Henoch-Schönlein Purpura Patients Research Articles

Exploring potential predictors of Henoch-Schönlein purpura nephritis: a pilot investigation on urinary metabolites.

Henoch-Schönlein purpura nephritis (HSPN) is the most severe manifestation of Henoch-Schönlein purpura (HSP). This study aimed to determine the role of urine metabolomics in predicting HSPN and explore the potential mechanisms of HSP. A liquid chromatography-tandem mass spectrometry-based untargeted metabolomics analysis was performed to investigate the urinary metabolic profiles of 90 participants, comprising 30 healthy children (group CON) and 60 patients with HSP, including 30 HSP patients without renal involvement (group H) and 30 HSPN patients (group HSPN). The differentially expressed metabolites (DEMs) were identified using orthogonal partial least squares discriminant analysis (OPLS-DA), and subsequent bioinformatics analysis was conducted to elucidate the perturbed metabolic pathways. A total of 43 DEMs between H and HSPN groups were analyzed by the Kyoto Encyclopedia of Gene and Genome (KEGG) database, and the result indicates that glycine, serine and threonine metabolism, and cysteine and methionine metabolism were significantly disturbed. A composite model incorporating propionylcarnitine and indophenol sulfate was developed to assess the risk of renal involvement in pediatric patients with HSP. Conclusion: This study reveals the metabolic alterations in healthy children, HSPN patients, and HSP patients without renal involvement. Furthermore, propionylcarnitine and indophenol sulfate may be potential predictive biomarkers of the occurrence of HSPN. What is Known: • HSP is the predominant type of vasculitis observed in children. The long-term prognosis of HSP is contingent upon the extent of renal impairment. In severe nephritis, a delay in appropriate treatment may lead to fibrosis progression and subsequent development of chronic kidney disease (CKD), even leading to renal failure. • The application of metabolomics in investigating diverse renal disorders has been documented. Urine is a robust and sensitive medium for metabolomics detection. What is New: • The metabolic profiles were identified in urine samples of healthy children and those with HSP at the early stage of the disease. Different metabolites were identified between HSP patients without nephritis and those who developed HSPN. • These different metabolites may affect oxidative stress in the progression of HSPN.

Association between polymorphism at codon 469 of the ICAM-1 gene and Henoch-Schönlein purpura in an Iranian cohort

Henoch-Schönlein purpura (HSP) is a common form of IgA1-mediated blood vessel inflammation affecting mainly children. Intercellular adhesion molecule-1 (ICAM-1) gene polymorphisms have been shown to be associated with HSP in different populations; in this study, we investigated its potential association and influence on the development of severe complications in Iranian HSP patients. Twenty-three patients diagnosed with IgAV/HSP according to the criteria of the American College of Rheumatology (ACR) with 53 age- and sex-matched control subjects were referred to us. Cases and controls were genotyped using Sanger sequencing. Based on our research data, we found an association between codon 469 K/E of the ICAM1 gene and risk of HSP. Our results revealed that KK genotype and allele K are more common in control than in the HSP group, therefore the subjects with KK genotype are protected against HSP. Our data also suggested that the genotype EE is associated with higher risk of HSP progression compared to KK genotype.

Ang-1, Ang-2, and Tie2 are diagnostic biomarkers for Henoch-Schönlein purpura and pediatric-onset systemic lupus erythematous.

