文摘Autoimmune hepatitis(AIH)is a rare cause of chronic liver disease.The exact pa-thophysiology of AIH is unknown.Breakdown of self-tolerance against hepatic antigens and molecular mimicry are often implicated in the pathogenesis of AIH.Immunosuppressive therapy is the mainstay of treatment;however,10%–25%of patients with AIH may not respond to primary therapy.Those patients are often salvaged with second-and third-line immunosuppressive therapy.Workup for other concomitant diseases should be done for patients who fail to respond to primary immunosuppressive therapy.Concurrent metabolic dysfunction-asso-ciated steatotic liver disease,alcohol-related liver disease,overlap syndrome(AIH with primary biliary cholangitis or sclerosing cholangitis),chronic hepatitis B virus,hepatitis C virus,and human immunodeficiency virus infection should be ruled out in such cases.Targeting the concomitant etiology may lead to resolution of the clinical symptoms and induce biochemical and histological remission.Isolated AIH without other etiologies for liver injury should be managed with a higher dose of steroids,azathioprine,or other immunosuppressive agents.Second-and third-line immunosuppressive agents include mycophenolate mofetil,cyclosporine,tacrolimus,infliximab,and rituximab.Patients with AIH may present with acute severe AIH(AS-AIH)and AIH-related acute on chronic liver failure,and they often require liver transplantation.The terms refractory or difficult-to-treat AIH have been used interchangeably and have no distinct definition.Difficult-to-treat AIH includes patients with intolerable side effects,fulminant disease(AIH with acute on chronic liver failure and AS-AIH),AIH in pregnancy,and HIV infection.Patients who fail to respond to standard first-line immunosuppressive therapy should be classified as refractory AIH.This review addresses the issues in the management of difficult-to-treat AIH with recent advances in pharmacological management.
文摘BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High levels of anti-ganglionic nicotinic acetylcholine receptor(gAChR)serum antibodies are detected in approximately 50%of AAG cases,confirming the diagnosis.CASE SUMMARY We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period.Recently,the patient was unable to stand upright for more than a few seconds before fainting.Additionally,he presented with decreased sweating,dry mouth,urinary retention,early satiety,weight loss,bloating,constipation,and erectile dysfunction.Neurological examination revealed dilated pupils that were unresponsive to light.Deep tendon reflexes were absent or diminished.Serologic evaluation revealed the presence of gAChR autoantibodies.An orthostatic hypotension test yielded a positive result.The patient did not respond to symptomatic therapy,including midodrine,fludrocortisone and atomoxetine.Second-line therapy with immunoadsorption produced a noticeable clinical improvement;however,orthostatic hypotension persisted.Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.CONCLUSION Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.
文摘Drug-induced autoimmune-like hepatitis(DI-ALH)is an increasingly recognized phenotype within the spectrum of drug-induced liver injury.Several drugs,including nitrofurantoin,minocycline,hydralazine,methyldopa and infliximab,have a well-documented capacity to induce DI-ALH.Distinguishing DI-ALH from classic de novo autoimmune hepatitis(AIH)can be challenging due to overlapping clinical,biochemical,and serological features.Accurate distinction from classic AIH is crucial,as management and prognosis differ.While some DIALH cases resolve spontaneously after drug withdrawal,others show persistent or worsening liver injury.Histological studies have shown that fibrosis and cirrhosis are more prevalent in classic AH.Unfortunately,there are no pathognomic clinical,biochemical or immunological features that reliably distinguish DI-ALH from classic AIH.However,most patients with DI-ALH do not relapse after corticosteroid withdrawal,in contrast to the high relapse rate observed in classic AIH.Most patients respond well to corticosteroids,and once liver tests normalize,biochemical parameters should be monitored,and long-term immunosuppression should not be indicated.However,DI-ALH is not exempt from risk of relapse,underscoring the need for long-term follow-up.Most patients with DIALH have a favorable prognosis;however,although rare,cases of cirrhosis and,in exceptional instances,acute liver failure have been reported.International collaborative studies are needed to further characterize DI-ALH.In this review,we update current controversies,present emerging concepts,and outline future challenges in the diagnosis and management of this complex condition learned so far.
