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Objectives Henoch–Schonlein purpura (HSP) is the most common childhood vasculitis, infection is the most essential inducement. We hypothesized that infection could impact the blood routine characteristics and/or outcome of vasculitis. Thus, we aim to find the most common infectious agent in HSP patients and identify convenient indicators to predict renal involvement in HSP patients with infection. Method We conducted a retrospective study of 208 HSP children and 98 healthy children. Clinical parameters were compared in those cases. Results A total of 68.75% of patients were infected with various pathogens. The mean platelet volume (MPV) ( P  < 0.02) was lower in HSP patients with infection than patients without infection. Mycoplasma pneumoniae (MP) infection accounted for the largest proportion (45.77%). MPV in HSP nephritis (HSPN) group was lower than in HSP patients (excluded renal involvement) in patients with MP infection. Logistic regression analysis found that age and MPV were risk factors for the occurrence of MP-infected HSPN. The receiver operating characteristic curve (ROC) analysis showed that the combination of MPV with the onset age at the optimal cut-off point had 81% sensitivity in predicting whether HSP patients with MP infection would develop into HSPN. Conclusions Our research revealed that MP was the most commonly infected pathogen of children’s HSP. MPV was an essential predictor of nephritis in HSP patients with MP infection. This discovery can prompt clinical treatments as well as reduce costs. Key Points • Mycoplasma pneumoniae (MP) accounts for the largest proportion in HSP children with infection. • MPV can be used as a predictor for the development of MP-triggered HSP to HSPN.

Background Henoch‐Schonlein purpura nephritis (HSPN) is a serious complication of Henoch‐Schonlein purpura (HSP), which is usually treated with immunosuppressant and glucocorticoid. This study was designed to explore the effect of dexamethasone and gamma globulin combined with prednisone in the treatment of pediatric HSPN. Methods According to the treatment plan, 60 children treated with dexamethasone and gamma globulin were included in the control group, and the rest 55 children treated with dexamethasone and gamma globulin combined with prednisone were selected as the research group. The clinical manifestations, therapeutic effect, immune function, serum inflammatory factors, blood coagulation function, urine routine, renal function, and adverse reactions were compared between the two groups. Results The clinical manifestations of children in the research group were significantly better than those in the control group after treatment (P < .05). The total effective rate in the research group (94.55%) was markedly higher than that in the control group (76.67%) (P < .05). CD3+, CD4+, CD8+, IL‐10, PT, and APTT increased while CD4+/CD8+, IgA, IL‐8, TNF‐α, FIB, urine protein, urine red blood cell, Scr, and BUN decreased in both groups after treatment, and the changes of all the above indexes in the research group were significant than those in the control group (P < .05). The incidence of adverse reactions in the research group was remarkably superior to that in the control group (P < .05). Conclusion Dexamethasone and gamma globulin combined with prednisone can improve the immune function of children with HSPN and promote the recovery of renal function. Comparison of immune function indexes between the two groups before and after treatment. A, After treatment, the CD3+ level of children in both groups increased, and its change in the research group was more obvious than that in the control group. B, After treatment, the CD4+ level of children in both groups elevated, and its change in the research group was more significant than that in the control group. C, After treatment, the CD8+ level of children in both groups increased, and its change in the research group was more significant than that in the control group. D, After treatment, the ratio of CD4+/CD8+ decreased in both groups, and the change in the research group was more significant than that in the control group. E, After treatment, the IgA level of the two groups of children declined, and the IgA level in the research group changed more significantly than that in the control group. Note: * represents the comparison between the two groups, P < .05