Henoch-Schönlein purpura (HSP) and pediatric-onset systemic lupus erythematosus (pSLE) are closely associated with vasculitis and vascular diseases. This study aimed to investigate the clinical diagnostic values of Ang-1, Ang-2, and Tie2 for HSP and pSLE. We surveyed 82 HSP patients, 34 pSLE patients, and 10 healthy children. The expression levels of Ang-1, Ang-2, and Tie2 in the serum and urine were assessed using enzyme-linked immunosorbent assay. The diagnostic values of Ang-1, Ang-2, and Tie2 for HSP and pSLE were evaluated using receiver operating characteristic curve analysis. The results revealed that the serum and urine expression levels of Ang-2 and Tie2 were significantly elevated in HSP and pSLE patients, whereas the Ang-1/Ang-2 values were reduced. Additionally, Ang-1 was highly expressed in the serum and urine of HSP patients and in the serum of pSLE patients. Ang-1, Ang-2, and Tie2 showed differential expression in various types of HSP and pSLE compared with their expression in healthy controls. In summary, Ang-1, Ang-2, and Tie2 can serve as biomarkers for HSP and pSLE. Moreover, Ang-1/Ang-2 values are reduced in HSP and pSLE patients. Ang-1, Ang-2, and Tie2 can be used as biomarkers for HSP and pSLE.

Incidence and risk factors of nephritis in childhood Henoch-Schonlein purpura

Background Henoch-Schönlein Purpura (HSP) is the most common systemic vasculitis disease in children. It is characterized by involvement of the skin, joints, gastrointestinal tract, and kidney. Kidney manifestations may progress to severe nephritis, even lead to end-stage kidney disease.
 Objective To identify the incidence and risk factors of nephritis in childhood HSP.
 Methods A retrospective cohort study was performed to evaluate clinical, demographic, laboratory, and therapeutic parameters of HSP patients aged 0-18 years between 2011-2019 at Dr. Cipto Mangunkusumo Hospital, Jakarta. Diagnoses of HSP were made according to the 2008 EULAR/PRES/PRINTO criteria. Wefollowed subjects’ medical records for at least 3 months after disease onset to observe incidence and risk factors of Henoch-Schönlein nephritis (HSN).Results There were 112 HSP patients (aged 2-17 years) included in this study. HSN was found in 40 out of 112 patients (35.7%). Nephritis developed within the first 4 weeks for a majority of cases. Multivariate analysis showed that persistent purpura (OR 3.306; 95%CI 1.315 to 8.315; P=0.011) and acute phase leukocytosis(OR 2.585; 95%CI 1.047 to 6.385; P=0.039) were significantly associated risk factors for HSN. We found that corticosteroid use did not reduce the risk of HSN. The accumulation of several risk factors was associated with the likelihood of developing HSN.
 Conclusion Persistent purpura and acute phase leukocytosis are independent risk factors for HSN. Therefore, blood tests are needed to estimate the risk of HSN. Early corticosteroid therapy do not reduce the risk of kidney impairment.

Serum total bile acid levels assist in the prediction of acute intussusception with abdominal type Henoch-Schonlein purpura in children.

The severe acute abdomen associated with Henoch-Schonlein purpura (HSP) is an acute intussusception (AI). There is no reliable specific marker for AI with abdominal-type HSP. The serum total bile acid (TBA) level is a new prognostic marker associated with the severity of intestinal inflammation. The purpose of this study was to identify the prognostic value of serum TBA levels for the diagnosis of AI in children with abdominal-type HSP. A retrospective study of 708 patients with abdominal-type HSP was conducted, with demographic data, clinical symptoms, hepatic function index, immune function markers, and clinical outcomes assessed. Patients were divided into two groups: HSP (613 patients) and HSP with AI (95 patients). The data were analysed using SPSS 22.0. Of the 708 patients, the serum TBA levels were higher in the HSP with AI group than in the HSP group (P < 0.05). Logistic regression analysis showed that vomiting (OR = 396.492, 95% CI = 14.93-10,529.67, P < 0.001), haematochezia (OR = 87.436, 95% CI = 5.944-1,286.214, P = 0.001), TBA (OR = 16.287, 95% CI = 4.83-54.922, P < 0.001), and D-dimer (OR = 5.987, 95% CI = 1.892-15.834, P = 0.003) were independent risk factors for abdominal-type HSP with AI. Receiver operating characteristic (ROC) curve analysis showed that the optimal cut-off serum TBA value (sensitivity = 91.58%, specificity = 84.67%, AUC = 93.6524%) was >3 μmol/L for predicting AI in children with abdominal-type HSP. In this group of HSP patients with AI, a serum TBA level ≥6.98 μmol/L was significantly associated with an increased incidence of operative treatment (51.85% vs. 75.61%, P = 0.0181), intestinal necrosis (9.26% vs. 29.27%, P = 0.0117), and length of hospital stay [15.76 ± 5.31 vs. 10.98 ± 2.83 (days), P < 0.0001]. In children with HSP and AI, the serum TBA level was significantly higher. A novel but promising haematological indicator, the serum TBA level, helps identify HSP with and without AI and predicts intestinal necrosis in HSP with AI.