文摘BACKGROUND Autoimmune hepatitis(AIH)is typically treated with immunomodulators and steroids.However,some patients are refractory to these treatments,necessitating alternative approaches.Biological therapies have recently been explored for these difficult cases.AIM To assess the efficacy and safety of biologics in AIH,focusing on patients unresponsive to standard treatments and evaluating outcomes such as serological markers and histological remission.METHODS A case-based systematic review was performed following the PRISMA protocol to evaluate the efficacy and safety of biological therapies in AIH.The primary focus was on serological improvement and histological remission.The secondary focus was on assessing therapy safety and additional outcomes.A standardized search command was applied to MEDLINE,EMBASE,and Cochrane Library databases to identify relevant studies.Inclusion criteria encompassed adult AIH patients treated with biologics.Data were analyzed based on demographics,prior treatments,and therapy-related outcomes.A narrative synthesis was employed to address biases and provide a comprehensive overview of the evidence.RESULTS A total of 352 studies were reviewed,with 30 selected for detailed analysis.Key findings revealed that Belimumab led to a favourable response in five out of eight AIH patients across two studies.Rituximab demonstrated high efficacy,with 41 out of 45 patients showing significant improvement across six studies.Basiliximab was assessed in a single study,where the sole patient treated experienced a beneficial outcome.Additionally,a notable number of AIH cases were induced by anti-tumor necrosis factor(TNF)medications,including 16 cases associated with infliximab and four cases with adalimumab.All these cases showed improvement upon withdrawal of the biologic agent.CONCLUSION Belimumab and Rituximab show promise as effective alternatives for managing refractory AIH,demonstrating significant improvements in clinical outcomes and liver function.However,the variability in patient responses to different therapies highlights the need for personalized treatment strategies.The risk of AIH induced by anti-TNF therapies underscores the need for vigilant monitoring and prompt symptom recognition.These findings support the incorporation of biologic agents into AIH treatment protocols,particularly for patients who do not respond to conventional therapies.
基金Supported by the Oman Ministry of Higher Education,Research,and Innovation,No.BFP/RGP/HSS/24/015.
文摘Chloroquine(CQ)and hydroxychloroquine(HCQ),originally developed as anti-malarial drugs,have found a new purpose in treating various autoimmune dis-eases due to their immunomodulatory properties.These drugs work through mu-ltiple mechanisms,including inhibiting Toll-like receptor signaling,suppressing antigen presentation,and modulating autophagy.This review article provides a comprehensive analysis of the immunomodulatory effects of CQ and HCQ in several autoimmune diseases such as systemic lupus erythematosus,rheumatoid arthritis,systemic sclerosis,and others.We delve into the intricate mechanisms of action,highlighting the key immune cells involved and discussing the clinical implications of these drugs in managing autoimmune conditions.Our review covers the latest research and clinical trials,offering a comprehensive under-standing of the therapeutic potential of CQ and HCQ in autoimmune diseases.We also discuss the challenges and controversies surrounding the use of these drugs,such as their long-term side effects and the need for personalized treatment approaches.By synthesizing current knowledge and identifying areas for future research,this review aims to provide a valuable resource for healthcare profes-sionals and researchers involved in the management of autoimmune diseases.
基金supported in part by the Japan Pancreas Soci-ety and the MHLW Research Program on Rare and Intractable Dis-eases(Grant Number 23FC1015,Principal investigator:Mitsuhiro Kawano).
文摘Background:Previous studies have highlighted the frequent occurrence of sarcopenia in patients with pancreatic diseases,including chronic pancreatitis.We aimed to clarify the prevalence of skeletal muscle(SM)loss and sarcopenia,and their associations with clinical characteristics,bone mineral density,and pancreatic imaging findings in patients with autoimmune pancreatitis(AIP).Methods:This study included 114 patients with AIP treated at Tohoku University Hospital.The SM index was assessed using a bioelectrical impedance analysis device,grip strength was measured using a hand dynamometer,and bone mineral density was evaluated using dual-energy X-ray absorptiometry.Univari-ate and multivariate logistic regression analyses were used to analyze factors associated with SM loss and sarcopenia.Results:Among 114 patients,57(50.0%)had SM loss,31(27.2%)had reduced grip strength,and 27(23.7%)had both.Patients with SM loss were older and had a lower body mass index,weaker grip strength,higher Controlling Nutritional Status scores,and lower serum lipase and albumin levels compared to those without SM loss.Computed tomography scans revealed a higher prevalence of pancreatic parenchy-mal atrophy in patients with SM loss.Similar differences were observed between patients with sarcopenia and those without.Osteopathy was observed in 35.6%of patients with SM loss and 38.1%of those with sarcopenia,whereas only 4.1%of patients without SM loss had osteopathy.Low BMI(<21.0 kg/m^(2))was also found to be an independent risk factor for SM loss in multivariate analysis.Age>72 years,low BMI(<20.0 kg/m^(2)),and low serum lipase levels(<13 U/L)were independent risk factors for sarcopenia in multivariate analysis.Conclusions:SM loss and sarcopenia are prevalent in patients with AIP and are associated with aging,poor nutritional status,low serum lipase levels,and pancreatic parenchymal atrophy.In addition to the high risk of osteopathy,careful attention should be paid to maintain muscle health in AIP patients.
