Immunoglobulin G4-related spinal pachymeningitis:A case report

BACKGROUND Immunoglobulin G4-related disease(IgG4-RD)is a complex immune-mediated condition that causes fibrotic inflammation in several organs.A significant clinical feature of IgG4-RD is hypertrophic pachymeningitis,which manifests as inflammation of the dura mater in intracranial or spinal regions.Although IgG4-RD can affect multiple areas,the spine is a relatively rare site compared to the more frequent involvement of intracranial structures.CASE SUMMARY A 70-year-old male presented to our hospital with a two-day history of fever,altered mental status,and generalized weakness.The initial brain magnetic resonance imaging(MRI)revealed multiple small infarcts across various cerebral regions.On the second day after admission,a physical examination revealed motor weakness in both lower extremities and diminished sensation in the right lower extremity.Electromyographic evaluation revealed findings consistent with acute motor sensory neuropathy.Despite initial management with intravenous immunoglobulin for presumed Guillain-Barrésyndrome,the patient exhibited progressive worsening of motor deficits.On the 45th day of hospitalization,an enhanced MRI of the entire spine,focusing specifically on the thoracic 9 to lumbar 1 vertebral level,raised the suspicion of IgG4-related spinal pachymeningitis.Subsequently,the patient was administered oral prednisolone and participated in a comprehensive rehabilitation program that included gait training and lower extremity strengthening exercises.CONCLUSION IgG4-related spinal pachymeningitis,diagnosed on MRI,was treated with corticosteroids and a structured rehabilitation regimen,leading to significant improvement.

丙型肝炎病毒IgG抗体阳性者确诊丙型肝炎的高危因素分析及列线图预测模型构建

目的分析丙型肝炎(丙肝)病毒(HCV)免疫球蛋白G(IgG)抗体阳性者中确诊HCV的高危因素,同时建立列线图(nomogram)预测模型,为基层医疗机构丙肝的临床诊断和转诊决策提供依据。方法回顾性收集2022年1月—2023年10月住院筛查患者中同时进行HCV\|RNA、HCV-IgG抗体、肝功能和血常规检测HCV-IgG抗体阳性者的人口学特征及丙肝相关各项指标。采用logistic回归分析HCV-IgG抗体阳性者确诊丙肝的高危因素,并构建nomogram模型,分别采用一致性系数和校准曲线评估模型的预测性能和符合度。结果394例HCV-IgG抗体阳性者中,HCV-RNA阳性率为30.2%。多因素logistic分析显示,在HCV-IgG抗体阳性的人群中,HCV-IgG≥5.0 S/CO、天冬氨酸氨基转移酶(AST)>35 U/L和有肝炎相关临床症状为HCV-RNA阳性的独立危险因素,其OR值分别为233.926(95%CI:31.814~1720.046)、4.079(95%CI:2.105~7.904)和5.295(95%CI:1.505~18.634)。用于预测HCV-RNA阳性的nomogram模型准确度为0.923,灵敏度为99.2%,特异度为74.5%。结论基于HCV-IgG抗体、AST和肝炎相关临床症状的nomogram模型具有高准确度,可用于指导临床医生判断HCV-IgG抗体阳性者确诊HCV感染的风险。

Clinical and pathological differences between serum immunoglobulin G4-positive and -negative type 1 autoimmune pancreatitis

AIM: To identify clinical and pathological differences between serum immunoglobulin G4 (IgG4)-positive (SIP) and IgG4-negative (SIN) type 1 autoimmune pancreatitis (AIP) in South Korea. METHODS: AIP was diagnosed by the international consensus diagnostic criteria. The medical records and pathology were retrospectively reviewed and IgG4-positive cells were counted in a high power field (HPF). Type I AIP was defined as a high serum level of IgG4or histological finding. SIN type 1 AIP was defined as a histological evidence of type 1 AIP and a normal serum IgG4 level. The clinical and pathological findings were compared between the two groups. The analysis was performed using Student's t test, Fischer's exact test and Mann-Whitney's U test. A P value of < 0.05 was considered statistically significant. As repeated com- parison was made, P values of less than 5% (P < 0.05) were considered significant. RESULTS: Twenty five patients with definite type 1 AIP (19 histologically and six serologically diagnosed cases) were enrolled. The mean tissue IgG4 concentrations were significantly higher in SIP than SIN group (40 cells per HPF vs 18 cells per HPF, P = 0.02). Among eight SIN patients, the tissue IgG4 concentrations were less than 15 cells per HPF in most of cases, except one. The sensitivity of serum IgG4 was 68% (17 SIP and eight SIN AIP). Other organ involvement was more frequent- ly associated with SIP than SIN AIP (59% vs 26%, P = 0.016). However, the relapse rate and diffuse swelling of the pancreas were not associated with serum IgG4 level. The concentrations of IgG4-positive cells per HPF were higher in SIP than SIN AIP (40 vs 18, P = 0.02). CONCLUSION: The sensitivity of serum IgG4 was 68% in type 1 AIP. High serum IgG4 level was associated with other organ involvement and tissue IgG4 concentration but did not affect the relapse rate in type 1 AIP.

