Asunto(s)

Vasculitis por IgA , Síndrome Nefrótico , Humanos , Síndrome Nefrótico/diagnóstico , Síndrome Nefrótico/inmunología , Síndrome Nefrótico/tratamiento farmacológico , Vasculitis por IgA/diagnóstico , Vasculitis por IgA/tratamiento farmacológico , Vasculitis por IgA/complicaciones , Vasculitis por IgA/inmunología , Resultado del Tratamiento , Niño , Masculino , Femenino , Inmunoglobulina A/sangre , Inmunoglobulina A/inmunología , Inmunosupresores/uso terapéutico , Biopsia

RESUMEN

Duodenal ulcer (DU) is the most common gastroscopic manifestation of abdominal Henoch-Schönlein purpura (HSP), which may cause severe bleeding and often requires esophagogastroduodenoscopy (EGD) to confirm the diagnosis. However, the condition of children with HSP changes rapidly; not all children are able to undergo EGD on time, and some hospitals do not have a pediatric EGD unit. Therefore, assessing the risk factors for developing DU in HSP using simple and readily available indicators is essential. Children with HSP at Wuhan Children Hospital from June 2020 to June 2022 were included in the training set and completed EGD. The patients were divided into 2 groups: those with (DU group) and without DU (non-DU group). Data were collected from the 2 groups, and univariate and multivariate logistic regression analyses were used to compare the 2 groups. Children with HSP admitted between July 2022 and June 2023 were included in the validation set. Four indicators, prognostic nutrition index, albumin (ALB), gastrointestinal (GI) bleeding, and duration of onset before EGD, were found in the DU and non-DU groups. GI bleeding and prognostic nutritional index (PNI) ≤ 53.0 have strong predictability for patients with HSP and DU. GI bleeding and PNI ≤ 53.0 may provide new reference evidence for condition assessment and treatment.

Asunto(s)

Úlcera Duodenal , Hemorragia Gastrointestinal , Vasculitis por IgA , Humanos , Vasculitis por IgA/complicaciones , Vasculitis por IgA/diagnóstico , Masculino , Niño , Femenino , Estudios Retrospectivos , Úlcera Duodenal/complicaciones , Estudios de Casos y Controles , Pronóstico , Preescolar , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/diagnóstico , Hospitalización/estadística & datos numéricos , Evaluación Nutricional , Endoscopía del Sistema Digestivo/métodos , Factores de Riesgo , Adolescente , China/epidemiología

RESUMEN

The utility of Rituximab (RTX) for IgA vasculitis nephritis (IgAVN) is not well established. Up to now, we analysed the largest samples of IgAVN patients treated by RTX with a total of 41 retrieved subjects up to December 29, 2023 in the present systematic review. We assessed the clinical profiles, efficacy, and safety of RTX treatments. The present review showed that the renal function tended to be stabilized (P = 1.000) and urinalysis tended to normalize after RTX treatment with no serious adverse events reported. Moreover, 40% (16/40) of patients was freed use of glucocorticoid after RTX administration (P < 0.001). The remission rate was 92.7% (38/41) and complete remission rate was 46.3% (19/41) in IgAVN patients. Interestingly, 76.9% (10/13) of IgAVN child patients achieved complete remission when compared with 32.1% (9/28) of adult patients (P = 0.017). In summary, our results support the benefit of RTX therapy in IgAVN patients, especially children subjects.

Asunto(s)

Rituximab , Humanos , Rituximab/uso terapéutico , Resultado del Tratamiento , Masculino , Adulto , Femenino , Factores Inmunológicos/uso terapéutico , Niño , Glomerulonefritis por IGA/tratamiento farmacológico , Inducción de Remisión , Vasculitis por IgA/tratamiento farmacológico , Nefritis/tratamiento farmacológico , Persona de Mediana Edad , Vasculitis/tratamiento farmacológico

RESUMEN

OBJECTIVE: Investigate Proanthocyanidins (PCs) efficacy and mechanisms in treating Henoch-Schönlein purpura (HSP)-like rat models, focusing on inflammatory and oxidative stress (OS) responses. METHODS: An HSP-like rat model was established using ovalbumin (OVA) injection, leading to symptoms mimicking HSP. The study measured inflammatory markers (IL-4, IL-17, TNF-α), OS markers (MDA, SOD, CAT), and assessed the TLR4/MyD88/NF-κB signaling pathway's involvement via histopathological and immunofluorescence analyses. RESULTS: PCs treatment significantly improved HSP-like symptoms, reduced inflammatory cell infiltration, and decreased IgA deposition in renal mesangial areas. Serum analyses revealed that PCs effectively lowered IL-4, IL-17, TNF-α, and MDA levels while increasing SOD and CAT levels (p < 0.05). Crucially, PCs also downregulated TLR4, MyD88, and NF-κB expressions, highlighting the blockage of the TLR4-mediated signaling pathway as a key mechanism. CONCLUSION: PCs show promising therapeutic effects in HSP-like rats by mitigating inflammatory responses and oxidative damage, primarily through inhibiting the TLR4/MyD88/NF-κB pathway. These findings suggest PCs as a potential treatment avenue for HSP, warranting further investigation.

