Henoch-Schönlein Purpura Nephritis Research Articles

Clinical Significance of Serum Midkine in Children with Henoch-Schonlein Purpura

To investigate the clinical significance of serum midkine (MK) in children with henoch-schnlein purpura(HSP). One hundred and six children with HSP admitted in our hospital from March 2013 to March 2016 were enrolled in HSP group, but then 80 healthy volunteers were used as control. MK, interleukin-2 (IL-2), IL-4, IL-6, IL-10, tumor necrosis factor (TNF), interferon γ (IFN-γ) and IL-17 in peripheral blood were measured by ELISA, biochemical indicators including white blood cell count (WBC), platelet (Plt), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), D-dimer, immunoglobulin A (IgA), IgE, IgG, IgM and other clinical data were recorded and analyzed. Among 106 cases of henoch-schonlein purpura, 42 patients combined with renal failure (isolated hematuria in 4 cases, 17 cases of isolated proteinuria, hematuria and proteinuria in 21 cases). Midkine level in children with HSP was significantly higher than that in the control group [291.70(248.50-396.41) pg/ml vs 217.30(198.98-243.65) pg/ml)](P<0.05); the MK level in group of HSP with nephritis was higher than that in group of HSP without nephritis [326.58(266.58-459.25) pg/ml vs 280.72(233.67-384.36) pg/ml] (P<0.05). IL-4, IL-6, IL-17, TNF and IFN-γ levels in children with HSP were higher than those in the control group(P<0.05), but IL-10 level was lower than that in control group. IL-2 level was not significantly different between 2 groups. Cytokines levels in HSP nephritis group and HSP without renal involvement were not significantly different. Pearson correlation analysis showed that midkine level positively correlated with IL-4, IL-6, IL-17, IgA and IgE (P<0.05). By ROC curve analysis, the AUC of midkine was 0.902, 95% CI 0.841-0.963 (P<0.001), threshold 295.50 pg/ml. The sensitivity and specificity of midkine for predicting Henoch-Schonlein purpura nephritis were 80.60% and 88.30% respecitvely. Plasma MK plays an important role in the development of Henoch-Schonlein purpura and Henoch-Schonlein purpura nephritis. Plasma MK can be used as an effective indicator for early diagnosis and prediction of HSP and renal damage.

Association of MMP-9 C-1562T polymorphism with risk of Henoch-Schönlein purpura nephritis in children of southeast China

Henoch-Schonlein purpura nephritis (HSPN) is the most prevalent vasculitis in childhood worldwide. Although the etiology of HSPN is not yet fully elucidated, it is believed to be closely related with infections and genetic predispositions. In this study, a cohort of children from southeast China, including 108 healthy controls and 184 patients were involved in. We scanned the promoter region and the whole gene for genetic variations. A C/T transition located in the promoter region of MMP-9 gene, C-1562T was identified. Subjects were genotyped by using SNaPshot sequencing and the frequencies of CC, CT and TT genotypes were 43, 33 and 24% in patients and 53, 37 and 10% in healthy controls. Allele distribution was found to be in Hardy–Weinberg equilibrium (P = 0.17). Association analysis revealed that the polymorphism was significantly related with HSPN risk (P < 0.05) and T allele was shown to be a risk factor (P = 0.003, odds ratio (OR) 1.63, 95% confidence interval (CI) 1.13–2.34). Additionally, we measured the serum MMP-9 concentration and quantified the mRNA expression. Results showed that an elevated protein level was observed in patients compared with controls in both ELISA (P = 0.0029, 136.5 μg/L vs. 103.9 μg/L) and Western blot assays. Furthermore, we also detected 1.93-fold increase in the mRNA transcription in patients carrying homozygous TT genotype, indicating enhanced promoter activity caused by the polymorphism. Based on the incidence risk, a cut-off value of serum MMP-9 served as HSPN diagnosis was suggested at 155 μg/L. Conclusively, our findings suggested that MMP-9 polymorphism was significantly associated with HSPN susceptibility, and C-1562T T allele contributed to HSPN development.

Clinical effects of leflunomide in the treatment of patients with Henoch-Schonlein purpura nephritis who were not response to cyclophosphamide

Objective To evaluate the effect of leflunomide(LEF) in the treatment of adult Henoch-Schonlein purpura nephritis(HSPN) patients with nephrotic syndrome who were not response to cyclophosphamide. Methods 26 patients with nephrotic syndrome HSPN performance treated by CTX therapy, relapse or discontinued due to adverse reactions were selected.LEF joint glucocorticoid treatment was given to the patients for 6 months.Collected the blood routine, urine routine, 24 hours urine protein, urinary red blood cell count, serum albumin, hepatic and renal functions before treatment and 6 months after treatment, and the adverse drug reactions were recorded. Results In 26 patients, 20 cases had clinical remission.After treatment, compared with before treatment, the urinary protein, urinary red blood cell count decreased significantly(P<0.05), and serum albumin increased significantly(P<0.05) in 20 patients, renal function recovered to normal in 6 patients.11 patients achieved complete remission, 9 patients achieved partial remission.Compared with cyclophosphamide treatment was invalid, the clinical remission rate was 76.9%(χ2=6.783, P<0.01). Of 26 patients, 1 patient had mild gastrointestinal symptoms, 1 patient had mild neutropenia, 1 patient had mild liver transaminases elevated, compared with cyclophosphamide side effects incidence rate of 46.2%(12/26), the incidence rate of adverse reactions was 11.5%(3/26)(χ2=5.496, P<0.05), they were relieved after expectant treatments, no patients stop treatment. Conclusion LEF combined with glucocorticoid had good efficacy and mild adverse reactions for adult HSPN patients with nephrotic syndrome. Key words: Purpura, Schoenlein-Henoch; Nephritis; Leflunomide; Cyclophosphamide

Aberrant IgA1 Glycosylation in IgA Nephropathy: A Systematic Review.

ObjectiveGalactose-deficient IgA1 was evaluated in patients with IgA nephropathy(IgAN) and controls in order to determine the predictive value of galactose-deficient IgA1 in cases of IgA nephropathy.MethodsPubMed, EMBASE, Cochrane central register of controlled trials, CNKI, CBM disc, and VIP database were searched to identify eligible studies that evaluated a difference in aberrant IgA1 glycosylation in IgAN patients compared with controls. A meta-analysis was conducted to evaluate the impact of galactose-deficient IgA1(Gd-IgA1) levels in different groups.ResultsA total of 22 studies (n = 1657) met inclusion criteria. The mean Newcastle-Ottawa Scale (NOS) score was 7.2 and ranged from 6 to 8. The standard mean difference(SMD) in the meta-analysis of 20 studies of the level of Gd-IgA1 in the serum and/or supernatant of cultured cells was higher in the IgAN group compared with healthy controls as well as in those with other renal diseases (SMD = 1.76, 95% CI = 1.18–2.34, P<0.00001; SMD = 1.05, 95% CI = 0.05–2.04, P = 0.04). The data synthesis suggested that IgAN patients had similar levels of serum Gd-IgA1, with no significant differences, compared with first-degree relatives and Henoch-Schonlein purpura nephritis (HSPN) patients (MD = 0.04, 95% CI = 0.00–0.08, P = 0.05; MD = -46.03, 95% CI = -217.70–125.64, P = 0.60). In addition, the combined MD of 5 studies indicated that there were no significant differences in Gd-IgA1 levels among patients with varying severities of IgAN (MD = 0.02, 95% CI = -0.02–0.05, P = 0.28).ConclusionsThe pooled evidence suggests that the level of Gd-IgA1 in the serum or supernatant of cultured cells from peripheral blood or tonsils may be a useful biomarker for predicting IgA nephropathy, though the level of Gd-IgA1 was not significantly associated with disease severity.

Clinicopathological characteristics in 996 cases with renal biopsy

Objectives To retrospectively analyze the pathologic type of renal biopsy and the change of disease spectrum of recent 10 years in our hospital. Methods The clinical data of 996 patients with renal biopsy from February 2006 to February 2016 in our department were collected and analyzed. Results Among the 996 patients, primary glomerular disease (PGD) was 76.31%, secondary glomerular disease (SGD) was 21.18%, renal tubulointerstitial was 2.21% and hereditary kidney disease was 0.30%. Among the PGD, IgA nephropathy was the most common pathological type with 238 cases (31.32%), followed by mesangial proliferative glomerulonephritis with 199 cases (26.18%), membranous nephropathy with 127 cases (16.71%). Among the SGD, lupus nephritis was the most common pathological type with 76 cases (36.02%), followed by henoch-schonlein purpura nephritis with 56 cases (26.54%) and hepatitis B virus associated glomerulonephritis (HBV-GN) with 24 cases (11.37%). Among the PGD, nephritic syndrome was the most common clinical manifestation with 446 cases (58.68%), followed by chronic glomerulonephritis with 223 cases (29.34%) and latent glomerulomephritis with 69 cases (9.08%). Conclusions PGD is the most frequent glomerular disease in clinic. IgA nephropathy is the most common pathological type in PGD and the proportion of membranous nephropathy increases significantly in recent years. In SGD, Lupus nephritis is the most common pathological type and the proportion of HBV-GN also increases significantly. Key words: Kidney Diseases; Biopsy, Needle

Effect of CD40/CD40L signaling on IL-10-producing regulatory B cells in Chinese children with Henoch-Schönlein purpura nephritis.

The aim of the present study was to examine the role and mechanism of interleukin-10 (IL-10)-producing regulatory B cells (B10 cells) in the pathogenesis of Henoch-Schönlein purpura nephritis (HSPN). We examined the percentage of B10 cells, CD19+CD24hiCD38hi B cells, CD19+CD24hiCD27+ B cells, Th17 cells, and T regulatory (Treg) cells within the peripheral blood mononuclear cell (PBMC) population in healthy subjects and HSP/HSPN patients. The percentage of B10 cells and CD19+CD24hiCD38hi B cells was reduced in HSPN patients and that of CD19+CD24hiCD27+ B cells was decreased only in HSPN patients with hematuria and proteinuria or massive proteinuria. The expression of IL-10 by B10 cells and their subsets was decreased in HSPN patients and returned to normal levels in HSP/HSPN patients in remission. B10 cells and their subsets negatively correlated with the Th17/Treg ratio. There was no difference in B10pro + B10 cells, Th17 cells, Treg cells, and the Th17/Treg ratio between children with HSP/HSPN and healthy controls after CD40L stimulation. On the other hand, the level of IL-10 expressed by CD19+CD40+ B cells was decreased in HSPN, and the percentage of B10pro + B10 cells and Treg cells was reduced and that of Th17 cell was increased in the presence of anti-CD40L monoclonal antibody (mAb). Thus, decreased B10 cells and CD19+CD24hiCD38hi B cells may function as an early marker of renal impairment in HSPN. The dysfunction of B10 cells may play a role in the pathogenesis of HSPN by regulating the Th17/Treg balance. Moreover, the CD40/CD40L signaling pathway may play a role in B10 cell differentiation and functional maturation.

Differences in pathological characteristics and laboratory indicators in adult and pediatric patients with Henoch-Schönlein purpura nephritis.

We aimed to investigate the differences in renal histopathological changes and laboratory parameters between adult and pediatric patients with Henoch-Schönlein purpura nephritis (HSPN), and to analyze the correlation between laboratory parameters and renal histopathological grading. A total of 139 patients diagnosed with HSPN between September 2010 and December 2014 at the First Hospital of Jilin University, China, were retrospectively reviewed. The clinical and pathological characteristics were examined and compared between the adult and the pediatric patients. A majority of adult (75.0%) and pediatric (66.2%) patients were categorized as pathological grade III HSPN. Adults having crescent lesions, interstitial fibrosis and renal artery involvement significantly outnumbered child counterparts (all P<0.05). Pathological grading showed a positive correlation with 24-h urine protein (r=0.307, P=0.009), microalbuminuria (r=0.266, P=0.000) and serum globulin (r=0.307, P=0.014), and a negative correlation with serum albumin (r=0.249, P=0.037) in pediatric patients with HSPN. Among adult patients with HSPN, histopathological grading showed a positive correlation with 24-h urine protein (r=0.294, P=0.015), microalbuminuria (r=0.352, P=0.006), α1-microglobulin (r=0.311, P=0.019) and immunoglobulin G (r=0.301, P=0.023) in urine, and serum creatinine (r=0.292, P=0.018). Further, a negative correlation between serum albumin and pathological grading was also observed (r=0.291, P=0.018). In conclusion, the severity of renal pathological lesions in HSPN patients is well reflected by the levels of proteinuria. Adult patients have more severe renal histopathological changes than pediatric patients.

Hemorragia pulmonar, una complicación poco frecuente en una niña con púrpura de Schönlein-Henoch

Henoch-Schonlein purpura (HSP) is the most common vasculitis in children. Vasculitic processes can involve the lung. Although diffuse alveolar hemorrhage may be seen as one of the manifestation of HSP, it is not a frequent presentation. Here we reported the case of a 10-year-old girl with HSP nephritis who developed pulmonary hemorrhage. The patient was treated successfully with intravenous methylprednisolone. A review of the literature revealed that young age may be a good prognostic sign and that immunosuppressive drugs and supportive management are essential in the treatment.

Recurrent adult onset Henoch-Schonlein Purpura: a case report

Henoch-Schonlein purpura is an immunoglobulin A (IgA)-immune complex mediated leukocytoclastic vasculitis that classically manifests with palpable purpura, abdominal pain, arthritis, and hematuria or proteinuria. The condition is much more predominant in children (90% of cases) and commonly follows an upper respiratory infection. We present a case of recurrent Henoch-Schonlein purpura (HSP) complicated by nephritis in an adult female initially categorized as IgA nephropathy (IgAN). We review the pathophysiologic basis of HSP nephritis as the variant of HSP accompanied by renal involvement and its pathogenetic commonality with IgA nephropathy.

Changing trends in pediatric renal biopsies: analysis of pediatric renal biopsies in national nephrology registry data

Renal biopsy is the gold standard method for determining the diagnosis, treatment, and prognosis in children with renal disease. This study aims to evaluate the histopathological features of pediatric renal biopsies obtained from the national nephrology registry in the last two decades. Data recorded in the Turkish Society of Nephrology Registry System (TSNRS) in 1991 as well as in between 2001 and 2010 were analyzed. A total of 3892 biopsies were recorded; with the least number in 1991 (total 103 biopsies from 17 centers) and the highest number in 2008 (total 654 biopsies from 23 centers). Glomerular diseases constituted the main group in the registry (62.64%), followed by systemic diseases (20.06%). Focal and segmental glomerulosclerosis (FSGS) and Henoch–Schönlein purpura (HSP) nephritis (IgA vasculitis) were the most common glomerular and systemic diseases, respectively. Overall prevalence of renal amyloidosis and membranous nephropathy (MN) was quite low (1.87% and 1.56%, respectively) in all periods. Compared to 1991, there was an increasing trend in the frequencies of certain disorders including hemolytic uremic syndrome (HUS), IgA nephropathy, and HSP nephritis; and there was a decrease in acute proliferative glomerulonephritis (GN) in 2008. As well as demonstrating the etiologies of renal diseases which can only be identified by renal biopsies, this study provides important information regarding the changing patterns of histopathological findings due to better management of pediatric renal diseases over the years in Turkey.

An adult case of severe steroid-resistant Henoch-Schönlein purpura nephritis treated with intravenous cyclophosphamide and tonsillectomy.

A previously healthy 37-year-old Canadian man living in Japan visited a hospital in Thailand while traveling because of edematous legs, purpura, arthralgia, bloody stool, and fever after an insect bite. Henoch-Schönlein purpura (HSP) was suspected. His creatinine level was 5.2mg/dL. He was treated with oral prednisolone (PSL) and oral cyclophosphamide (CPA); after treatment, his creatinine level improved to 2.4mg/dL. Upon returning to Japan, he was admitted to the National Center for Global Health and Medicine Hospital in Tokyo. A kidney biopsy was performed, and HSP nephritis (HSPN) was diagnosed. Renal dysfunction and proteinuria persisted despite 4 administrations of steroid-pulse therapy and 3 sessions of plasma exchange. Finally, he was treated with intravenous cyclophosphamide (IVCY). His creatinine level and proteinuria markedly improved. His microscopic hematuria disappeared after he underwent tonsillectomy. There have been only a few case reports describing patients with adult-onset HSPN necessitating IVCY. We present here a rare case of steroid-resistant HSPN treated with IVCY and tonsillectomy, with reference to some recent findings.

Parainfluenza infection is associated with Henoch-Schönlein purpura in children

Abstract Background To investigate the association of respiratory tract viruses with Henoch-Schonlein purpura (HSP) in children. Methods 40 children with HSP and 42 controls with upper respiratory tract infections were enrolled in the study. In both groups, the serum IgM antibodies and viral antigens in peripheral blood mononuclear cells (PBMCs) of common respiratory tract viruses including parainfluenza virus (PIV), influenza, respiratory syncytial virus (RSV), and adenovirus were detected. Children with HSP nephritis (HSPN) received renal biopsy, and antigens of the aforementioned viruses were examined. Results Viral IgM antibodies in sera and antigens in PBMCs were detected in HSP and control groups (55% vs. 42.9% and 60% vs. 42.9% respectively), but the differences were not significant. The prevalence of PIV IgM antibody and antigen in PBMCs in HSP group was significantly higher than that in controls (P Conclusions The presence of respiratory tract viruses was common in HSP children. PIV may be an important contributing factor in the pathogenesis of HSP.

Potential of Serum and Urinary Matrix Metalloproteinase-9 Levels for the Early Detection of Renal Involvement in Children With Henoch-Schönlein Purpura.

BackgroundMatrix metalloproteinase-9 (MMP-9) is an enzyme implicated in the pathogenesis of renal diseases. Renal involvement is the principal cause of morbidity and mortality in children with Henoch-Schönlein purpura (HSP).ObjectivesThe aim of this study was to evaluate whether serum and urinary MMP-9 levels are associated with renal involvement in HSPPatients and MethodsWe evaluated 40 children with HSP (patient group) and 27 healthy volunteer children (control group). The patient group was divided into two subgroups based on the presence or absence of nephritis. Nephritis was defined as the existence of hematuria and/or proteinuria. All anthropometric data, physical examination findings, blood pressure, and laboratory parameters were recorded. The serum and urine samples were analyzed to determine the MMP-9 levels three days after the initial phase of the disease.ResultsThe mean age was 7.65 ± 3.41 (range 2 - 16) years in the patient group and 9.52 ± 3.91 (range 2 - 16) years in the control group. Henoch-Schonlein purpura nephritis (HSPN) was identified in eight patients. There was no significant difference in the serum MMP-9 levels between the HSPN subgroup and the controls (P > 0.05). However, there were significant differences in the urinary MMP-9 levels between the HSP subgroup and the control group (P < 0.05), with the urinary MMP-9 levels being significantly higher in patients in the HSP subgroup (P = 0.001). Further, the urinary MMP-9 levels were significantly higher in the patients with nephritis than in the patients without nephritis (P = 0.001) and the controls (P = 0.001). The optimal cut-off point (sensitivity; specificity) of the urinary MMP-9 level for the diagnosis of HSPN was 94.7 pg/mL.ConclusionsThe levels of MMP-9 in the urine were remarkably high in patients with HSPN. This non-invasive marker may therefore be an important indicator for the early diagnosis of nephritis in children with HSP.

Reduced INF2 expression in nephrotic syndrome is possibly related to clinical severity of steroid resistance in children.

Mutations of the inverted formin 2 gene (INF2), which encodes a member of the formin family, cause autosomal dominant focal segmental glomerulosclerosis (FSGS) and Charcot-Marie-Tooth (CMT) disease-associated FSGS. However, their role in idiopathic FSGS remains unclear. This study investigated INF2 localization in the normal adult kidney and its expression in children with idiopathic nephrotic syndrome. We generated a rabbit polyclonal antibody against the conjugated peptide from human INF2 and studied the glomerular expression of INF2 and synaptopodin using normal human adult kidney tissues and tissues from children with glomerular diseases such as minimal change disease (MCD), FSGS, IgA nephropathy (IgAN), non-IgA mesangial proliferative glomerulonephritis (non-IgAN), and Henoch-Schönlein purpura nephritis (HSPN). The anti-INF2 antibody detected an approximately 140-kD fragment isolated from adult mature glomeruli by western blotting. Immunohistochemically, INF2 was detected in podocytes and renal arteries. Among 56 patients, INF2 in glomeruli was expressed at a similar level in patients with MCD, IgAN, non-IgAN, or HSPN and controls. In FSGS patients, INF2 expression in glomeruli was either decreased or absent. There was a relationship between decreased INF2 expression and the clinical severity of steroid resistant nephrotic syndrome (SRNS). We propose that examination of INF2 expression may help to differentiate MCD from FSGS and evaluate the clinical severity of SRNS in children.

Prognosis factors of Henoch-Schonlein purpura nephritis in children

Objective To investigate the outcome predictors of renal injury in children with Henoch-Schonlein purpura nephritis(HSPN). Methods One hundred and thirty-seven patients with HSPN, who were admitted to Shanghai Children′s Hospital from January 2009 to December 2013, were retrospectively studied. The data of gender, age, clinical features, and pathological changes were collected. Results (1) Among the 137 children, whose median duration of follow-up was (43.10±16.39)months, 99 cases (72.26%) had good outcomes(grade A), 36 cases (26.28%) had grade B, only 2 cases without remission(grade C), and no cases progressed to renal failure(grade D). (2)Patients had relatively poor prognosis if they were presented with the higher urine protein, blood urea nitrogen(BUN), serum creatinine(Cr), cystatin C(Cys-C), IgA, thrombin time(TT), and the lower glomerular filtration rate (GFR). (3)The most common clinical types of HSPN were hematuria and proteinuria (111/137 cases, 81.02%), and 80 cases of them (72.07%) achieved grade A, and only 1 case (0.90%)got grade C. Only 1 case whose clinical ma-nifestations was acute nephritis got grade C. Clinical manifestations were associated with prognosis of HSPN(P 0.05). Conclusions The prognosis of HSPN is associated with clinical classification, pathological stage. Moreover, the prognosis predictors include elevated urine protein, BUN, Cr, Cys-C, IgA, TT, and decreased GFR.Optimistic prognosis could be generated from timely and early treatment. Key words: Henoch-Schonlein purpura nephritis; Clinical features; Pathological grade; Prognosis; Retrospective analysis

Skin Rash and Microscopic Hematuria in a 10-Year-Old Caucasian Male.

Henoch-Schonlein purpura (HSP) is an acute, systemic, vasculitis with IgA-dominant immune deposits. With the emphasis on educational value of HSP, which is the most common form of vasculitis in children, we report an actual case from a 10-year-old boy. The patient presented with the chief complaint of a skin rash. His illness history, family medical history, physical examination, and relevant laboratory findings were summarized, followed by a question and possible answer format discussion. With the significant elevation of red blood cells in his urine and moderate to severe deposition of IgA in kidney biopsy, the patient was diagnosed with HSP nephritis. Renal symptoms, such as proteinuria and hematuria, are mostly the last to develop and determine the long-term prognosis in HSP patients. The patient is currently undergoing steroid treatment, which is the primary intervention for HSP as it spontaneously resolves in most of affected children.

A Lower Proportion of Regulatory B Cells in Patients with Henoch-Schoenlein Purpura Nephritis.

BackgroundHenoch—Schoenlein purpura is the one of most common types of systemic vasculitis that involves impaired renal function and Henoch-Schoenlein purpura nephritis (HSPN). The diagnosis of this condition is largely based on immunohistologic detection of immunoglobulin A1-containing immune complex in the glomerular deposits of mesangium. Despite clinical advances, the etiopathogenesis of HSPN is still largely unknown.MethodsIn this study, we enrolled 25 newly diagnosed HSPN patients and 14 healthy controls. Then, fractions of B cell subtypes were determined in venous blood using flow cytometry. The serum interleukin (IL)-10 concentration was determined by enzyme-linked immunosorbent assay.ResultsCompared to those in healthy controls, the numbers of CD38+CD19+, CD86+CD19+, CD38+CD86+CD19+, and CD95+CD19+ B cells per microliter of blood were significantly higher in HSPN patients. In contrast, the numbers of CD5+CD19+, IL-10+CD19+, CD5+CD1d+CD19+, and IL-10+CD5+CD1d+CD19+ B cells per microliter of blood and the serum IL-10 concentration were significantly lower in HSPN patients. Following treatment, the numbers of CD38+CD19+ and CD86+CD19+ B cells per microliter of blood were significantly reduced in HSPN patients. However, the numbers of CD5+CD1d+CD19+, CD5+CD1d+IL-10+CD19+, and IL-10+CD19+ B cells per microliter of blood and the serum IL-10 concentration were significantly increased in HSPN patients following treatment. The estimated glomerular filtration rate (eGFR) was negatively correlated with the number of CD38+CD19+ B cells but positively correlated with the numbers of IL-10+CD19+, CD1d+CD5+CD19+, and IL-10+CD1d+CD5+CD19+B cells per microliter of blood and the serum IL-10 concentration. The 24-h urinary protein concentration was positively correlated with the number of CD38+CD19+B cells but negatively correlated with the numbers of IL-10+CD19+, CD1d+CD5+CD19+, and IL-10+CD1d+CD5+CD19+B cells per microliter of blood and the serum IL-10 concentration.ConclusionOur results suggest that CD38+CD19+ and CD1d+CD5+CD19+ B cells (Bregs) contribute to the pathogenesis of HSPN.

Association between IL17A and IL17F polymorphisms and risk of Henoch-Schonlein purpura in Chinese children.

Previous studies suggested that interleukin-17 and Th17 cell play an important role in the pathogenesis of childhood Henoch-Schonlein purpura (HSP). The purpose of our study is to elucidate whether the IL17A and IL17F gene polymorphisms are susceptibility genes for the development of HSP in Chinese children. A total of 148 HSP patients and 202 controls were enrolled for analyzing the single nucleotide polymorphisms (SNP) of IL17A (rs2275913, rs8193037 and rs3819025) and IL17F (rs763780 and rs9463772). TaqMan Real-Time polymerase chain reaction method was used in SNP genotyping. Compared to the healthy controls, the IL17A rs2275913 variant allele A showed a significant association with HSP [odds ratio (OR) 0.70; 95% CI 0.51-0.94, P=0.018]. Genotyping analysis demonstrated rs2275913 was associated with a decreased HSP risk (G/A vs. G/G: OR 0.56; 95% CI 0.33-0.95; A/A vs. G/G: OR 0.46; 95% CI 0.24-0.86; P=0.032). Also, our findings showed that the A allele of IL17A rs3819025 was associated with a higher risk of HSP nephritis (OR 1.61; 95% CI 1.00-2.58; P=0.047). In addition, a risk haplotype of IL17A (GGA) was found (OR 1.84; 95% CI 1.17-2.88; P=0.008). However, no significant differences between HSP patients and healthy controls were observed when comparing genotype, allele or haplotype frequencies of the IL17F rs763780 and rs9463772 polymorphisms. In this study, we confirmed that the rs2275913 polymorphism of the IL17A gene was associated with susceptibility to HSP in Chinese children. However, there was no relationship between IL17F rs763780 and rs9463772 polymorphisms and HSP susceptibility.

Incidence and clinical features of paediatric vasculitis in Eastern China: 14-year retrospective study, 1999-2013.

ObjectivesTo determine the incidence and clinical features of paediatric primary vasculitis in patients from one centre in Eastern China.MethodsMedical records of paediatric patients diagnosed with primary vasculitis between January 1999 and December 2013 were retrospectively reviewed. For Henoch–Schönlein purpura (HSP) and Kawasaki disease (KD), patients included in the analyses had data available for the previous 5 years.ResultsIn total, 1896 patients were identified, of whom 1100 had HSP, 760 had KD, 23 had Takayasu arteritis, five had polyarteritis nodosa, four had cutaneous polyarteritis, three had Behçet’s disease and one had microscopic polyangiitis. Of the 615 patients with HSP included in the analyses, 49.8% had HSP nephritis (for 90% of whom it occurred within 1 week of disease onset). Of the 470 patients with KD included in the analyses, 13.8% were diagnosed with incomplete KD and 29.0% had a concurrent coronary artery lesion. For the 23 patients with Takayasu arteritis, the common clinical symptoms were hypertension, asphygmia/weak pulse and heart failure; only one of these patients had been diagnosed at an early disease stage. The five patients with polyarteritis nodosa received immunosuppressant therapy following diagnosis. Other vasculitides were uncommon.ConclusionsThe most common primary vasculitides in this population of children from Eastern China were HSP and KD; other vasculitides were rare. Paediatricians should be suspicious of vasculitis when there is evidence of systemic inflammation and multisystem disease that cannot be explained by one specific disorder.

Urinary myeloid IgA Fc alpha receptor (CD89) and transglutaminase-2 as new biomarkers for active IgA nephropathy and henoch-Schönlein purpura nephritis

BackgroundIgA nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN) are glomerular diseases that share a common and central pathogenic mechanism. The formation of immune complexes containing IgA1, myeloid IgA Fc alpha receptor (FcαRI/CD89) and transglutaminase-2 (TG2) is observed in both conditions. Therefore, urinary CD89 and TG2 could be potential biomarkers to identify active IgAN/HSPN. MethodsIn this multicenter study, 160 patients with IgAN or HSPN were enrolled. Urinary concentrations of CD89 and TG2, as well as some other biochemical parameters, were measured. ResultsUrinary CD89 and TG2 were lower in patients with active IgAN/HSPN compared to IgAN/HSPN patients in complete remission (P<0.001). The CD89xTG2 formula had a high ability to discriminate active from inactive IgAN/HSPN in both situations: CD89xTG2/proteinuria ratio (AUC: 0.84, P<0.001, sensitivity: 76%, specificity: 74%) and CD89xTG2/urinary creatinine ratio (AUC: 0.82, P<0.001, sensitivity: 75%, specificity: 74%). Significant correlations between urinary CD89 and TG2 (r=0.711, P<0.001), proteinuria and urinary CD89 (r=−0.585, P<0.001), and proteinuria and urinary TG2 (r=−0.620, P<0.001) were observed. ConclusionsDetermination of CD89 and TG2 in urine samples can be useful to identify patients with active IgAN/HSPN.