Retroperitoneal fibrosis associated with immunoglobulin G4-related disease

Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.Retroperitoneal fibrosis can be of 2 types:idiopathic and secondary.The recently advocated concept and diagnostic criteria of immunoglobulin G4(IgG4)-related disease,derived from research on autoimmune pancreatitis(AIP),has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease.We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease;however,the actual prevalence is unclear.Conversely,some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease.Because retroperitoneal fibrosis has no specific symptoms,diagnosis is primarily based on diagnostic imaging(computed tomography and magnetic resonance imaging),which is also useful in evaluating the effect of therapy.Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP.Thus,the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis.High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease.The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause.For patients with concurrent AIP,i.e.,IgG4-related retroperitoneal fibrosis,the starting dose of steroid is usually 30-40 mg/d.The response to steroid therapy is generally favorable.In most cases,the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment.However,the epidemiology,treatment for recurring retroperitoneal fibrosis,and long-term prognosis are still largely unknown.Further analysis of such cases and research are necessary.

复发性口腔溃疡患者食物不耐受特异性IgG抗体检测特征分析

目的分析复发性口腔溃疡(ROU)患者食物不耐受特异性免疫球蛋白G(IgG)抗体检测特征结果,以期为ROU患者对食物不耐受的诊断和治疗提供依据。方法采用回顾性分析,选取2020年1月至2023年12月在郑州大学第一附属医院门诊就诊的1975例ROU患者为研究对象,收集患者的一般资料,统计患者食物不耐受特异性IgG抗体检测结果,比较不同年龄、不同性别患者食物不耐受的抗体检测结果差异。结果血清IgG阳性率最高的前5种食物分别是鸡蛋(47.34%)、牛奶(31.14%)、小麦(21.87%)、大豆(21.22%)、西红柿(10.38%),其中牛肉(0.46%)、猪肉(0.56%)、鸡肉(1.37%)、虾(1.82%)的血清IgG阳性率较低。女性受试者对鳕鱼、鸡蛋、牛奶3种食物的不耐受阳性率显著高于男性,差异有统计学意义(P<0.05)。不同年龄患者在鸡肉、鳕鱼、鸡蛋、蘑菇、牛奶、虾、大豆、小麦8种食物中的不耐受阳性率差异有统计学意义(P<0.05);未成年组对鸡蛋、牛奶、大豆、小麦4种食物的不耐受阳性率高于中青年组和老年组,对虾、蘑菇的不耐受阳性率低于中青年组和老年组,对鸡肉的不耐受阳性率显著高于中青年组,对鳕鱼的不耐受阳性率显著高于老年组,差异有统计学意义(P<0.05)。结论ROU患者发生食物不耐受的概率较高,食物不耐受与患者的性别和年龄有一定关系,及早对ROU患者进行食物血清特异性IgG抗体检测,可及时发现患者不耐受的食物以调整饮食,避免食源性的机体免疫反应。

Immunoglobulin G4-related gastrointestinal diseases, are they immunoglobulin G4-related diseases?

In immunoglobulin G4(IgG4)-related disease(RD),organ enlargement or nodular lesions consisting of abundant infiltration of lymphocytes and IgG4-positive plasma cells and fibrosis are seen in various organs.Although infiltration of many IgG4-positive plasma cells is detected in the gastric and colonic mucosa and major duodenal papilla of patients with autoimmune pancreatitis,it cannot be diagnosed as a gastrointestinal lesion involved in IgG4-RD,because none of the following is observed in these lesions:a mass-like formation;dense fibrosis;or obliterative phlebitis.Based on our review of the literature,there appear to be two types of IgG4-related gastrointestinal disease.One is a gastrointestinal lesion showing marked thickening of the wall of the esophagus and stomach,consisting of dense fibrosis with abundant infiltration of IgG4-positive plasma cells,which usually show submucosal spreading.The other is an IgG4-related pseudotumor occurring in gastrointestinal regions such as the stomach,colon,and major duodenal papilla,showing polypoid or mass-like lesions.Most solitary IgG4-related gastrointestinal lesions that are not associated with other IgG4-RD appear to be difficult to diagnose.It is of utmost importance to rule out malignancy.However,these lesions may respond to steroid therapy.To avoid unnecessary resection,IgG4-related gastrointestinal diseases should be considered in the differential diagnosis.