Asunto(s)

Modelos Animales de Enfermedad , Vasculitis por IgA , Factor 88 de Diferenciación Mieloide , FN-kappa B , Estrés Oxidativo , Proantocianidinas , Transducción de Señal , Receptor Toll-Like 4 , Animales , Receptor Toll-Like 4/metabolismo , Estrés Oxidativo/efectos de los fármacos , Factor 88 de Diferenciación Mieloide/metabolismo , Vasculitis por IgA/tratamiento farmacológico , Ratas , FN-kappa B/metabolismo , Proantocianidinas/farmacología , Transducción de Señal/efectos de los fármacos , Masculino , Inflamación/tratamiento farmacológico , Ratas Sprague-Dawley

RESUMEN

Background: Regulating the immune system is a crucial measure of gut microbiota (GM) that influences the development of diseases. The causal role of GM on Henoch-Schönlein Purpura (HSP) and whether it can be mediated by immune cells is still unknown. Methods: We performed a two-sample Mendelian randomization study using an inverse variance weighted (IVW) method to examine the causal role of GM on HSP and the mediation effect of immune cells between the association of GM and HSP. Results: We demonstrated the causal relationships between 14 axas and 6 pathways with HSP. Additionally, we identified 9 immune cell characteristics associated with HSP. Importantly, through mediation MR analysis, we identified several immune cell characteristics that mediate the impact of GM on HSP. For instance, Genus_Blautia affects HSP via Monocyte (HLA DR on CD14+ CD16- monocyte) and Monocyte (HLA DR on monocyte). The proportion of mediation effects further elucidated the complex dynamics between GM exposure, immune markers, and their combined impact on HSP. Conclusion: The study suggested a causal relationship between GM and HSP, which may be mediated by immune cells.

Asunto(s)

Microbioma Gastrointestinal , Vasculitis por IgA , Análisis de la Aleatorización Mendeliana , Vasculitis por IgA/inmunología , Vasculitis por IgA/genética , Humanos , Microbioma Gastrointestinal/inmunología , Biomarcadores

RESUMEN

A 73-year-old man with lung squamous cell carcinoma was administered carboplatin + nab-paclitaxel + pembrolizumab for four cycles. Subsequently, he presented with multiple purpuras on his extremities, joint swelling on his fingers, abdominal pain, and diarrhea, accompanied by acute kidney injury (AKI), increased proteinuria, hematuria, and elevated C-reactive protein levels. Skin biopsy showed leukocytoclastic vasculitis as well as IgA and C3 deposition in the vessel walls. Based on these findings, the patient was diagnosed with IgA vasculitis as an immune-related adverse event (irAE) induced by carboplatin + nab-paclitaxel + pembrolizumab. After discontinuation of pembrolizumab and glucocorticoids, the symptoms immediately resolved. Regular monitoring of skin, blood tests, and urinalysis are necessary, and the possibility of irAE IgA vasculitis should be considered in cases of purpura and AKI during treatment with immune checkpoint inhibitors.

Asunto(s)

Albúminas , Anticuerpos Monoclonales Humanizados , Protocolos de Quimioterapia Combinada Antineoplásica , Carboplatino , Carcinoma de Células Escamosas , Neoplasias Pulmonares , Paclitaxel , Humanos , Masculino , Anciano , Carboplatino/efectos adversos , Carboplatino/administración & dosificación , Carboplatino/uso terapéutico , Paclitaxel/efectos adversos , Paclitaxel/administración & dosificación , Paclitaxel/uso terapéutico , Anticuerpos Monoclonales Humanizados/efectos adversos , Anticuerpos Monoclonales Humanizados/uso terapéutico , Anticuerpos Monoclonales Humanizados/administración & dosificación , Neoplasias Pulmonares/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma de Células Escamosas/tratamiento farmacológico , Albúminas/efectos adversos , Albúminas/administración & dosificación , Vasculitis por IgA/inducido químicamente , Vasculitis por IgA/diagnóstico , Inmunoglobulina A , Inhibidores de Puntos de Control Inmunológico/efectos adversos