基金Supported by Xinjiang“Tianshan Talents”Medical and Health High-Level Talent Training Program-Young and Middle-Aged Backbone Medical Talents.
文摘BACKGROUND Hepatitis D virus-hepatitis B virus(HDV-HBV)co-infection accelerates liver disease progression and increases the risk of hepatocellular carcinoma,but the immunopathogenic mechanism of its combination with autoimmune hepatitis(AIH)has not been clarified.This study reveals for the first time that HDV may induce AIH through abnormalities in immunoregulation in two specific cases.This is the first report of HDV-HBV co-infected patients who did not receive interferon therapy and achieved serological conversion and histological remission by combining antiviral(entecavir)with immunosuppression(prednisone+azathioprine)therapy,providing new evidence of the mechanism of this complex disease.CASE SUMMARY A 40-year-old female developed malaise and jaundice with an alanine aminotransferase/aspartate aminotransferase>20 upper limit of normal(ULN),total bilirubin:97.20μmol/L,immunoglobulin G(IgG)47.1 g/L(>3×ULN),HDV RNA 1.6×10^(7)copies/mL and liver biopsy showed G3S4.Tenofovir alafenamide combined with prednisone and azathioprine was administered,and three months later the Child-Turcotte-Pugh class C was reduced to class B and IgG decreased to 13.62 g/L.Another 58-year-old male complained of pain in the liver area,antinuclear antibody was 1:320,IgG 22.6 g/L(>1.3×ULN),and liver biopsy showed G2S3.Entecavir was administered in combination with prednisone and azathioprine,and after 3 months,liver function returned to normal,and IgG reduced to 14.22 g/L.CONCLUSION Patients with HDV-HBV co-infection combined with AIH can achieve clinical remission following combination therapy,and the study of immunomodulatory mechanisms should be emphasized.
基金Supported by the Health Technology Project of Pudong New District Health Commission,No.PW2020D-12.
文摘BACKGROUND The relationship between autoimmune gastritis(AIG)and gastric polyps(GPs)is not well understood.AIM To explore the clinical characteristics and risk factors of AIG with GPs in patients.METHODS This double center retrospective study included 530 patients diagnosed with AIG from July 2019 to July 2023.We collected clinical,biochemical,serological,and demographic data were of each patient.Logistic regression analyses,both multivariate and univariate,were conducted to pinpoint independent risk factors for GPs in patients with AIG patients.Receiver operating characteristic curves were utilized to establish the optimal cutoff values,sensitivity,and specificity of these risk factors for predicting GPs in patients with AIG.RESULTS Patients with GPs had a higher median age than those without GPs[61(52.25-69)years vs 58(47-66)years,P=0.006].The gastrin-17 levels were significantly elevated in patients with GPs compared with those without GPs[91.9(34.2-138.9)pmol/mL vs 60.9(12.6-98.4)pmol/mL,P<0.001].Additionally,the positive rate of parietal cell antibody(PCA)antibody was higher in these patients than in those without GPs(88.6%vs 73.6%,P<0.001).Multivariate and univariate analyses revealed that PCA positivity[odds ratio(OR)=2.003,P=0.017],pepsinogen II(OR=1.053,P=0.015),and enterochromaffin like cells hyperplasia(OR=3.116,P<0.001)were significant risk factors for GPs,while pepsinogen I was identified as a protective factor.CONCLUSION PCA positivity and enterochromaffin like cells hyperplasia are significant risk factor for the development of GPs in patients with AIG.Elevated gastrin-17 levels may also play a role in this process.These findings suggest potential targets for further research and therapeutic intervention in managing GPs in patients with AIG.