Studies on Human Immunoglobulin G from GBS Patient (Ⅲ)-The Determination of Molecular Weight of Human Immunoglobulin G by Capillary SDS Gel Electrophoresis

Guillain-Barre syndrome (GBS) is considered to be an autoimmune disorder of peripheralnervous system. In this paper. capillary SDS gel electrophoresis was performed on neutral coatedfused-silica capillary to determine the molecular weight of purified IgG samples from GBS patient.

Biomarkers in autoimmune pancreatitis and immunoglobulin G4-related disease

Solitary organ autoimmune disorders,formerly known as autoimmune pancreatitis(AIP),autoimmune sialadenitis,and autoimmune sclerosing cholangitis,are now considered organ-specific manifestations of systemic immunoglobulin G4-related disease(IgG4-RD).AIP and IgG4-RD are characterized by elevated serum concentration of IgG4 antibody(Ab),accumulation of IgG4-expressing plasmacytes in the affected organs,and involvement of multiple organs.It is well established that enhanced IgG4 Ab responses are a hallmark of AIP and IgG4-RD for diagnosis and monitoring disease activity.However,a significant fraction of patients with AIP and IgG4-RD who develop chronic fibroinflammatory responses have normal serum concentrations of this IgG subtype.In addition,disease flare-up is sometimes seen even in the presence of normalized serum concentrations of IgG4 Ab after successful induction of remission by prednisolone.Therefore,it is necessary to identify new biomarkers based on the understanding of the pathophysiology of AIP and IgG4-RD.Recently,we found that activation of plasmacytoid dendritic cells producing both interferon-α(IFN-α)and interleukin-33(IL-33)mediate murine AIP and human IgG4-RD.More importantly,we provided evidence that serum concentrations of IFN-αand IL-33 could be useful biomarkers for the diagnosis and monitoring of AIP and IgG4-RD activity after induction of remission in these autoimmune disorders.In this Frontier article,we have summarized and discussed biomarkers of AIP and IgG4-RD,including Igs,autoAbs,and cytokines to provide useful information not only for clinicians but also for researchers.

Influence of birth order, birth weight, colostrum and serum immunoglobulin G on neonatal piglet survival

Background： Intake of colostrum after birth is essential to stimulate intestinal growth and function, and to provide systemic immunological protection via absorption of Immunoglobulin G （IgG）. The birth order and weight of 745 piglets （from 75 litters） were recorded during a one-week period of farrowing. Only pigs weighing greater than 0.68 kg birth weight were chosen for the trial. Sow colostrum was collected during parturition, and piglets were bled between 48 and 72 hours post-birth. Piglet serum IgG and colostral IgG concentrations were determined by radial immunodiffusion. Results： Sow parity had a significant （P 〈 0.001） effect on sow colostral IgG concentration, being 5% higher in multiparous females. Sow colostral IgG concentration explained 6% and piglet birth order accounted for another 4% of the variation observed in piglet serum IgG concentration （P 〈 0.05）; however, birth weight had no detectable effect. Piglet serum IgG concentration had both a linear （P 〈 0.05） and quadratic effect （P 〈 0.05） on % survival. Piglets with 1,000 mg/dl serum IgG or less （n=24） had a 67% survival; whereas, piglets with IgG concentrations between 2250 to 2500 mg/dl （n=247） had a 91% survival. Birth order had no detectable effect on survival, but birth weight had a positive linear effect （P 〈 0.05）. Piglets weighing 0.9 kg （n = 107） at birth had a 68% survival rate, and those weighing 1.6 kg （n = 158） had an 89% survival. Conclusion： We found that the combination of sow colostrum IgG concentration and birth order can account for 10% of the variation of piglet serum IgG concentration and that piglets with less than 1,000 mg/dl IgG serum concentration and weight of 0.9 kg at birth had low survival rate when compared to their larger siblings. The effective management of colostrum uptake in neonatal piglets in the first 24 hrs post-birth may potentially improve survival from birth to weaning.