Background Henoch-Schonlein purpura (HSP) is the most common systemic vasculitis of the childhood. However, its mechanisms and pathogenesis still need more exploration. Natural killer (NK) cells are innate lymphocytes, and there is a growing appreciation that cellular metabolism is important in determining the immune responsiveness of lymphocytes. Thus, we aimed to analyze the NK cells phenotype and explore the association between glucose metabolism and NK cells function in HSP patients. Results A total number of 64 HSP patients and 34 healthy children were included. The HSP patients were divided into two groups according to whether accompanied with nephritis or not. NK cells in HSP patients without nephritis showed a reduced frequency in peripheral blood, a down-regulated expression of activating receptors both NKp30 and NKp46, and an attenuated cytotoxic function against tumor cells. In addition, the function impairment of NK cells was shown to exacerbate in HSPN. Our data further revealed an aberrant metabolic reprogramming of NK cells in HSP patients. Upon stimulation with cytokines (IL-15, IL-12 and IL-2), NK cells from healthy controls switched to an elevated glycolysis rate to support their effector function. By contrast, the glycolysis rate of activated NK cells in HSP group was not significantly up-regulated from the resting level possibly owing to the inhibition of mTORC1. Conclusions Our study found that HSP patients were accompanied with dysfunction of NK cells. We concluded that the dysfunction of NK cells in HSP patients was induced with a decreased glycolysis rate and suggested that metabolic reprogramming of NK cells might be a player in the pathogenesis of HSP.

The clinical features of the kidney involvement in immunoglobulin A (IgA) vasculitis (IgAVN) differ in children and adults for both clinical presentation and progression. IgAVN in children has mostly a self-limiting course and favorable resolution, while in adults the kidney involvement is frequently severe with unfavorable outcome. However, a subset of children is at risk of progression within the pediatric age or decades later in adulthood, particularly when the diagnosis and a prompt intervention are delayed. Factors predicting progression and outcome in the whole spectrum of age have been investigated in recent research, as well as the relationship between IgAVN and primary IgAN, which share the same pathology features, in the light of peculiar clinical differences and progression tendencies, and hence need for selective treatments. The search for a personalized treatment in children with IgAV and in different ages of life should rely on the identification of different risks for progression. This review will focus on recent studies which contribute to improve our knowledge in this still largely unclear area.

Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese patent medicine, Tripterygium wilfordii poly-glycosides (TWP) have shown promising benefits in managing autoimmune diseases. To evaluate clinical effectiveness and safety of TWP in treating glomerulonephritis, we systematically searched PubMed, Cochrane Library, Web of Science, and Embase databases for controlled studies published up to 12 July 2023. We employed weighted mean difference and relative risk to analyze continuous and dichotomous outcomes. This meta-analysis included 16 studies that included primary membranous nephropathy (PMN), type 2 diabetic kidney disease (DKD), and Henoch-Schönlein purpura nephritis (HSPN). Analysis revealed that additional TWP administration improved patients’ outcomes and total remission rates, reduced 24-h urine protein (24hUP) and decreased relapse events. The pooled results demonstrated the non-inferiority of TWP to glucocorticoids in achieving total remission, reducing 24hUP, and converting the phospholipase A2 receptor (PLA2R) status to negative. For DKD patients, TWP effectively reduced 24hUP levels, although it did not significantly improve the estimated glomerular filtration rate (eGFR). Compared to valsartan, TWP showed comparable improvements in 24hUP and eGFR levels. In severe cases of HSPN in children, significant clinical remission and a reduction in 24hUP levels were observed with the addition of TWP treatment. TWP did not significantly increase the incidence of adverse reactions. Therefore, TWP could offer therapeutic benefits to patients with PMN, DKD, and severe HSPN, with a minimal increase in the risk of side effects.