Adult Henoch-Schönlein Purpura: Comprehensive Assessment of Demographic, Clinical, and Histopathological Features as Predictors for Systemic Involvement

Background: Henoch-Schönlein purpura (HSP) is a small-vessel IgA-predominant vasculitis. A major challenge in managing adult HSP is the difficulty assessing the risk of systemic involvement. There is currently a paucity of data in this area. Objective: The objective of this study was to determine demographic, clinical, and histopathological features associated with systemic involvement in adult HSP. Methods: In this retrospective study, we reviewed demographical features and clinical and pathology data of 112 adult HSP patients seen at Emek Medical Center between January 2008 and December 2020. Results: Of these patients, 41 (36.6%) had renal involvement, 24 (21.4%) had gastrointestinal tract involvement, and 31 (27.7%) had joint involvement. Age >30 years (p = 0.006) at diagnosis was an independent predictor of renal involvement. Platelet count (<150 K/µL) (p = 0.020) and apoptosis of keratinocytes on skin biopsy (p = 0.031) were also associated with renal involvement. History of autoimmune disease (p = 0.001), positive c-antineutrophil cytoplasmic antibody (p = 0.018), positive rheumatoid factor (p = 0.029), and elevated erythrocyte sedimentation rate (p = 0.04) were associated with joint involvement. Female sex (p = 0.003), Arab race (p = 0.036), and positive pANCA (p = 0.011) were associated with gastrointestinal tract involvement. Limitations: This study is retrospective. Conclusion: These findings may serve as a guide to stratify risk in adult HSP patients so that those at higher risk can be monitored more closely.

Henoch-Schonlein Purpura in Adult with Gastrointestinal and Renal Involvement

Background: The pathogenesis of Henoch-Schonlein Purpura (HSP), a multisystem organ-involved small vessel vasculitis, is unknown. HSP is more common in youngsters than in adults. HSP is associated with a history of malignancies, medications, vaccinations, and upper respiratory tract infections. Painful purpura, arthritis, stomach discomfort, and renal involvement are symptoms that may be seen in HSP patients. Adult patients had a much higher rate of renal involvement than children. Purpose: To report a case of HSP in an adult with gastrointestinal and renal involvement. Case: A 45-year-old man complained of an arm, leg, and waist rash for two weeks before admission to the hospital. He also has stomach pains, nausea, and vomiting to deal with. His symptoms have just appeared now for the first time. During a renal function test, blood urea nitrogen (BUN) and creatine serum levels rose. On a urinalysis, proteinuria and microscopic hematuria were found. Leucocytoclastic vasculitis was discovered during the histopathology investigation. HSP diagnosis was based on the ACR and ICC criteria. The patient was given 3x2 tablets of 0.5 mg dexamethasone, 3x2 tablets of cetirizine, 2x10 mg lisinopril, 2x50 mg ranitidine injection, and 2 grams ceftriaxone. Discussion: The clinical symptoms of HSP are used to make the diagnosis. In the vast majority of instances, the treatment is only supportive therapy. Corticosteroid usually are usually used for HSP with multisystem organ involvement to reduce pain severity and faster resolution of renal manifestation. Conclusion: Follow up on renal function is needed to monitor the worsening of renal disease.