Immunoglobulin G4-related autoimmune pancreatitis and sialadenitis: A case report

Immunoglobulin G4(Ig G4)-related disease is a rare systemic diseases. A 67-year-old male presented at our institution with mild upper abdominal pain and jaundice for 20 d. Laboratory results revealed high levels of Ig G4(15.4 g/L,range: 0.08-1.4 g/L). Computed tomography(CT) showed significant enlargement of the entire pancreas and a capsule-like low-density rim surrounding the whole pancreas. Positron emission tomography/CT revealed increased uneven metabolism of the entire pancreas. Both magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed stenosis of the distal common bile duct and proximal main pancreatic duct,and dilation of the proximal common bile duct and extra- and intra-hepatic bile ducts. He was diagnosed with Ig G4-related autoimmune pancreatitis. The patient was treated with prednisone for 14 mo. The patient responded well to prednisone but upon cessation of the corticosteroid developed enlargement of the submandibular gland. The patient's serum Ig G4 was elevated at 23.9 g/L. It is important to maintain treatment,so the patient was again treated with prednisone and had a good response. Follow-up of Ig G4-related disease is thus necessary.

Clinical characteristics and outcome of autoimmune pancreatitis based on serum immunoglobulin G4 level:A single-center,retrospective cohort study

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.

Immunoglobulin G in non-alcoholic steatohepatitis predicts clinical outcome:A prospective multi-centre cohort study

BACKGROUND Autoimmune markers including plasma cells(PC),anti-smooth-muscle antibody(ASMA),anti-nuclear antibody(ANA),and raised immunoglobulin G(IgG)are commonly observed in non-alcoholic steatohepatitis(NASH),however their clinical significance is unknown.AIM To determine if autoimmune markers in NASH patients are independently associated with poorer clinical outcomes.METHODS Consecutive patients with biopsy proven NASH from Christchurch Hospital,New Zealand and Singapore General Hospital(SGH)were included between 2005 to 2016 in a prospective multi-centre cohort study.Patients with other causes of chronic liver disease were excluded.IgG>14 g/L or globulin fraction>50%,ANA≥1:40,SMA≥1:40 were considered positive.Multivariate analysis was performed to assess which markers were independently associated with mortality and hepatic decompensation.RESULTS Total 261 patients were included of which 201 were from SGH.The median age was 53 and 51.9%were male.Advanced fibrosis was present in 31.4%at diagnosis.PC,ASMA,ANA and raised IgG were observed in 13.1%,4.9%,27.8%and 30.1%of patients respectively.After multivariate analysis,elevated IgG[Hazard Ratio(HR)6.79,95%CI:2.93-17.15]and fibrosis stage(HR 1.37,95%CI:1.03-1.87)were found to be independently associated with increased risk of liver decompensation.Age(HR 1.06,95%CI:1.02-1.10)and elevated IgG(HR 3.79,95%CI:1.90-7.68)were independent factors associated with higher mortality risk.CONCLUSION Elevated IgG,rather than ANA,ASMA or plasma cells,is independently associated with increased risk of hepatic decompensation and mortality in NASH.It could hence be important for prognostication.

Successful treatment of tubulointerstitial nephritis in immunoglobulin G4-related disease with rituximab: A case report

BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that consisted of disorders that share particular clinical,serologic and pathologic properties.The common presentation of disease includes tumor-like swelling of involved organs and the histopathological findings are a lymphoplasmacytic infiltrate enriched with IgG4-positive plasma cells,and a variable degree of fibrosis that has a characteristic "storiform" pattern in biopsy specimens of tumorlike masses.Major presentations of this disease,which often affects more than one organ,include autoimmune pancreatitis,salivary gland disease (sialadenitis),orbital disease and retroperitoneal fibrosis.The steroid treatment is essential for the treatment of the disease however,other immunosuppressive drugs including cyclophosphamide or rituximab could be an option in resistant cases.CASE SUMMARY Herein,we reported a 34-year-old woman whom previously had diagnosed with asthma,rheumatoid arthritis and Sj?gren’s syndrome (SS) referred our nephrology department due to acute kidney failure development at the last rheumatology visit.After kidney biopsy she has been diagnosed with IgG4-RD and tubuluointerstitial nephritis.She had been accepted resistant to steroid,mycophenolate mofetil,methotrexate and azathioprine therapies due to receiving in last two years.She refused to receive cyclophosphamide due to potential gonadotoxicity of the drug.Thus,rituximab therapy was considered.She received 1000 mg infusion,15 d apart and 6 mo later it has been administered same protocol.After one year from the last rituximab dose serum creatinine decreased from 4.4 mg/dL to 1.6 mg/dL,erythrocyte sedimentation rate decreased from 109 mm/h to 13 mm/h [reference range (RR) 0-20],and Creactive protein decreased from 55.6 mg/L to 5 mg/L (RR 0–6).All pathologic lymph nodes and masses were also disappeared.CONCLUSION Patients with IgG4-RD usually misdiagnosed with rheumatologic diseases including systemic lupus erythematous or SS and also they were screened for the presence of malignancy.Rituximab could be an important treatment option in cases with steroid resistant tubulointerstitial nephritis in IgG4-RD.