PurposeTo compare clinical, pathological, and long-term renal outcomes of children with Henoch–Schonlein purpura nephritis (HSPN) and IgA nephropathy (IgAN).MethodsThe medical records of patients diagnosed as HSPN and IgAN during childhood were evaluated retrospectively. HSPN and IgAN groups were compared in terms of gender, age, upper respiratory infection history, blood pressure; presence of nephrotic and/or nephritic syndrome; hemoglobin level, leukocyte count, C-reactive protein (CRP), serum albumin (sAlb), creatinine, complement 3 (sC3), complement 4 (sC4) and immunoglobulin A (sIgA) levels; estimated glomerular filtration rate (eGFR) and proteinuria levels; and renal pathology findings at the onset of disease; total follow-up time; and blood pressure, eGFR and proteinuria levels at the last visit.ResultsFifty-four patients were enrolled in the study [38 (70%) HSPN and 16 (30%) IgAN]. The median follow-up time was 60.5 and 72.0 months in HSPN and IgAN groups, respectively (p > 0.05). The HSPN and IgAN groups were also not different in terms of gender, age at the onset; leukocyte count, eGFR, sC3-sC4-sIgA levels; and the presence of endocapillary, extracapillary and mesangial proliferation, tubular atrophy, interstitial fibrosis and IgA, IgM, C3 accumulation in renal tissue. Upper respiratory tract infection history was more common in children with IgAN (8/16 vs 8/38, p = 0.045). sAlb (3.96 ± 0.58 vs 4.40 ± 0.46 g/dL, p = 0.005), hemoglobin (12.1 ± 1.3 vs 13.3 ± 1.2 g/dL, p = 0.004,) and the incidence of mesangial IgG deposition (15/38 vs 11/16, p = 0.049) were lower, while CRP (16.3 ± 7.2 vs 7.8 ± 4.4 mg/L, p = 0.002) and proteinuria (72.1 ± 92.4 vs 34.2 ± 37.9 mg/m2/24 h, p = 0.041) was higher in HSPN group at the onset of disease. Proteinuria and eGFR were similar between the two groups at last visit.ConclusionChildren with HSPN and IgAN have little clinical and histological differences in our population. The most prominent difference at presentation with nephritis was higher proteinuria in HSPN probably associated with inflammation due to systemic vasculitis. Long-term renal outcome was good in both HSPN and IgAN.

Immunoglobulin A (IgA) vasculitis (Henoch-Schonlein purpura (HSP)) is the most common vasculitis in children. It is characterized by purpuric rash, arthritis, gastrointestinal, and/or renal involvement. Spontaneous resolution is the typical outcome. In chronic cutaneous manifestations of IgA vasculitis, dapsone seems to show a good effectiveness. Multiple case reports and case series about dapsone in chronic IgA vasculitis are available. However, no clear evaluation of its indications, its effectiveness, or its usage guidelines (optimal dosage or duration of treatment) is available. We reviewed the published cases of IgA vasculitis treated by dapsone and compared them with 2 similar cases that we encountered. Seventeen patients (ranging from 22 months old to 16 years old) with severe or persistent clinical signs of IgA vasculitis were included. Dapsone showed good results on the resolution of cutaneous lesions but not on renal manifestations. Complications (methemoglobinemia) were observed on 1 patient. Half of the patients relapsed after treatment discontinuation. The difference between the time lapse before initiation and the duration of the treatment was not significant.Conclusion: We suggest that dapsone can have a positive effect in chronic IgA vasculitis when cutaneous manifestations last more than 6 weeks at the dosage of 1–2 mg/kg once per day during 1 week.What is Known:• IgA vasculitis or Henoch-Schonlein purpura is the most common vasculitis in children and affects mostly small vessels of the skin, kidney, and gastrointestinal tract. It resolves spontaneously in most of the cases. Exceptionally, cutaneous lesions can last several weeks.• Dapsone is a bacteriostatic antibacterial sulfonamide drug found to be effective in the treatment of some inflammatory dermatological diseases like IgA vasculitis.What is New:• Dapsone is effective against chronic purpuric lesion (> 6 weeks) at the minimal dose of 1 mg/kg/day.• Relapse occurs frequently after discontinuation but responds after a second course of treatment. A longer duration of treatment or a delay in treatment by dapsone does not seem to influence the relapse rate.

