Humans ; IgA Vasculitis/therapy* ; Vasculitis/therapy* ; Immunoglobulin A

This study used UPLC-TQ-MS technology to replicate a Henoch-Schonlein purpura(HSP) model in rats by administering warm drugs by gavage and injecting ovalbumin with Freund's complete adjuvant emulsion. The distribution differences and characteristics of eight major components(ferulic acid, caffeic acid, neochlorogenic acid, cryptochlorogenic acid, benzoyl oxypaeoniflorin, tracheloside, loganin, and paeoniflorin) in rat liver, lung, heart, spleen, and kidney tissues were determined after oral administration of the Liangxue Tuizi Mixture at a dose of 42 g·kg~(-1) in both normal physiological and HSP states at 0.5, 1, 2, 6, and 12 hours. The results showed that the distribution patterns of the eight components of Liangxue Tuizi Mixture in the tissues of normal and HSP model rats were different. The main component, paeoniflorin, in Moutan Cortex and Paeoniae Radix Alba had higher content in all tissues. The eight components were predominantly distributed in the liver, lung, and kidney tissues, followed by spleen and heart tissues.
Rats ; Animals ; IgA Vasculitis/drug therapy* ; Monoterpenes ; Administration, Oral ; Liquid Chromatography-Mass Spectrometry

OBJECTIVES: To investigate the clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Schönlein purpura nephritis (DEP-HSPN). METHODS: A retrospective analysis was performed on the clinical, pathological, and prognosis data of 44 children with DEP-HSPN and 765 children without DEP-HSPN. The children with DEP-HSPN were diagnosed by renal biopsy in Jiangxi Provincial Children's Hospital from January 2006 to December 2021. RESULTS: Among the 809 children with purpura nephritis, 44 (5.4%) had DEP-HSPN, with a mean age of (8±3) years, and there were 29 boys (65.9%) and 15 girls (34.1%). Compared with the non-DEP-HSPN group, the DEP-HSPN group had a significantly shorter time from onset to renal biopsy and a significantly higher proportion of children with respiratory infection or gross hematuria, and most children had nephrotic syndrome. The DEP-HSPN group had significantly higher levels of 24-hour urinary protein, urinary protein grading, microscopic hematuria grading, serum creatinine, and blood urea nitrogen and significantly lower levels of serum albumin and complement C3 (P<0.05). The DEP-HSPN group had a higher pathological grading, with predominant deposition of IgA in the mesangial area and capillary loops, and higher activity scores in the modified semi-quantitative scoring system (P<0.05). The Kaplan-Meier survival analysis showed that there was no significant difference in the renal complete remission rate between the two groups (P>0.05). CONCLUSIONS Children with DEP-HSPN have a rapid onset, severe clinical manifestations and pathological grading, and high activity scores in the modified semi-quantitative scoring system. However, most of the children with DEP-HSPN have a good prognosis, with a comparable renal complete remission rate to the children without DEP-HSPN.
Male ; Female ; Humans ; Child ; Child, Preschool ; Hematuria ; IgA Vasculitis ; Retrospective Studies ; Prognosis ; Nephritis

OBJECTIVES: To investigate the difference in the therapeutic effect of mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in children with Henoch-Schönlein purpura nephritis (HSPN) of different age groups. METHODS: A retrospective analysis was conducted on the clinical data of 135 children with HSPN who were treated with MMF or CTX in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from October 2018 to October 2020. According to the immunosuppressant used, they were divided into two groups: MMF group and CTX group, and according to the age, each group was further divided into two subgroups: ≤12 years and >12 years, producing four groups, i.e, the ≤12 years MMF subgroup (n=30), the >12 years MMF subgroup (n=15), the ≤12 years CTX subgroup (n=71), and the >12 years CTX subgroup (n=19). All children were followed up for at least 12 months, and the above groups were compared in terms of clinical outcomes and the incidence rate of adverse reactions. RESULTS: There was no significant difference in the complete response rate between the MMF group and the CTX group after 3, 6, and 12 months of treatment (P>0.05). There were no significant difference in the complete response rate and the incidence rate of adverse reactions between the >12 years MMF subgroup and the ≤12 years MMF subgroup at 3, 6, and 12 months of treatment (P>0.05). The >12 years CTX subgroup had a significantly lower complete response rate than the ≤12 years CTX subgroup at 6 and 12 months of treatment (P<0.05). The >12 years CTX subgroup had a significantly higher incidence rate of adverse reactions than the >12 years MMF subgroup (P<0.05). CONCLUSIONS The efficacy and adverse reactions of MMF are not associated with age, but the efficacy of CTX is affected by age, with a higher incidence rate of adverse reactions. CTX should be selected with caution for children with HSPN aged >12 years.
Child ; Humans ; Mycophenolic Acid/adverse effects* ; IgA Vasculitis/drug therapy* ; Retrospective Studies ; Cyclophosphamide/adverse effects* ; Immunosuppressive Agents/adverse effects* ; Vasculitis/drug therapy* ; Nephritis/complications*