Time of Onset and Risk Factors of Renal Involvement in Children with Henoch-Schönlein Purpura: Retrospective Study.

Background: Henoch-Schönlein purpura (HSP) is a common systemic vasculitis in children, involving the skin, musculoskeletal system, gastrointestinal tract and kidneys. Some studies in children have shown possible risk factors linked with the development and severity of HSP Nephritis (HSPN). The aim of this study was to research predicting factors for the development of HSPN. Methods: We retrospectively evaluated 132 pediatric patients with HSP, according to EULAR/PRINTO/PRESS criteria. All patients were screened for HSPN by urinalysis. Finally, we compared demographic, clinical and laboratory data in HSP patients with and without nephritis. Results: The median age at HSP diagnosis [6.2 (2.6–17.5) vs. 5.5 (0.8–15.4) years, p = 0.03] and the incidence of abdominal pain (48 vs. 27%, p = 0.01) were significantly higher in HSPN patients. No differences were evidenced regarding gender, allergic diseases, skin recurrences, gastrointestinal involvement, musculoskeletal involvement, scrotal involvement, and laboratory data (white blood cell count, neutrophil count, lymphocyte count, platelet count, C-reactive protein, erythrocyte sedimentation rate, and blood concentration of IgA). Conclusions: The age at diagnosis and abdominal pain were independent risk factors for renal involvement in HSP patients. However, due to the retrospective nature of this study, further long-term and prospective studies will be necessary.

215.11: Evaluating Kidney Transplant Characteristics and Outcomes in Patients With Henoch-Schönlein Purpura

Introduction: Henoch-Schönlein Purpura (HSP) is a small-vessel vasculitis characterized by deposits of immunoglobulin A (IgA) in target tissues. HSP is more frequently seen in children and usually has benign and self-limiting renal involvement; however, it is estimated that up to 2% of children with HSP nephritis can develop End-Stage Renal Disease (ESRD) and require renal replacement therapy. Due to the infrequency of HSP causing ESRD, large-scale studies of HSP and kidney transplant (KT) outcomes are scarce. Methods: We used data from Scientific Registry of Transplant Recipients to evaluate differences for HSP patients listed for KT. Adult and pediatric patients listed from October 1987 to April 2021 were included. Recipients without HSP were randomly selected and matched in a 3:1 ratio to HSP recipients according to age (exact year), gender, ethnicity, donor source (living versus deceased), and year of transplantation (±3 years). Inferential statistics were used to evaluate differences between HSP and non-HSP patients. Regression modeling using a forward conditional approach and Kaplan-Meier survival analysis with a Mantel-Cox log-rank test were performed to calculate survivals and compare outcomes. Results: During the study time period, 504,878 KT were performed; 893 (0.17%) were for HSP. Compared with non-HSP patients, HSP KT recipients were younger (30.6±14.2vs 47.2±15.8; p<0.001) and more commonly Caucasian (87.5% vs 68.6%; p<0.001), received a living donor allograft (44.5 vs 32.9; p<0.001), and female (45.3% vs. 39.5%, p=0.001). Most HSP KT patients were preemptive (79.8%). A total of 773 HSP recipients were matched to 2,319 non-HSP recipients. For HSP patients, 1-, 3-, and 5-year death-censored graft survival (DCGS) was 87.3%, 70.4%, and 53.3%, respectively. For matched non-HSP patients, DCGS was 86.9%, 70.2%, and 52.5%, respectively (all p>0.05). Overall patient survival for HSP patients was 89.8%, 72.4%, and 55.6% at 1, 3, and 5 years, respectively. Overall patient survival for matched non-HSP patients was 89.3%, 72.9%, and 55.4% at 1, 3, and 5 years, respectively (all p>0.05). Acute rejection and graft thrombosis were the two leading causes of graft loss for both HSP (28.6% and 19.0%, resp.) and non-HSP patients (18.8% and 31.6%, resp.). In HSP patients who did not suffer recurrence, 1-, 3-, 5-, and 10-year DCGS was 95.8%, 88.8%, 81.3%, and 64.0%, respectively. Using regression model analysis of HSP KT patients for recurrence, older age (OR, 95% CI 1.05, 1.03-1.07, p<0.001) was significantly associated with recurrence; while a living donor allograft (0.50, 0.32-0.79, p=0.003) and a higher BMI (0.95, 0.91-0.99, p=0.020) were protective. Conclusion: In a contemporary cohort of HSP KT patients, we demonstrate comparable graft and patient survival for HSP patients, compared to matched non-HSP patients, and superior survival if recurrence can be avoided.