An aortoduodenal fistula as a complication of immunoglobulin G4-related disease

Most primary aortoduodenal fistulas occur in the presence of an aortic aneurysm,which can be part of immunoglobulin G4(IgG4)-related sclerosing disease.We present a case who underwent endovascular grafting of an aortoduodenal fistula associated with a high serum IgG4 level.A 56-year-old male underwent urgent endovascular reconstruction of an aortoduodenal fistula.The patient received antibiotics and other supportive therapy,and the postoperative course was uneventful,however,elevated levels of serum IgG,IgG4 and C-reactive protein were noted,which normalized after the introduction of steroid therapy.Control computed tomography angiography showed no endoleaks.The primary aortoduodenal fistula may have been associated with IgG4-related sclerosing disease as a possible complication of IgG4-related inflammatory aortic aneurysm.Endovascular grafting of a primary aortoduodenal fistula is an effective and minimally invasive alternative to standard surgical repair.

Immunoglobulin G4-related kidney diseases: An updated review

This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.

IgG4相关肾病患者血清IgG4表达水平与肾功能、微炎症状态的相关性分析

目的分析IgG4相关肾病患者血清IgG4水平与肾功能、微炎症状态的相关性。方法选取内蒙古医科大学附属医院2018年1月至2022年8月收治的60例IgG4相关肾病患者记为研究组,另基于倾向性匹配法按照1∶1的比例选取60名健康志愿者记为对照组。2组受试者均检测血清IgG4水平、肾功能指标[血肌酐(serum creatinine,Scr)、血尿素氮(blood urea nitrogen,BUN)、估算肾小球滤过率(estimated glomerular filtration rate,eGFR)、24 h尿蛋白定量(24 h urinary total protein quantification,24 hUTP)]和微炎症状态[白细胞介素(interleukin,IL)6、超敏C反应蛋白(high-sensitivity C-reactive protein,hs-CRP)、肿瘤坏死因子α(tumor necrosis factor-α,TNF-α)]。比较2组受试者血清IgG4、肾功能及微炎症状态指标;Pearson法分析研究组中患者血清IgG4水平与肾功能、微炎症状态的相关性;Logistic回归分析确定患者肾功能不全的危险因素。结果研究组血清IgG4[(67.75±18.63)mg/L比(6.54±1.19)mg/L]、Scr[(182.75±32.69)μmol/L比(75.40±15.61)μmol/L]、BUN[(10.15±2.90)mmol/L比(5.28±1.04)mmol/L]、IL-6[(5.18±1.04)ng/L比(0.40±0.05)ng/L]、hs-CRP[(24.69±5.06)mg/L比(4.58±0.92)mg/L]、TNF-α[(6.85±1.79)μg/L比(1.18±0.21)μg/L]和24 hUTP[(182.75±32.69)mg比(89.75±12.36)mg]水平均高于对照组(P<0.05),eGFR低于对照组[(90.10±9.87)mL·min^(-1)·(1.73 m^(2))-1比(104.36±20.15)mL·min^(-1)·(1.73 m^(2))^(-1),P<0.05];研究组血清IgG4水平与Scr、BUN、24 hUTP、IL-6、hs-CRP、TNF-α均呈正相关(r=0.586、0.543、0.575、0.602、0.596、0.574,P<0.05),与eGFR呈负相关(r=-0.602,P=0.001);研究组肾功能不全发生率为78.33%,病程、合并高血压、血清IgG4水平均是肾功能不全发生的影响因素(OR=3.190、2.401、3.466,P<0.05)。结论IgG4相关肾病患者血清IgG4升高,且与肾功能、微炎症状态均有关,病程、合并高血压、血清IgG4水平均是肾功能不全发生的影响因素。

Immunoglobulin G4 associated autoimmune cholangitis and pancreatitis following the administration of nivolumab:A case report

BACKGROUND Immune checkpoint inhibitors have significantly improved survivals for an increasing range of malignancies but at the cost of several immune-related adverse events,the management of which can be challenging due to its mimicry of other autoimmune related disorders such as immunoglobulin G4(IgG4)related disease when the pancreaticobiliary system is affected.Nivolumab,an IgG4 monoclonal antibody,has been associated with cholangitis and pancreatitis,however its association with IgG4 related disease has not been reported to date.CASE SUMMARY We present a case of immune-related pancreatitis and cholangiopathy in a patient who completed treatment with nivolumab for anal squamous cell carcinoma.Patients IgG4 levels was normal on presentation.She responded to steroids but due to concerns for malignant biliary stricture,she opted for surgery,the pathology of which suggested IgG4 related disease.CONCLUSION We hypothesize this case of IgG4 related cholangitis and pancreatitis was likely triggered by nivolumab.

Clinical characteristics and outcomes of autoimmune pancreatitis based on serum immunoglobulin G4 levels:A single-center,retrospective cohort study

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.

Novel mechanism of hepatobiliary system damage and immunoglobulin G4 elevation caused by Clonorchis sinensis infection

Clonorchis sinensis infection is still a major public health problem.It is estimated that more than 15 million people worldwide are infected,especially in Northeast China,Taiwan,South Korea,and North Vietnam.The detection of Clonorchis sinensis eggs in feces and bile is still the only gold standard for the diagnosis of Clonorchis sinensis infection,and new detection methods are needed to improve the detection rate.After Clonorchis sinensis invades the human body,it mainly parasitizes the hepatobiliary tract.Therefore,it is closely related to hepatobiliary diseases such as cholangitis,bile duct stones,liver fibrosis,and cholangiocarcinoma.The increase in immunoglobulin G4(IgG4)caused by Clonorchis sinensis infection is rare and there are few reports about the relevant mechanism.It may be related to the inflammatory factors interleukin(IL)-4,IL-10,and IL-13 produced by human phagocytes,T cells,B cells,and other immune cells in the process of resisting the invasion of Clonorchis sinensis.However,this finding still needs further clarification and confirmation.This article reviews the epidemiology,clinical manifestations,serology,imaging,pathogenic mechanism,and control measures of Clonorchis sinensis infection to help establish the diagnostic process for Clonorchis sinensis.We report novel mechanisms of IgG4 elevation due to Clonorchis sinensis infection to provide more experience and a theoretical basis for clinical diagnosis and treatment of this infection.

EB病毒IgM、IgG及DNA检测在儿童呼吸道感染性疾病中的诊断研究

目的探析爱泼斯坦-巴尔病毒(EB病毒,EBV)免疫球蛋白(Ig)M、IgG及脱氧核糖核酸(DNA)检测在儿童呼吸道感染性疾病中的诊断价值。方法选取400例呼吸道感染性疾病患儿作为观察组,同时选取100例无呼吸道感染患儿作为对照组。对两组患儿EBV-IgM、EBV-IgG及EBV-DNA进行检测。比较观察组不同年龄段患儿EBV-IgM、EBV-IgG及EBV-DNA阳性率,两组EBV-IgM、EBV-IgG及EBV-DNA检测结果;分析各项指标的诊断价值。结果观察组不同年龄段患儿EBV-IgM、EBV-IgG阳性率比较,差异具有统计学意义(P<0.05);观察组不同年龄段患儿EBV-DNA阳性率比较无明显差异(P>0.05)。EBV-IgM检测中,观察组化学发光值(11.39±2.12)高于对照组的(9.54±1.35),阳性率50.50%(202/400)高于对照组的5.00%(5/100),差异有统计学意义(P<0.05);EBV-IgG检测中,观察组吸光度值(11.33±2.15)高于对照组的(9.03±1.06),阳性率58.00%(232/400)高于对照组的12.00%(12/100),差异有统计学意义(P<0.05);EBV-DNA检测中,观察组循环数Ct值(34.13±4.88)低于对照组的(43.75±11.62),阳性率57.00%(228/400)高于对照组的6.00%(6/100),差异均具有统计学意义(P<0.05)。EBV-DNA对呼吸道感染性疾病患儿的诊断价值最高[曲线下面积(AUC)=0.644],其次依次是EBV-IgM(AUC=0.618)、EBV-IgG(AUC=0.584),EBV-IgM对呼吸道感染性疾病患儿的诊断特异度最高,为96.12%。结论EBV-IgM、EBV-IgG、EBV-DNA检测在儿童呼吸道感染性疾病的诊断中具有重要的指导意义。