OBJECTIVE: To explore whether streptococcal infection may aggravate renal damage in children with Henoch-Schönlein purpura nephritis and its possible mechanism. METHODS: In the study, 485 children diagnosed with Henoch-Schönlein purpura nephritis from July 2015 to December 2019 were selected to analyze their clinical data retrospectively. According to the diagnosis of discharge, whether it was combined with streptococcal infection, the children were divided into two groups. The experimental group contained 91 children with Henoch-Schönlein purpura nephritis combined with streptococcal infection, and there were 394 children who were not infected with Streptococcus in the control group. Suitable test items were preliminarily selected through artificial neural network, and then data analysis was performed through SPSS 23.0. RESULTS: The children with Henoch-Schönlein purpura nephritis infected with streptococcus had statistically significant differences compared with the uninfected children in the test items of urine protein, liver and kidney function, immunoglobulin and complement. Anti-streptolysin O had mild correlation with IgG (Spearman r=-0.328), fibrin degradation products (Spearman r=-0.207), total protein (Spearman r=-0.202) and globulin (Spearman r=-0.223). Compared with the children who were not infected with streptococcus, the differences of the average levels of age (P=0.001), IgG (P < 0.001), fibrin degradation products (P=0.019), total protein (P < 0.001), globulin (P < 0.001), IgA (P < 0.001), IgM (P=0.003), complement 3 (P=0.016), complement 4 (P=0.002), albumin/globulin ratio (P=0.007), alkaline phosphatase (P=0.036), and estimated glomerular filtration rate (P=0.039) in the infected children were statistically significant. In order to explore the risk factors of kidney damage in the children with Henoch-Schönlein purpura nephritis, Logistic regression was performed using anti-streptolysin O, age, immunoglobulin and complement as independent variables, urine protein detection parameters, liver and kidney functions as dependent variables. Age ≤10 years old and hypocomplementemia might be risk factors for aggravating renal damage in the children with Henoch-Schönlein purpura nephritis. CONCLUSION Streptococcal infections may aggravate renal damage in children with Henoch-Schönlein purpura nephritis, in which hypocomplementemia, inflammation, fibrinolysis and disorders of coagulation perhaps play an important role. Children with streptococcal infection should be treated with anti-infective treatment in time and necessarily, and followed up after discharge regularly.
Humans ; IgA Vasculitis/complications* ; Streptococcal Infections/complications* ; Child ; Male ; Female ; Nephritis/microbiology* ; Retrospective Studies ; Child, Preschool ; Kidney/pathology* ; Adolescent

IgA nephropathy (IgAN) is the most common primary glomerular disease in China and a leading cause of end-stage renal disease (uremia) in young adults. The diagnosis, prognostic assessment, and treatment strategies for IgAN and IgA vasculitis with nephritis (IgAVN) have been comprehensively evaluated by the Scientific Committee of the China IgA Nephropathy Network (IIgANN-China) and the Chinese Preventive Medicine Association's Committee for the Prevention and Control of Kidney Diseases based on recent literature and evidence-based medicine. As a result, clinical practice guidelines specifically tailored to Chinese patients have been developed. These guidelines introduce an integrated therapeutic framework that incorporates risk-stratified treatment, targeting both immune-mediated renal injury and chronic kidney disease progression, as well as stage-specific treatment, including both the induction and maintenance phases. The aim is to provide standardized guidance and practical recommendations for the clinical management of IgAN and IgAVN in China.
Humans ; Glomerulonephritis, IGA/diagnosis* ; China ; Adult ; Vasculitis/complications* ; Practice Guidelines as Topic ; Immunoglobulin A ; Prognosis ; Nephritis/therapy*

Monoclonal gammopathy of undetermined significance combined with renal damage is named monoclonal gammopathy of renal significance. There are few reports about IgA vasculitis in patients with monoclonal gammopathy of undetermined significance. Here, we report a case of monoclonal gammopathy of renal significance, who had manifestations of IgA vasculitis, including purpura, gastrointestinal bleeding and joint pain. The patient had elevated serum creatinine levels, prompting further investigation through immunofixation electrophoresis and bone marrow aspiration biopsy. Immunofixation electrophoresis showed IgA-λ-type monoclonal immunoglobulin, while the bone marrow aspiration biopsy suggested plasmacytosis. Kidney biopsy indicated membranous hyperplastic glomerulonephritis, light and heavy chain deposition, IgA-λ. The patient was diagnosed with monoclonal gammopathy of renal significance. In light of the elevated serum creatinine, the patient was treated with chemotherapy regimen (bortezomib +cyclophosphamide +dexamethasone). After chemotherapy, there was no significant improvement in the patient's renal function. Subsequently, the patient experienced abdominal pain, skin purpura, joint pain and severe gastrointestinal bleeding. Gastroenteroscopy did not find the exact bleeding position. Angiography revealed hyperplasia of left jejunal artery. Surgical operation found that the bleeding site was located between the jejunum and ileum, where scattered hemorrhagic spots and multiple ulcers were present on the surface of the small intestine, with the deepest ulcers reaching the serosal layer. And the damaged intestine was removed during the operation. Intestinal pathology showed multiple intestinal submucosal arteritis, rusulting in intestinal wall necrosis and multiple ulcers. Considering intestinal lesions as gastrointestinal involvement of IgA vasculitis, methylprednisolone was used continually after the operation, and the patient's condition was improved. However, after half a year, the patient suffered a severe respiratory infection and experienced a recurrence of serious gastrointestinal bleeding. It was considered that the infection triggered the activity of IgA vasculitis, accompanied by gastrointestinal involvement. Finally, the patient died from gastrointestinal bleeding. The present case represented a patient with monoclonal gammopathy of renal significance and IgA vasculitis, prominently presenting with renal insufficiency and severe gastrointestinal bleeding, making the diagnosis and treatment process complex. Patients with IgA monoclonal gammopathy who presented with abdominal pain, purpura, and arthralgia should be vigilant for the possibility of concomitant IgA vasculitis. The treatment of cases with IgA vasculitis combined with monoclonal gammopathy of renal significance was rather challenging. Plasma cell targeting therapy might be an effective regimen for IgA vasculitis with monoclonal gammopathy. However, patients with poor renal response to the treatment indicated poor prognosis.
Humans ; Cyclophosphamide/administration & dosage* ; Gastrointestinal Hemorrhage/etiology* ; IgA Vasculitis/complications* ; Immunoglobulin A ; Intestine, Small/pathology* ; Kidney/pathology* ; Kidney Diseases/pathology* ; Monoclonal Gammopathy of Undetermined Significance/complications* ; Necrosis ; Paraproteinemias/complications* ; Vasculitis/etiology*

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*

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*

No abstract available.
Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications/diagnosis/drug therapy/immunology ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Biomarkers/blood ; Biopsy ; Drug Therapy, Combination ; Fluorescent Antibody Technique ; Glomerulonephritis, IGA/*complications/diagnosis/drug therapy/immunology ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Myeloblastin/*immunology ; Treatment Outcome