文摘Foot reflexology(FR)is a Chinese-originated and non-invasive complementary therapy increasingly used by functional,alternative and para-medical professionals.Enhance attempts are made to study FR in non-functional organic conditions.The present invited Editorial discusses the application of FR in autoimmune diseases(AD),highlighting a few successful studies demonstrating symptomatic relief and objective improvements.Despite promising results,the FR domain remains under-investigated and an urgent need to confirm and understand the effect of FR in chronic diseases,including AD,is highly recommended.
基金Supported by the General Program of the National Natural Science Foundation of China,No.81973920.
文摘BACKGROUND Autoimmune gastritis(AIG)is recognized endoscopically by the presence of antrum-sparing corpus-dominant atrophy,known as reverse atrophy.However,a past Helicobacter pylori(H.pylori)infection can obscure this classic pattern.We present two cases of AIG with past H.pylori infection and highlight a novel endoscopic sign that may aid AIG recognition when typical features are absent.CASE SUMMARY One patient reported postprandial fullness,while the other was asymptomatic.Neither had a history of H.pylori eradication therapy.Both tested negative on a urea breath test and positive for anti-parietal cell antibodies.In both patients,endoscopy revealed mucosal atrophy involving both the corpus and antrum,which was counter to the characteristic reverse atrophy pattern typically seen in AIG.Beyond the atrophic border,we observed a distinct pattern of gyrus-like changes,manifesting as elevated mucosa between deep fissures.Histologically,targeted biopsies from these gyrus-like areas revealed parietal cell degeneration,lymphocytic infiltration,and hyperplasia of enterochromaffin-like cells,consistent with early histopathologic changes seen in AIG.These results supported diagnoses of AIG with past H.pylori infection.CONCLUSION Gyrus-like changes may serve as a novel endoscopic clue of AIG with past H.pylori infection.
文摘Autoimmune diseases frequently present with ophthalmological manifestations,posing significant diagnostic and therapeutic challenges.This review delved into the complex interplay between autoimmunity and ocular health,highlighting common manifestations such as uveitis,keratitis,and optic neuritis.We explored advanced diagnostic tools and techniques to improve early detection and accurate diagnosis.Additionally,the review addressed current therapeutic strategies,emphasizing the need for tailored treatments to manage ocular symptoms effectively while minimizing systemic side effects.By overcoming these challenges we aimed to enhance patient outcomes and quality of life for those affected by autoimmune-related eye diseases.
文摘BACKGROUND Noninvasive tests are crucial for the management and follow-up of patients with autoimmune hepatitis,but their validation is limited because of insufficient data.AIM To investigate the diagnostic performance of three fibrosis noninvasive tests[FibroTest,vibration-controlled transient elastography(VCTE),and the fibrosis-4 index(FIB-4)and two activity biomarkers(alanine aminotransferase(ALT)and ActiTest].METHODS This study enrolled 103 patients for whom liver biopsy,hepatic elastography results,and laboratory markers were available.Diagnostic performance was assessed with receiver operating characteristic(ROC)curves,the Obuchowski measure(OM),and the Bayesian latent class model.RESULTS FibroTest and VCTE outperformed FIB-4 in cases of significant fibrosis(≥F2),with areas under the ROC curve of 0.83[95%confidence interval(CI):0.73-0.90],0.86(95%CI:0.77-0.92),and 0.71(95%CI:0.60-0.80),respectively.The mean(standard error)OM values were 0.92(0.01),0.93(0.01),and 0.88(0.02)for FibroTest,VCTE,and FIB-4,respectively;FibroTest and VCTE performed comparably,and both were superior to FIB-4(P=0.03 and P=0.005).The areas under the ROC curve values for activity biomarkers were 0.86(95%CI:0.76-0.92)for ActiTest and 0.84(95%CI:0.73-0.90)for ALT(P=0.06).The OM values for ActiTest and ALT were 0.92(0.02)and 0.90(0.02),respectively(P=0.005).CONCLUSION FibroTest and VCTE outperformed FIB-4 according to the OM.FibroTest-ActiTest facilitated the evaluation of both fibrosis and activity.