Various types of vasculitides have been identified in patients with familial Mediterranean fever (FMF); however, FMF characteristic in patients who experience vasculitis during the disease course have not been described. This study aimed to describe the types of vasculitides in FMF and characterize the patients. This nested case-control study compared 27 patients with FMF (12 male) diagnosed with vasculitis with 100 patients (49 men) who did not develop vasculitis. Most patients (25/27) developed vasculitis after FMF diagnosis. Four types of vasculitides were observed: cutaneous small vessel vasculitis (10 patients, 37%), Henoch-Schonlein purpura/immunoglobulin A vasculitis (8 patients, 30%), periarteritis nodosa (three patients, 11%), and Behçet disease (six patients, 22%). The vasculitis group was younger at FMF onset (6.6 [± 5.9] years vs. 16.2 [± 13.7] years, p < 0.002) and diagnosis (13.1 [± 13.1] years vs. 25.1 [± 17.92] years, p < 0.001). This group showed a higher frequency of homozygosity for the M694V mutation (73.9% vs. 29.4%, p < 0.001), had a more severe FMF (mean Pras severity score: 10.4 [± 2.6] vs. 7.3 [± 3.1], p < 0.001), required higher colchicine doses (1.96 [± 0.61] mg/d vs. 1.66 [± 0.65] mg/d, p < 0.025), and tended to show higher rates of colchicine resistance (29.6% vs. 12%, p = 0.053). However, vasculitis was not an independent factor influencing FMF severity. Patients with FMF and vasculitis are characterized by a more severe disease, likely due to factors other than vasculitis itself. Yet, its presence can serve as a clinical clue to disease severity.

This study aimed to develop and validate a nomogram to forecast severe kidney disease (SKD) outcomes for hospitalized Henoch–Schönlein purpura (HSP) children. The predictive model was built based on a primary cohort that included 2,019 patients with HSP who were diagnosed between January 2009 and December 2013. Another cohort consisting of 461 patients between January 2014 and December 2016 was recruited for independent validation. Patients were followed up for 24 months in development/training and validation cohorts. The data were gathered at multiple time points after HSP (at 3, 6, 12, and 24 months) covering severe kidney disease as the severe outcome after HSP. The least absolute shrinkage and selection operator (LASSO) regression model was utilized to decrease data dimension and choose potentially relevant features, which included socioeconomic factors, clinical features, and treatments. Multivariate Cox proportional hazards analysis was employed to establish a novel nomogram. The performance of the nomogram was assessed on the aspects of its calibration, discrimination, and clinical usefulness. The nomogram comprised serious skin rash or digestive tract purpura, severe gastrointestinal (GI) manifestations, recurrent symptoms, and renal involvement as predictors of SKD, providing favorable calibration and discrimination in the training dataset with a C-index of 0.751 (95% CI, 0.734–0.769). Furthermore, it demonstrated receivable discrimination in the validation cohort, with a C-index of 0.714 (95% CI, 0.678–0.750). With the use of decision curve analysis, the nomogram was proven to be clinically useful. The nomogram independently predicted SKD in HSP and displayed favorable discrimination and calibration values. It could be convenient to promote the individualized prediction of SKD in patients with HSP.

Background: Gut microbiota plays an important role in the pathogenesis of immune-mediated diseases. However, the complex pathogenesis of Henoch-Schonlein Purpura (HSP) remains elusive. This study aimed to characterize the gut microbiota in HSP patients and explore the potential association between gut microbiota composition and phenotypic changes in HSP.Methods: 16SrRNA gene sequencing and bioinformatic analyses were performed using total DNA extracted from the fecal microbiota of 34 children with HSP, including 18 primary cases, 16 recurrent cases, and 23 healthy children.Results: The diversity indexes showed significant differences in the microbial community among the primary HSP groups, the recurrent HSP group and healthy controls. The abundance of Escherichia-Shigella in the recurrent HSP group was significantly higher than that in the primary HSP group, and the constructed ROC curve had an AUC value of 0.750. According to the Spearman correlation analysis, the abundance of Bacteroides was positively associated with the serum IgG level in children with HSP, while the abundance of Lachnoclostridium was negatively correlated with the complement component 3 (C3). The diversity indexes of gut microbiota in the HSP group with abdominal symptoms were higher than those in the HSP group without GI involvement, and also higher than those in the healthy control group. In the HSP group with GI involvement, the abundance of Faecalibacterium was decreased, while the abundance of Streptococcus and Fusobacteria was increased, compared to the HSP group without GI involvement.Conclusions: The gut microbiota of children with HSP was different from that of healthy children. The genus Escherichia-Shigella has a diagnostic value for HSP recurrence. Bacteroides and Lachnoclostridium may affect IgG and complement C3 levels in children with HSP. Abdominal symptoms in HSP children were related to gut microbiota (Streptococcus and butyric acid-producing bacteria).