The Effect of Prednisone Dose on the Clinical and Laboratory Course of Henoch–Schönlein Purpura in Children

Objective: Henoch–Schönlein purpura (HSP) is the most common form of systemic vasculitis in children. Most cases are self-limited. Corticosteroids may be considered for HSP patients with severe renal or extrarenal involvement. However, they have some adverse effects related to the dose and the period of treatment. The study aimed to assess the efficacy and safety of dose 1mg/kg/day of prednisone in the treatment of HSP clinical and laboratory manifestations compared with dose 2 mg/kg/day. Methods: A total of 28 patients (14 treated with prednisone 1 mg/kg and 14 treated with prednisone 2 mg/kg, followed up for 6 months) were included in a prospective randomized trial study, at University Children Hospital in Damascus, Syria, between 2020 and 2021. Results: As prednisone was only used in severe manifestations, severe abdominal pain was the most common indication (53.5%), followed by gastrointestinal hemorrhage (32.1%). However, prednisone effect on all symptoms and laboratory findings for all patients was studied. Both doses of prednisone (1 and 2 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) were effective in reducing the intensity of extrarenal symptoms, and no statistical difference was found (rash: P = 0.47, abdominal pain: P = 0.32, gastrointestinal hemorrhage: P = 0.32, arthritis: P = 0.84, and soft-tissues edema: P = 1). In both groups, new renal symptoms and gastrointestinal complications were developed. Elevated inflammatory markers (CRP: 59.3%, ESR: 77.3%) turned up normal at the end of treatment in both groups. Children treated with prednisone 2mg/kg showed increased appetite; three cases developed Cushing’s moon face; one case showed a transient hypertension at the end of treatment. Conclusions: The routine, early use of prednisone in uncomplicated HSP is not recommended. However, when it is indicated, a 2-week course of prednisone 1 mg/kg/day could be an effective alternative to the higher dose (2 mg/kg/day), with less side effects.

Anesthetic Management of A Patient with Henoch-Schonlein Purpura for Caesarean Section

Introduction: Henoch-Schonlein Purpura or Immunoglobulin-A vasculitis is a systemic vasculitis caused by immune complexes that attack small blood vessels. The classic symptoms of Henoch-Schonlein Purpura include erythema purpura, arthralgia, gastrointestinal complaints, and renal involvement. Some cases show that pregnancy itself could be the trigger for its recurrence and lead to early delivery. Case report: A 33-year-old patient, G2P1A0 and 35 weeks and 4 days pregnant complained of diarrhea 8 days before hospital admission (8-15 times per day). The patient was diagnosed with Henoch-Schonlein Purpura 3 years ago. Upon monitoring in the ward, the fetus was found to be in a compromised condition and an emergency cesarean section was needed. The patient was assessed as having an ASA II physical status and was anesthetized with regional anesthesia epidural in the sitting position, with a median approach, puncture at L3-L4 level, and with 12 ml of Bupivacaine 0.5% isobaric. Postoperative care was continued in the ward. Discussion: As long as there are no contraindications, a neuraxial block could be performed on parturient patients with Henoch-Schonlein Purpura who would undergo a cesarean section. Neuraxial block, namely epidural block, has the added advantage of being a postoperative analgesic and helps to avoid the use of Non-Steroidal Anti Inflammatory Drugs (NSAIDs) in Henoch-Schonlein Purpura patients who often have renal complications. Conclusion: Caesarean Section with Henoch-Schonlein Purpura disease has been reported with Epidural Block Anesthesia without complications.