RESUMEN

OBJECTIVE: The exact effects of MEFV variants on inflammation are still under investigation, and reports on variants of unknown significance, particularly the E148Q variant, have been conflicting. Therefore, this study aims to investigate patients exhibiting E148Q heterozygosity, focusing on diagnoses and disease courses to assist physicians in interpreting the variant. METHODS: Data of pediatric patients presenting to the Pediatric Rheumatology clinic between November 2016 and September 2023, exhibiting only E148Q heterozygosity in MEFV gene analysis, were extracted. Patients who were lost before 9 months of follow-up have been excluded to ensure the completion of initial diagnostic tests and evaluations. RESULTS: Among the 119 patients with E148Q variant, the diagnoses were as follows: healthy, 51.3%; IgA vasculitis, 10.1%; Familial Mediterranean Fever (FMF), 7.6%; Periodic fever, Aphtous stomatitis, Pharyngitis, Adenitis (PFAPA), 6.7%; and other diagnoses, 19.3%. IgA vasculitis patients experienced articular, gastrointestinal, and renal involvement at rates of 91.7%, 58.3%, and 16.7%, respectively. Complete response, partial response, and no response to colchicine were 37.5%, 12.5%, and 50%, respectively, in PFAPA patients. All FMF patients responded to colchicine treatment resulting in reduced mean FMF episode counts in 6 months from 3.22 ± 0.92 to 0.56 ± 0.52. CONCLUSIONS: The E148Q variant may amplify inflammation and modify disease courses. Patients with the E148Q variant experiencing typical FMF episodes should receive colchicine, but clinicians should exercise caution regarding alternative diagnoses. Additionally, the E148Q variant may increase acute phase reactants and disease severity in IgA vasculitis. However, to reach definitive conclusions on its treatment-modifying role in PFAPA, universal diagnosis and treatment response criteria should be adopted.

Asunto(s)

Colchicina , Fiebre Mediterránea Familiar , Heterocigoto , Pirina , Humanos , Femenino , Masculino , Niño , Fiebre Mediterránea Familiar/genética , Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/tratamiento farmacológico , Fiebre Mediterránea Familiar/fisiopatología , Pirina/genética , Colchicina/uso terapéutico , Preescolar , Adolescente , Vasculitis por IgA/genética , Vasculitis por IgA/diagnóstico , Mutación

Asunto(s)

Vacunas contra la COVID-19 , Vasculitis por IgA , Humanos , Vasculitis por IgA/diagnóstico , Vasculitis por IgA/inducido químicamente , Vacunas contra la COVID-19/efectos adversos , Vacunas contra la COVID-19/administración & dosificación , Anticuerpos Antinucleares/sangre , Anticuerpos Antinucleares/inmunología , COVID-19/prevención & control , COVID-19/complicaciones , Masculino , SARS-CoV-2/inmunología , Femenino

RESUMEN

OBJECTIVES: To explore the evidence, urinary biomarkers, and partial mechanisms of hypercoagulability in the pathogenesis of IgA vasculitis (IgAV). METHODS: Differential expression of proteins in the urine of 10 healthy children and 10 children with IgAV was screened using high-performance liquid chromatography-tandem mass spectrometry, followed by Reactome pathway analysis. Protein-protein interaction (PPI) network analysis was conducted using STRING and Cytoscape software. In the validation cohort, 15 healthy children and 25 children with IgAV were included, and the expression levels of differential urinary proteins were verified using enzyme-linked immunosorbent assay. RESULTS: A total of 772 differential proteins were identified between the IgAV group and the control group, with 768 upregulated and 4 downregulated. Reactome pathway enrichment results showed that neutrophil degranulation, platelet activation, and hemostasis pathways were involved in the pathogenesis of IgAV. Among the differential proteins, macrophage migration inhibitory factor (MIF) played a significant role in neutrophil degranulation and hemostasis, while thrombin was a key protein in platelet activation and hemostasis pathways. PPI analysis indicated that thrombin directly interacted with several proteins involved in inflammatory responses, and these interactions involved MIF. Validation results showed that compared to healthy children, children with IgAV had significantly higher urine thrombin/creatinine and urine MIF/creatinine levels (P<0.05). CONCLUSIONS: Thrombin contributes to the pathogenesis of IgAV through interactions with inflammatory factors. Urinary thrombin and MIF can serve as biomarkers reflecting the hypercoagulable and inflammatory states in children with IgAV.

Asunto(s)

Vasculitis por IgA , Proteómica , Trombina , Humanos , Niño , Masculino , Proteómica/métodos , Femenino , Vasculitis por IgA/orina , Trombina/metabolismo , Factores Inhibidores de la Migración de Macrófagos/orina , Mapas de Interacción de Proteínas , Preescolar , Oxidorreductasas Intramoleculares