OBJECTIVES: To study the effect of vaccination on the short-term risk of immunoglobulin A vasculitis (IgAV) in children. METHODS: A retrospective analysis was conducted on the general data and the vaccination history within one year prior to onset in children with IgAV hospitalized in the Children's Hospital Affiliated to Zhengzhou University from November 2021 to January 2023. Vaccine exposure rates in the risk period (3 months prior to IgAV onset) and the control period were compared by autocontrol-case crossover analysis, and the odds ratio and 95% confidence interval (95%CI) were calculated. A sensitivity analysis for the one-month and two-month risk periods was conducted. RESULTS: A total of 193 children with IgAV were included, with a median age of 7.0 years. Among the 193 children, 36 (18.7%) received at least one dose of the vaccine within 1 year prior to IgAV onset, and 14 (7.3%) received at least one dose of the vaccine during the 3-month risk period. Compared to the unvaccinated IgAV group, the vaccinated IgAV group had a significantly younger age of onset (P<0.05). There were no significant differences in the proportions of children with gastrointestinal involvement, renal involvement, and joint involvement between the two groups (P>0.05). The odds ratio for developing IgAV after receiving any type of vaccine within 3 months prior to IgAV onset was 2.08 (95%CI: 0.82-5.27, P>0.05). Further sensitivity analysis for the 1-month and 2-month risk periods demonstrated that the odds ratios for developing IgAV after receiving any type of vaccine were 2.74 (95%CI: 0.72-10.48, P>0.05) and 2.72 (95%CI: 0.95-7.77, P>0.05), respectively. CONCLUSIONS Vaccination dose not increase the risk of IgAV, nor does it exacerbate clinical symptoms in children with IgAV.
Humans ; Child ; Retrospective Studies ; Immunoglobulin A ; IgA Vasculitis ; Vaccination ; Vaccines

Immunoglobulin A vasculitis (IgAV), also known as Henoch-Schönlein purpura, has complex etiology and pathogenesis which have not been fully clarified. The latest research shows that SARS-CoV-2 and related vaccines, human papilloma vaccine, and certain biological agents can also induce IgAV. Most studies believe that the formation of galactose-deficient IgA1 (Gd-IgA1) and Gd-IgA1-containing immune complex plays a crucial role in the pathogenesis of IgAV. It is hypothesized that the pathogenesis of IgAV is associated with the binding of IgA1 to anti-endothelial cell antibodies. In addition, genetics also constitutes a major focus of IgAV research. This article reviews the new advances in the etiology of IgAV and summarizes the role of Gd-IgA1, Gd-IgA1-containing immune complex, anti-endothelial antibody, IgA1 conjugates, T lymphocyte immunity, and genetic factors in the pathogenesis of IgAV.
Humans ; IgA Vasculitis ; Antigen-Antibody Complex ; Immunoglobulin A/genetics*

OBJECTIVE: To investigate the predictive value of complete blood count (CBC) and inflammation marker on the recurrence risk in children with Henoch-Schönlein purpura (HSP). METHODS: One hundred and thirty-three children with HSP admitted to Cangzhou Central Hospital from February 2017 to March 2019 were enrolled. The clinical data of the children were collected, at the time of admission CBC and C-reactive protein (CRP) were detected. After discharge, the children were followed up for 1 year, the clinical data of children with and without recurrence were compared, and multivariate logistic regression was used to analyze the risk factors affecting HSP recurrence. Receiver operating characteristic (ROC) curve should be drawn and the predictive value of CBC and CRP on HSP recurrence should be analyzed. RESULTS: In the follow-up of 133 children, 8 cases were lost and 39 cases recurred, with a recurrence rate of 31.20% (39/125). The age, skin rash duration, proportion of renal damage at the initial onset, percentage of neutrophils, percentage of lymphocytes, platelet count (PLT), mean platelet volume (MPV) and neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), MPV/PLT ratio (MPR), and CRP level of patients with recurrence were statistically different from those without recurrence (P <0.05). Multivariate logistic regression analysis showed that long skin rash duration, renal damage at the initial onset, increased PLR, high PLT, increased MPV and elevated CRP level were independent risk factors for recurrence in children with HSP (P <0.05). The ROC curve analysis showed that the area under the curve (AUC) of the combination of the four blood and inflammation marker (PLT, MPV, PLR and CPR) in the early prediction of HSP recurrence was 0.898, which was higher than the initial renal damage (AUC=0.687) and persistent skin rash time (AUC=0.708), with a sensitivity of 84.62% and a specificity of 83.72%. CONCLUSION Observation of CBC and CPR can predict the risk of HSP recurrence early and guide early clinical intervention.
Humans ; Child ; IgA Vasculitis ; Blood Cell Count ; Inflammation ; C-Reactive Protein ; Lymphocytes ; Neutrophils ; Exanthema ; Retrospective Studies

Ultra-performance liquid chromatography-quadrupole time of fight/mass spectrometry(UPLC-Q-TOF-MS) and UNIFI were employed to rapidly determine the content of the components in Liangxue Tuizi Mixture. The targets of the active components and Henoch-Schönlein purpura(HSP) were obtained from SwissTargetPrediction, Online Mendelian Inheritance in Man(OMIM), and GeneCards. A "component-target-disease" network and a protein-protein interaction(PPI) network were constructed. Gene Ontology(GO) functional annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway enrichment analysis were performed for the targets by Omishare. The interactions between the potential active components and the core targets were verified by molecular docking. Furthermore, rats were randomly assigned into a normal group, a model group, and low-, medium-, and high-dose Liangxue Tuizi Mixture groups. Non-targeted metabolomics was employed to screen the differential metabolites in the serum, analyze possible metabolic pathways, and construct the "component-target-differential metabolite" network. A total of 45 components of Liangxue Tuizi Mixture were identified, and 145 potential targets for the treatment of HSP were predicted. The main signaling pathways enriched included resistance to epidermal growth factor receptor tyrosine kinase inhibitors, phosphatidylinositol 3-kinase/protein kinase B(PI3K-AKT), and T cell receptor. The results of molecular docking showed that the active components in Liangxue Tuizi Mixture had strong binding ability with the key target proteins. A total of 13 differential metabolites in the serum were screened out, which shared 27 common targets with active components. The progression of HSP was related to metabolic abnormalities of glycerophospholipid and sphingolipid. The results indicate that the components in Liangxue Tuizi Mixture mainly treats HSP by regulating inflammation and immunity, providing a scientific basis for rational drug use in clinical practice.
Animals ; Rats ; IgA Vasculitis/drug therapy* ; Network Pharmacology ; Molecular Docking Simulation ; Phosphatidylinositol 3-Kinases ; Metabolomics

OBJECTIVE: To investigate the risk factors of different types of Henoch-Schönlein purpura (HSP) in Tibetan patients at high altitude, as to provide reference for correctly identifying high-risk patients. METHODS: A retrospective study was used to analyze the 304 HSP patients admitted to Tibet Autonomous Region People's Hospital from April 2014 to March 2022. The gender, age, allergic history, family history, clinical type, laboratory indexes (hemoglobin, platelet count, eosinophil, C-reactive protein (CRP), albumin, immunoglobulin G, immunoglobulin A, complement C3 and C4) were analyzed retrospectively. Univariate and multivariate Logistic regression analysis to screen for risk factors affecting different types of HSP. RESULTS: Renal HSP patients showed higher IgA [(9.2±1.7) g/L vs. (6.4±2.4) g/L, P=0.015], lower complement C3 [(203.3±21.6) mg/dL vs. (301.1±19.5) mg/dL, P=0.043], and complement C4 [(33.5±2.3) mg/dL vs. (53.0±7.2) mg/dL, P=0.032]. The patients with abdominal HSP showed lower levels of hemoglobin [(119.6±19.6) g/L vs. (146.6±47.3) g/L, P=0.038] and plasma albumin [24.8 (22.1, 33.9) g/L vs. 32.6 (24.6, 35.1) g/L, P=0.045]. The patients with articular HSP exhibited higher CRP [13.5 (0.2, 20.6) g/L vs. 7.5 (0.1, 15.2) g/L, P=0.036] and erythrocyte sedimentation rate (ESR) [24 (5, 40) mm/h vs. 15 (4, 30) mm/h, P=0.049]. Elevated IgA and decreased complement C4 were risk factors for renal HSP, anemia and decreased plasma albumin were risk factors for abdominal HSP, and elevated CRP was a risk factor for articular HSP. CONCLUSION The clinical characteristics of different types of HSP in plateau areas were different. Patients with high IgA, low complement C4, anemia, hypoalbuminemia, and significantly elevated CRP should be highly vigilant. Early and effective intervention can improve the clinical efficacy, avoid severe development, and improve the prognosis.
Humans ; Retrospective Studies ; Tibet/epidemiology* ; Complement C3/analysis* ; IgA Vasculitis/complications* ; Altitude ; Complement C4 ; C-Reactive Protein/analysis* ; Immunoglobulin A ; Risk Factors ; Anemia ; Hemoglobins/analysis* ; Serum Albumin/analysis*

Henoch-Schönlein purpura (HSP) is the most common form of vasculitis in children that is clinically characterized by the classic triad of palpable purpura, joint symptoms, and abdominal pain. A 6-year-old girl, one of fraternal twins, was admitted to the Pediatric Department, Universitas Airlangga with fever, rashes on legs and arms and intermittent mild abdominal pain. She had multiple purpuric rashes on her extremities, abdomen and buttocks. Laboratory investigation revealed immunoglobulin A level of 289.6 mg/dL. The patient was diagnosed as HSP vasculitis according to EULAR criteria and treated with intravenous methylprednisolone. She was discharged after three days with normal physical examination and laboratory findings. Intraoral examination showed dental infection in the upper tooth region. The paediatrician suspected a correlation between HSP and her dental infection. The dental infection and genetic susceptibility may be the stimulant factors for the autoimmune reactions that caused HSP vasculitis. Hence, it might be useful to investigate the presence of dental infection in the etiology of HSP cases.
IgA Vasculitis ; Twins, Dizygotic