Investigation of neuraminidase 1 gene association in Henoch-Schönlein Purpura (HSP) with renal involvement

Objectives: HSP is a common small vessel vasculitis. It is the most common cause of non-thrombocytopenic purpura in childhood. The role of genes in etiopathogenesis of the disease, which has not yet been clearly elucidated, is being emphasized. Many genes called sialidases are being studied and is thought that the NEU1 gene may be particularly important in the etiopathogenesis of HSP. The aim of this study is to investigate the role of the NEU1 gene in the etiopathogenesis of HSP and its relation to renal involvement. Materials and methods: Fifty patients followed in the Celal Bayar University Hafsa Sultan Hospital Pediatric Nephrology Department, with the diagnosis of HSP renal involvement were included into the study. For the control group, age and gender matched 50 cases were accepted among the outpatients admitted to Pediatric Department without any chronic diseases. NEU1 gene mutation analysis was performed in blood samples of both patient and control groups by using the Sanger DNA sequencing method. Results: NEU1 genetic mutation was not detected in any HSP patient with renal involvement and control group. Conclusion: In our study, the NEU 1 gene was not found to be associated with HSP nephritis. No changes were detected in the investigated regions of the NEU1 gene.

Serum immunoglobulin profiles in Chinese children with Henoch-Schönlein purpura.

The present study focuses on the associations of serum immunoglobulin with disease activity, relapse/recurrence and renal involvement in Henoch-Schönlein purpura (HSP) children. 1683 Chinese children with HSP were recruited from January 2015 to January 2021. Laboratory data of blood samples and urine tests were collected. Renal biopsy was performed by the percutaneous technique. Patients' histories associated with HSP onset were obtained by interviews and questionnaires. (1) IgA and IgE were dramatically elevated in HSP patients as compared to their normal values and subject to a 1.75-3.09 fold and a 1.97-2.61 fold increase, respectively. (2) No significant correlation of the disease activity with IgA and IgE was determined, respectively. (3) The serum IgA levels were significantly lower in patients with relapse/recurrence than that in patients without relapse/recurrence, which may be attributed to the transmission of IgA-mediated immune complexes from blood to vessel walls. (4) No significant correlation was found between serum IgA, IgE levels and the pathological classification. HSP children have marked disorders of serum immunoglobulin profiles, characterized by significant increases in IgA and IgE. The detection of serum IgA may be applicable to predict relapse/recurrence of HSP whereas not associated with disease activity and renal involvement.

Lack of Association Between HMGB1 Gene Polymorphism and Risk of Henoch–Schönlein Purpura in Childhood

Background: High-mobility group box-1 (HMGB1), a nuclear protein, plays an important role in the pathogenesis of Henoch-Schönlein purpura (HSP). In a Chinese child population, the correlation between susceptibility to HSP and genetic variation in the HMGB1 gene and also the relationship between HMGB1 gene polymorphism and clinical heterogeneity of HSP were investigated. Methods:: We analyzed two HMGB1 tag single nucleotide polymorphisms (SNPs; rs3742305 and rs9508752) in 182 HSP patients and 202 healthy controls using the matrix-assisted laser desorption/ionization time-of-flight mass spectrometry method. Results: There were no significant differences between HSP patients and controls in the frequency of alleles, genotypes, and haplotypes of HMGB1 SNPs. In addition, there was a slight association between HMGB1 gene polymorphisms and the clinical manifestations of HSP. Conclusions: It is suggested that the variation of the HMGB1 gene was not highly correlated with the susceptibility of Chinese children to HSP.

Let-7a Targeting TNFAPI3 Promotes Vascular Endothelial Cell Apoptosis of Pediatric Patients with Henoch-Schönlein Purpura via NF-κB Signaling Pathway.

Objective We aimed at exploring the role of let-7a in the pathogenesis of pediatric Henoch–Schönlein purpura (HSP) and its related mechanism. Methods Fifty-five pediatric HSP patients and 20 paired healthy controls were included. The expressions of let-7a and TNFAIP3 were detected by RT-qPCR or/and western blot. Vessel fibrinoid necrosis was evaluated in skin tissues by PTAH staining. The serum IgA level was measured by ELISA. Cells were transfected with let-7a inhibitor and/or TNFAIP3 siRNA, accompanied by pretreatment with NF-κB inhibitor PDTC or not. After being cultured in HSP serum, the changes in cell viability, cell apoptosis, apoptosis-related proteins, and NF-κB pathway-related proteins were detected by CCK8, flow cytometry, and western blot. Results The let-7a expression level was positively correlated with the serum IgA level and severity degree of vascular fibrinoid necrosis in HSP patients. Let-7a expression was significantly increased, whereas cell viability and TNFAIP3 expression were obviously decreased 48 h after HUVECs were incubated with HSP serum. Let-7a knockdown upregulated the cell viability, whereas it reduced the apoptotic ratio, apoptosis protein expressions (Bax/Bcl2 ratio, cleaved-caspase 3), and NF-κB pathway activation (reflected by reduced P65 nuclear translocation and p-IκBα expression) in HUVECs (all p < 0.05). The changes induced by let-7a knockdown were obviously reversed by TNFAIP3 siRNA transfection (p < 0.05). Besides, PDTC treatment remarkably diminished the anti-apoptosis effect of let-7a knockdown and pro-apoptosis effect of TNFAIP3 siRNA on HUVECs induced by HSP serum. Conclusions Let-7a knockdown significantly suppressed vascular endothelial cell apoptosis induced by HSP serum by targeting TNFAPI3 via NF-κB signaling pathway. Our findings provided a potential therapeutic target for the treatment of HSP.

Analysis of children with Henoch–Schonlein purpura secondary to infection

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. We conducted a retrospective study of 208 HSP children and 98 healthy children. Clinical parameters were compared in those cases. 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. 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.

Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura.

ObjectiveHenoch–Schönlein purpura (HSP) is the most common vasculitis in children. Renal involvement is the main long-term complication of HSP, and presently there is no way to predict which children may have irreversible renal damage from the outset. This study aimed to explore the prediction value of laboratory indexes on renal involvement in children with HSP, which could help the early identification and intervention.MethodsChildren with HSP hospitalized at the First Affiliated Hospital of Henan University of Chinese Medicine from June 2019 to December 2020 were included. The demographic findings, clinical features, laboratory findings including blood routine examination, serum immunoglobulin, complement, T cell subsets levels, liver and kidney function, coagulation function were recorded. Laboratory indexes were analyzed, logistic regression analysis was performed to identify the independent predictors in HSP patients with renal involvement, and receiver operating characteristic (ROC) curves were further used to assess the value of prediction indexes, as well as the efficacy of combined diagnosis.ResultsThe study included 146 HSP patients, among them, 50 patients (34.2%) had renal involvement. Age, platelet distribution width (PDW), CD3+ and fibrinogen (FIB) were positively correlated with renal involvement, while the levels of Immunoglobulin G (IgG), C-reactive protein (CRP), and neutrophil/lymphocyte ratio (NLR) were negatively correlated with renal involvement. The area under the ROC Curve (AUC) of these biomarkers ranged from 0.6284 to 0.7009, and among the combinations, a combination of NLR, CRP, CD3+, FIB, PDW, IgG and age had the best AUC value (0.9774).ConclusionAge, PDW, CD3+, FIB, CRP, NLR and IgG were prediction indexes for renal involvement in HSP patients, and these indexes can be combined appropriately to improve the diagnostic efficacy.