RESUMEN

Endothelial cell injury is a hallmark of IgA vasculitis (IgAV), possibly associated with various factors, including oxidative stress. Certain single nucleotide polymorphisms (SNPs) of glutathione S-transferases (GST) genes have been shown to increase susceptibility to oxidative stress. The objective of our study was to evaluate the gene polymorphisms of GSTM1, GSTT1, GSTP1, and GSTA1 in patients with IgAV. DNA was extracted from the blood of 124 children with IgAV and 168 age-matched healthy controls. A higher frequency of the GSTM1 null genotype was observed in patients with gastrointestinal (GI) system involvement compared to those without GI system involvement (51.5% vs. 28.6%, p = 0.011). Additionally, the GSTM1 null genotype was less prevalent (30.8% vs. 69.2%, p = 0.032), while the GSTP1 Val/Val genotype was significantly more prevalent in patients who developed urogenital complications (scrotal swelling) during the course of the disease (60% vs. 40%, p = 0.039). This study is the first to suggest an association between GSTM1 and GSTP1 polymorphisms and various phenotypes observed during the clinical course of IgAV in the pediatric population. However, it was performed on a national and likely single ethnic cohort, too small for definitive conclusions, so larger studies are needed to confirm this association.

Asunto(s)

Predisposición Genética a la Enfermedad , Gutatión-S-Transferasa pi , Glutatión Transferasa , Vasculitis por IgA , Polimorfismo de Nucleótido Simple , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios de Casos y Controles , Frecuencia de los Genes , Estudios de Asociación Genética , Genotipo , Gutatión-S-Transferasa pi/genética , Glutatión Transferasa/genética , Vasculitis por IgA/genética , Inmunoglobulina A/sangre , Vasculitis/genética

RESUMEN

BACKGROUND: Immunoglobulin A vasculitis, historically known as Henoch-Schönlein purpura, is a rare form of autoimmune-induced vasculitis most common in children. This disease is characterized by a purpuric rash, arthritis, digestive tract complication, and renal inflammation (Hopkins). CASE REPORT: We present the case of a 78-year-old man in the emergency department with findings of weakness, abdominal pain, and bloody diarrhea for 3 days and a new-onset bilateral lower extremity rash. Diagnostic imaging and labs diagnosed this patient with immunoglobulin A vasculitis (IgAV) with associated acute kidney injury and abdominal mesenteric edema. Why Should an Emergency Physician be Aware of This? Recognition of IgAV by emergency physicians and assessment of multiple organ involvement is critical to expedite treatment and minimize complications. Particularly, physicians should consider and recognize the increased severity and different presentation of IgAV in adults in comparison with the more widely known manifestation in children.

Asunto(s)

Dolor Abdominal , Vasculitis por IgA , Humanos , Masculino , Anciano , Dolor Abdominal/etiología , Vasculitis por IgA/complicaciones , Vasculitis por IgA/diagnóstico , Vasculitis por IgA/fisiopatología , Lesión Renal Aguda/etiología , Servicio de Urgencia en Hospital/organización & administración , Edema/etiología

RESUMEN

INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare disease characterized by the inflammation and destruction of small blood vessels and circulating ANCAs. Drugs such as antithyroid drugs (ATDs), especially propylthiouracil (PTU), have been used for the production of ANCAs and cause the development of drug-induced AAV. The pathogenesis of this disease is unclear but could be related to the physiological processes affecting the degradation of neutrophil extracellular traps (NETs). At present, PTU is widely used in patients with Graves' disease (GD) who are preparing for pregnancy and whose condition has not been controlled. Once drug-induced AAV has occurred with important organ damage, considering NETs have a significant role in the immune system, whether the cessation of drugs could stop the progression of organ damage is unclear, and a consensus regarding standard treatment has not been established. PATIENT CONCERNS: In this case report, a female patient who planned pregnancy was hospitalized with multiple joint pain, impaired renal function, and hematuria. Immunofluorescence of the renal biopsy demonstrated spherical and diffuse mesangial distribution of IgA (3+). Autoimmune serology demonstrated positivity for autoantibodies against p-ANCA and an anti-MPO titer 74.72 RU/mL. DIAGNOSIS: She was diagnosed with PTU-induced p-ANCA-associated and IgA-associated vasculitis (IgAV). INTERVENTIONS: The patient accepted low doses of glucocorticoid, immunosuppressive therapy and RAI treatment. OUTCOMES: Both her kidney function and thyroid function remained were on the mend. CONCLUSION: The authors believe that this type of patient needs to fully consider their pregnancy preparation needs, suspend pregnancy when a small chance of GD remission is indicated, and avoid the use of drugs with reproductive toxicity and other serious adverse events. The multidisciplinary combination therapy of low-dose glucocorticoids and immunosuppressants combined with iodine radiotherapy is one reasonable scheme. At the same time, it is necessary to eliminate the organ damage caused by other reasons. This report provides a clinical treatment basis for patients with drug-induced vasculitis manifestations who cannot receive an accurate diagnosis.

Asunto(s)

Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Antitiroideos , Propiltiouracilo , Humanos , Propiltiouracilo/efectos adversos , