Immunoglobulin A glomerulonephropathy:A review

In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.

Airway management of a patient with linear immunoglobulin A bullous dermatosis:A case report

BACKGROUND There is limited literature on managing the airway of patients with linear immunoglobulin A(IgA)bullous dermatosis,a rare mucocutaneous disorder that leads to the development of friable bullae.Careful clinical decision making is necessary when there is a risk of bleeding into the airway,and a multidisciplinary team approach may lead to decreased patient morbidity during these high-risk scenarios,especially when confronted with an unusual cause for bleeding.CASE SUMMARY A 45-year-old African American female presented to our ambulatory surgical center for right corneal transplantation due to corneal perforation after blunt trauma in the setting of cicatricial conjunctivitis and diffuse corneal neovascularization from linear IgA bullous dermatosis.The diagnosis of IgA dermatosis was recent,and the patient had been lost to follow-up.The severity of the disease and extent of airway involvement was unknown at the time of the surgery.Significant airway bleeding was noticed upon intubation and the otorhinolaryngology team had to be called to the operating room.The patient required transfer to the intensive care unit where a multidisciplinary team was involved in her case.The patient was extubated on postoperative day 4.CONCLUSION A multidisciplinary approach to treating this disease is the best course of action before a surgical procedure.In our case,key communication between the surgery,anesthesia,and dermatology teams led to the quick and safe treatment of our patient’s disease.Ambulatory surgery should not be considered for these cases unless they are in full remission and there is no mucous membrane involvement.

Immunoglobulin A vasculitis nephritis:Current understanding of pathogenesis and treatment

The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.

Novel COL4A3 synonymous mutation causes Alport syndrome coexistent with immunoglobulin A nephropathy in a woman:A case report

BACKGROUND Alport syndrome(AS)is an inherited disease of the glomerular basement membrane caused by mutations in genes encodingα3,α4,orα5 chains of type IV collagen.It manifests with hematuria or proteinuria,which is often accompanied by hearing impairments and ocular abnormalities.Histopathologically,AS shows mesangial proliferation and sometimes incidental immunoglobulin A(IgA)deposition.Hematuria or proteinuria is also a common presentation in patients with IgA nephropathy that makes it difficult to differentially diagnose AS and IgA nephropathy solely based on these clinical and pathological features.CASE SUMMARY Herein,we present the case of a 59-year-old female patient who was admitted to our hospital with persistent microscopic hematuria and occasional proteinuria that had lasted for>2 years.This patient had a familial history of renal disease and was diagnosed with autosomal dominant AS(ADAS)and IgA nephropathy based on the findings of renal biopsy as well as genetic testing performed using whole-exome sequencing,which suggested that the patient carried a novel heterozygous variation(c.888G>A:p.Gln296Gln)in the COL4A3 gene that enriches the mutation spectrum of ADAS.The proband received an angiotensin receptor blocker therapy after a definitive diagnosis was established.After one year of therapy,a significant reduction in proteinuria was observed.The number of microscopic red blood cells per high-power field decreased to one-quarter of the baseline levels.Renal function also maintained well during the follow-up.CONCLUSION Our case highlights the significance of performing kidney biopsy and genetic testing in the diagnosis of AS and familial IgA nephropathy.

Autoimmune hepatitis in a patient with immunoglobulin A nephropathy:A case report

BACKGROUND Immunoglobulin A nephropathy(IgAN)is the most commonly encountered glomerular disease in Asian countries.It has a broad clinical presentation,and it is frequently associated with other conditions.Chronic liver disease is well recognized as the leading cause of secondary IgAN.However,cases of IgAN associated with autoimmune hepatitis(AIH)have seldom been reported.CASE SUMMARY A 63-year-old Korean woman was admitted to Pusan National University Hospital for an evaluation of abdominal pain and elevated liver enzymes.Two weeks prior,she had presented to our hospital with proteinuria of approximately 1350 mg/d and hematuria and was diagnosed with IgAN.Autoimmune profiles were highly positive for antinuclear antibodies,and symptoms related to portal hypertension including ascites and peripheral edema were present.A diagnosis of AIH was made according to the simplified scoring system of the International Autoimmune Hepatitis Group.Despite immunosuppression with prednisolone and azathioprine,rapid deterioration of liver function led to end-stage liver disease.After a living-donor liver transplantation,liver function gradually improved,and she had maintained stable liver and kidney function at the six months follow-up.CONCLUSION Cases of secondary IgAN with chronic liver disease have been frequently reported in the literature but are rarely associated with AIH.We encountered an IgAN patient with concurrent progressive liver failure due to AIH.

Correlations of Serological Markers with Development of Systemic Involvement in Adult Immunoglobulin A Vasculitis:A Retrospective Study of 259 Patients in Central China

Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identification and prevention for patients prone to develop systemic involvement are essential.The purpose of this study was to explore the correlations of common serological markers with the development of systemic involvement in adult IgAV.Methods:A retrospective analysis was performed for adult IgAV patients,who were hospitalized in Wuhan Union Hospital between January 2016 and December 2019.A total of 259 patients were enrolled,and the pre-treatment serological markers were comprehensively assessed.Results:In the present study,49.0% and 33.2% of patients developed renal and gastrointestinal(GI)involvement,respectively.Furthermore,the elevated levels of white blood cells count,D-Dimer(D-D),C-reactive protein(CRP)and neutrophil granulocyte ratio(NE%)>60% were significantly associated with GI involvement in the univariate analysis,while the decrease in high density lipoprotein level,and the elevated D-D and CRP levels were significantly associated with renal involvement(P<0.05).Moreover,a prediction model that combined multiple markers was established by performing a logistic regression analysis,and this presented a more favorable value of prediction than the individual serological markers.Conclusion:The present study suggests that common serological markers have close correlations with systemic involvement in adult IgAV,and that the establishment of a prediction model for systemic involvement may be helpful in facilitating personalized therapeutic strategies and clinical management for IgAV patients.

Comparison of total immunoglobulin A levels in different samples in Leghorn and broiler chickens

Objective: To standardize an ELISA protocol to quantify total immunoglobulin A(IgA)from different biological samples.Methods: Two independent experiments were conducted. In Experiment 1, total IgA levels were quantified from the lachrymal fluid, tracheal swab, and cloacal swab at various time points from Days 30 to 89 in white Leghorn chickens. Experiment 2 was conducted to evaluate the effect of 50 or 500 ppb of aflatoxin B1(AFB1) on total IgA quantified in samples from the lachrymal fluid, tracheal swab, gut content and cloacal swab in broiler chickens at 21 days of age.Results: Lachrymal fluid contained the highest level of IgA; however, the sampling procedure was time consuming and stressful to the bird, and the sample volume depends largely on the size of the chicken. Cloacal swabs also contained a high concentration of IgA; this sampling procedure was faster than lachrymal fluid sampling and was not affected by the age of the bird. Tracheal sampling was more difficult than cloacal sampling; the age of the bird limited the sampling, and the IgA concentration was the lowest detected at all sampling ages. 500 ppb of AFB1 significantly reduced total IgA concentration in the gut content compared with control or 50 ppb of AFB1 treated groups.Interestingly, a significant reduction in total IgA was also observed in those chickens that received 50 ppb of AFB1 in gut rinse when compared with cloacal swabs.Conclusions: The results of this study suggest that cloacal swab is an easy and reliable way to evaluate mucosal IgA concentration in both Leghorn and broiler chickens.

Delayed diffuse lamellar keratitis after small-incision lenticule extraction related to immunoglobulin A nephropathy:A case report

BACKGROUND Diffuse lamellar keratitis(DLK)is a complication of laser-assisted in situ keratomileusis(LASIK).This condition can also develop after small-incision lenticule extraction(SMILE)with a distinctive appearance.We report the case involving a female patient with delayed onset DLK accompanied by immunoglobulin A(IgA)nephropathy.CASE SUMMARY A 22-year-old woman was referred to our department for DLK and a decline in vision 1 mo after undergoing SMILE.The initial examination showed grade 2 DLK in the flap involving the central visual axis of the right eye.She was immediately administered with a large dose of a topical steroid for 30 d.However,the treatment was ineffective.Her vision deteriorated from 10/20 to 6/20,and DLK gradually worsened from grade 2 to 4.Eventually,interface washout was performed,after which her vision improved.DLK completely disappeared 2 mo after washout.Six months after SMILE,the patient was diagnosed with IgA nephropathy due to a 4-year history of interstitial hematuria.CONCLUSION DLK is a typical complication of LASIK but can also develop after SMILE.Topical steroid therapy was ineffective in our patient,and interface washout was required.IgA nephropathy could be one of the factors contributing to the development of delayed DLK after SMILE.

Unusual Cause of Stridor in an Adult Man, Selective Immunoglobulin A Deficiency

The common causes of stridor in adults are abscesses or swelling of the upper airway, tumors, paralysis, or malfunction of vocal cords. Tracheitis due to immunoglobulin deficiency may be a rare explanation for stridor in adults, although occasionally reported in children. We report an adult man having stridor secondary to isolated immunoglobulin A deficiency. We did an in-depth review of the literature to seek out no reported cases of stridor thanks to immunoglobulin deficiency in adults. This case underlines the very fact that a standard symptom like stridor rarely occurs thanks to uncommon causes. This case is exclusive because it reports the presence of stridor associated with isolated immunoglobulin A deficiency in an adult patient. The explanation for the stridor was an intraluminal narrowing of the upper part of the extrathoracic trachea.

The influence of immunomodulator on the immunoglobulin and T cell subsets in children with intractable epilepsy

Objective:To observe the influence of immunomodulator on the immunoglobulin and T cell subsets in children with intractable epilepsy.Method:A total of 82 children with intractable epilepsy in our hospital were selected and randomly divided into 2 groups: the control group (41 cases) and the observation group (41 cases). Routine antiepileptic drugs were given to the control group. Medication regimen was that more than 2 kinds of anti epileptic drugs were be combined used. But the immunomodulator wasn't used. Treatment of immune globulin was given to the control group on the basis of observation group. By taking the 400 mg/kg/d as the standard of dosage, for 5 d every course of treatment. One course of treatment was carried out every month, 3 months in total. The changes of IgA, IgG, IgM and CD3+, CD4+, CD8+, CD4+/CD8+ were compared in 2 groups before and after treatment.Result: The comparison of IgA, IgG, IgM in the two groups before treatment was not statistically significant. After treatment, IgA, IgG in observation group were significantly higher than that before treatment and the difference was statistically significant. However, there was no significant difference on the IgM. There was no significant difference on the IgA, IgG, IgM in the control group compared with that before treatment. IgA, IgG in observation group was significant higher than that in the control group. The comparison of IgM between 2 groups was not statistically significant. The comparison of CD3+, CD4+, CD8+, CD4+/CD8+ in the two groups before treatment was not statistically significant. After treatment, CD3+, CD4+, CD4+/CD8+ in observation group were significantly higher than that before treatment;CD8+ in observation group was significantly lower than that before treatment. The difference was statistically significant. There was no significant difference on the CD3+, CD4+, CD8+, CD4+/CD8+ in the control group compared with that before treatment. CD3+, CD4+, CD4+/CD8+ in observation group was significant higher than that in the control group. CD8+ in observation group was significant lower than that in the control group. The difference was statistically significant.Conclusion:Compared with using routine antiepileptic drugs , application of immune globulin as an immunomodulator combined with conventional antiepileptic drug in children with refractory epilepsy can effectively improve the expression of IgA, IgG, IgM and T cell subsets, which has a positive effect on the immune function of the children.

Successful treatment of pyoderma gangrenosum with concomitant immunoglobulin A nephropathy: A case report and review of literature

Pyoderma gangrenosum(PG)is an uncommon ulcerative cutaneous condition of an unknown etiology and is often associated with immune diseases.However,PG rarely shows visceral involvement,especially in the kidney.A 20-year-old female presented with pedal edema and skin ulceration of both lower limbs.The skin lesion began as an erythematous plaque and then became a blister.She also complained of abdominal distension and a decreasing urine volume.Laboratory data showed high proteinuria,hypoalbuminemia and hyperlipidemia.Her skin and kidney were biopsied.The pathological results indicated PG and immunoglobulin A(IgA)nephropathy.The patient was finally cured with prednisolone in combination with cyclosporine A(CsA).

Intravenous immunoglobulin accompanied with high-dose methylprednisolone therapy for 17 children with anti-N-methyl-D-aspartate receptor encephalitis:Clinic and nursing

Objective:An increasing number of pediatric patients are being diagnosed with anti-N-methyl-Daspartate receptor(NMDAR)encephalitis,whose treatment requires immunotherapy through nursing interventions.This study aimed to analyze the clinical features and long-term prognosis of pediatric anti-NMDAR encephalitis and to gather nursing experiences of immunotherapy.Methods:Seventeen children diagnosed with anti-NMDAR encephalitis were admitted to the pediatric department.They were subjected to a therapy of intravenous immunoglobulin(IVIG)accompanied with high-dose methylprednisolone(HDMP).Multidisciplinary cooperation and intensive care were used to manage them.The effects of nursing intervention and therapy were repeatedly assessed and analyzed throughout the course of treatment and recovery.Results:None of the patients manifested adverse drug reaction(ADR)during IVIG administration.At the first administration of HDMP,ADRs were promptly and efficiently treated in four patients(24%;i.e.,one case each of hyperglycosemia,hypertension,aggravated symptoms,and gastrointestinal bleed).Two patients underwent rehabilitation,and six patients received hyperbaric oxygenation during hospitalization.Nine patients with indwelling gastric tubes experienced four times of unplanned extubation.Hospital stay ranged from 11 days to 59 days,with the mean duration of 26 days.Discharge evaluation revealed that 16 patients who scored 0e2 on the modified Rankin scale presented obvious remission,and one patient who had a mRS score of 4 exhibited less improvement.The mRS scores of hospitalization,discharge,and six-month follow-up displayed statistically significant differences.Conclusions:Nursing interventions of immunotherapy ensures the security of IVIG administration.Multidisciplinary cooperation promotes remission.Our findings can serve as reference for healthcare teams.

A case of Bickerstaff brainstem encephalitis successfully treated with intravenous immunoglobulin and methylprednisolone after unsuccessful immunoadsorption plasmapheresis

Bickerstaff brainstem encephalitis (BBE) is a rare post-infectious neurological syndrome for which an effective treatment strategy has not been established. Here, we report a case of a 71-year-old male who suffered from an upper respiratory tract infection, and 7 days later, developed numbness of the bilateral upper and lower limbs, unsteady gait and dysarthria. Brain magnetic resonance imaging was normal, nerve conduction study and cerebral spinal fluid analysis were nonspecific. Based on the clinical features, we tentatively diagnosed Guillain-Barré syndrome and started immunoadsorption plasmapheresis. However, consciousness progressively declined to coma level within 10 days. Electroencephalogram showed diffuse slowing, and auditory evoked brainstem response (ABR) demonstrated absence of waves II, III and V. Serum anti-GQ1b IgG autoantibody and anti-GM1b IgG autoantibody were negative. Subsequently, we diagnosed BBE, and clinical symptoms resolved after treatment with intravenous immunoglobulin and methyllprednisolone. On day 62, neurological symptoms were remarkably alleviated with an improvement in ABR. Our observations suggest that immunoadsorption plasmapheresis should be used only when anti-ganglioside antibodies are detected. Combination therapy with intravenous immunoglobulin and methylprednisolone or plasma exchange?is recommended as initial therapy.

A successful birth of severe secondary recurrent miscarriage case after a decline of phosphatidylserine-dependent anti-prothrombin antibody by intravenous immunoglobulin administration

A 33 years old woman was referred to our hospital since her sixth pregnancy had been revealed. In fact, at 19 years of age she had diagnosed as having systemic lupus erythematosus without organ failure. In addition, she had a past history of uncontrollable severe pregnancy-induced hypertension occurred during the second pregnancy, resulting in extremely premature delivery and following postpartum HELLP syndrome. It was so severe that we employed administration of dexamethasone and plasma exchange to ameliorate a life-threatening situation. In the course of her recovery it was revealed that she had been complicated with antiphospholipid antibodies, and at the same time we observed that phosphatidylserine-dependent anti-prothrombin antibody IgG levels were declining as her condition was getting better. There-after, she became pregnant three times, but all pregnancies ended in miscarriage despite administration of prednisolone and anticoagulant therapy. Therefore, we realized that her recurrent miscarriages could not be prevented with generally acceptable therapies, so we tried intravenous immunoglobulin shortly after fetal heart beats were detected. In fact, her sixth pregnancy was going well, but we had to terminate it at the 35th week of gestation due to the onset of HELLP syndrome-like condition. However, she could achieve an almost intact pregnancy outcome without neonatal complications or persistently worsening postpartum HELLP syndrome-like condition. Considering the etiologic relation overlapping between systemic lupus erythematosus, antiphospholipid syndrome and recurrent miscarriage, intravenous immunoglobulin can be one of the treatment options for severe secondary recurrent miscarriage, although the evidence of the treatment is always certain. In addition, a decline of phosphatidylserine-dependent anti-prothrombin antibody IgG levels we observed in this case may represent its therapeutic immunomodulatory effects.

Effect of laparoscopic hepatectomy on growth factor, immunoglobulin and related factors in patients with hepatocellular carcinoma

Objective:To compare the effects of laparoscopic surgery and traditional open surgery on serum growth factor, immunoglobulin and related factors in patients with hepatocellular carcinoma after operation.Methods: 60 patients with hepatocellular carcinoma admitted to our hospital from 2014 to 2018 were randomly divided into the control group and the observation group, with 30 cases in each group. Patients in the control group underwent traditional open radical hepatectomy, while patients in the observation group underwent laparoscopic radical hepatectomy. Before and after the operation, the levels of Alpha-alanine aminotransferase (ALT), aspartate aminotransferase (AST), vascular endothelial growth factor (VEGF), acidic fibroblast growth factor (aFGF), basic fibroblast growth factor (bFGF), immunoglobulin A (IgA), immunoglobulin M (IgM), immunoglobulin G (IgG), matrix metalloproteinase-13 (MMP-13), Procalcitonin (PCT) and C-reactive protein (CRP) were compared between the patients in the two groups.Results:The levels of ALT, AST, VEGF, aFGF, bFGF, IgA, IgM, and IgG of patients in the control group were significantly decreased after the operation, but the levels of MMP-13, PCT and CRP increased significantly. The serum levels of ALT, AST, VEGF, aFGF, bFGF, IgA, IgM, and IgG of patients in the observation group were significantly decreased after operation, but the levels of MMP-13, PCT and CRP increased significantly. The above indexes of patients in the observation group were better than those in the control group (P<0.05).Conclusion: Laparoscopic hepatectomy has a good clinical effect. It can significantly reduce the level of growth factor in serum of patients. It has less damage to immune function than open surgery and can reduce the level of inflammatory stress caused by operation.

Effect of sublingual dust mite drops on pulmonary function, immunoglobulin and T lymphocyte subsets in patients with allergic rhinitis

Objective:To investigate the effects of sublingual dust mite drops on Pulmonary function, immunoglobulin and T lymphocyte subsets in patients with allergic rhinitis.Methods:One hundred and ten patients with allergic rhinitis treated in our hospital from June 2014 to December 2016 were retrospectively analyzed. According to the treatment methods, the patients were divided into control group and observation group, with 53 cases in control group and 57 cases in observation group. The patients were given oral kastitium and risperidone nasal spray. The observation group was given sublingual dust mite droplet on the basis of the control group. Afterwards, the Pulmonary function, immunoglobulin and T lymphocyte subsets Group level testing.Results: After treatment, the levels of FEV1, FVC and PEF increased significantly in the two groups. After treatment, the levels of FEV1, FVC and PEF in the observation group were (2.97±0.09) L, (3.96±0.07) L and (6.61±0.03) L/S, were significantly higher than the control group;After treatment, the levels of sIgE and T-IgE in both groups were significantly decreased, sIgG4 levels were significantly increased,After treatment, the levels of sIgE and T-IgE in the observation group were (12.22±1.08) kUA/L and (155.32 ± 9.51) kUA/L, respectively, which were significantly lower than those in the control group,The level of sIgG4 was (434.17±12.31) mg/L, which was significantly higher than that of the control group;After treatment, the levels of CD3+, CD4+ and CD4+/CD8+ in both groups were significantly higher than those before treatment, and the levels of CD8+ in the two groups were significantly lower than those before treatment. The levels of CD3+, CD4+ and CD4+/CD8 in the two groups were (65.90±2.11)%, (39.18±0.33)% and (1.75±0.07), respectively, were significantly higher than that of the control group after treatment, the level of CD8+ was (24.13±0.77)%, which was significantly lower than that of the control group Significance.Conclusion: Sublingual dust mite drops can effectively improve pulmonary ventilation in patients with allergic rhinitis, regulate patient immune function, a significant effect, worthy of further clinical application.

Can serum immunoglobulin G4 levels and age serve as reliable predictors of relapse in autoimmune pancreatitis?

We are writing in response to the paper published in the World Journal of Gastroenterology by Zhou et al.The authors identified higher serum immunoglobulin(Ig)G4 levels and age over 55 years as independent risk factors for disease relapse.Despite notable strengths,it is crucial to address potential biases.Firstly,the cohort study included 189 patients with autoimmune pancreatitis(AIP)type 1(with higher IgG4 seropositivity and higher relapse)and 24 with type 2(with lower IgG4 seropositivity and lower relapse).Consequently,most,if not all,AIP type 2 patients were assigned to the normal group,possibly inflating the association of higher serum IgG4 levels with relapse and potentially exaggerating the association of older age with relapse.Secondly,the authors did not provide sufficient details regarding AIP diagnosis,such as the ratio of definitive vs probable cases and the proportion of biopsies.In cases where histological evidence is unavailable or indeterminate,AIP type 2 may be misdiagnosed as definitive type 1,and type 1 may also be misdiagnosed as probable type 2,particularly in cases with normal or mildly elevated serum IgG4 levels.Lastly,in this retrospective study,approximately one-third of the consecutive patients initially collected were excluded for various reasons.Accordingly,the impact of nonrandom exclusion on relapse outcomes should be carefully considered.In conclusion,the paper by Zhou et al offers plausible,though not entirely compelling,evidence suggesting a predictive role of elevated serum IgG4 levels and advanced age in AIP relapse.The foundation for future investigations lies in ensuring a reliable diagnosis and accurate disease subtyping,heavily dependent on obtaining histological specimens.In this regard,endoscopic ultrasound-guided fine-needle biopsy emerges as a pivotal component of the diagnostic process,contributing to mitigating biases in future explorations of the disease.

Serum Immunoglobulin Concentrations in Juvenile Idiopathic Arthritis Cases during Active and Inactive Disease States

Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Both the humoral and cell mediated immunities are involved in the pathogenesis of JIA. It is reported that overall immunoglobulin levels in JIA patients are significantly higher than their control during the active state of disease. Methodology: This prospective observational study was conducted over a period of 18 months All the newly diagnosed oligo-articular and poly-articular JIA patients having active disease were included by purposive sampling. Data were collected by a semi-structured predesigned questionnaire. Result: Most of the study subjects (57.6%) belonged to age group > 3 - 9 years. Oligo JIA was diagnosed in 66.7% and poly JIA in 33.3% of JIA children. The difference in mean (±SD) ESR (33.52 ± 21.29 and 15.09 ± 7.71 mm in 1st hour) at active and inactive states was highly significant. Mean (±SD) difference of IgG, IgM and IgA in active and inactive states of disease were highly significant. Conclusion: Higher and abnormal levels of immunoglobulin (IgG, IgM, and IgA) were present among JIA patients in active disease state which became normal during inactive disease state after treatment.

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.

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.