Differences in Manifestations and Gut Microbiota Composition Between Patients With Different Henoch-Schonlein Purpura Phenotypes.

BackgroundGut 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.Methods16SrRNA 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.ResultsThe 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.ConclusionsThe 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).

ANCA-associated vasculitis with protein-losing enteropathy is characterized by hypocomplementemia.

Protein-losing enteropathy (PLE) has been reported to be associated with various systemic autoimmune diseases. However, reports regarding PLE in ANCA-associated vasculitis (AAV) patients are limited. We herein aimed to describe the clinical characteristics of AAV with PLE. We conducted a retrospective chart review of patients who were diagnosed with AAV and who began treatment at the University of Tokyo Hospital between June 2003 and June 2020. Among 68 AAV patients, there were four patients (5.9%) with PLE, consisting of two patients with MPA, one patient with GPA, and one patient with EGPA. Clinical courses were described, and their data were compared with AAV patients without PLE. Demographic characteristics, disease activity, and the pattern of organ involvement were similar between patients with PLE and without PLE. Patients with PLE had hypocomplementemia more frequently than the patients without PLE (CH50 75.0% vs 1.8%, p < 0.001, C3 50.0% vs 1.8%, p = 0.01, C4 75.0% vs 3.5%, p = 0.001). Although hypoalbuminemia improved with immunosuppressive therapy for AAV, the improvement in hypoalbuminemia was slow in most cases. We also performed a systematic review on PLE associated with vasculitis. Thirteen reports were included, and Henoch-Schonlein Purpura patients with PLE also tended to have hypocomplementemia. In conclusion, PLE is a rare complication of AAV and complement system may associate with the mechanism of PLE.

The changes in pyroptosis-related inflammatory factors in the peripheral blood of patients with Henoch-Schonlein purpura.

Henoch-Schonlein purpura (HSP) is a common capillary allergic bleeding disease. To explore the variation of pyroptosis-related inflammatory factors level in the peripheral blood of patients with HSP. A total of 87 HSP patients treated in our hospital from June 2020 to March 2021 were selected and divided into the renal impairment group (n=29) and the non-renal impairment group (n=58) according to the presence of hematuria and proteinuria. A total of 50 healthy individuals from the hospital were selected as the control group. The renal impairment and non-renal impairment groups were treated with a regular regimen of compound glycyrrhizin tablets and glucocorticoids, respectively. Serum interleukin (IL)-18, IL-1β, and peripheral caspase-1-positive cells were compared pre- and post-treatment among the three groups. The pre-treatment serum IL-1β levels in the renal impairment and non-renal impairment groups were significantly higher than that in the control group (P<0.01). After treatment, the IL-1β level in the non-renal impairment group was not significantly different from that in the control group (P>0.05). However, the IL-1β level in the renal impairment group post-treatment was significantly higher than that in the other two groups (P<0.01). The positive rate of caspase-1 expression in peripheral blood before treatment in the renal impairment group and non-renal impairment group was significantly higher than that in the control group (P<0.01). After treatment, the positive rate of caspase-1 expression in the non-renal impairment group was comparable to that in the control group (P>0.05), whereas the rate in the renal impairment group was significantly higher than that in the other two groups (P<0.01). After treatment, the serum IL-1β levels and caspase-1 positive rate in HSP patients who were responsive to treatment (as assessed by hematuria or proteinuria levels after treatment) were lower than that in patients who were unresponsive to treatment P<0.001), but not significantly different to the control group (P>0.05). The levels of serum IL-1β and caspase-1 changed in response to alterations in the disease condition and treatment response in HSP patients, which suggested that pyroptosis-related inflammatory factors may have potential application value in predicting disease progression and efficacy of hormone therapy.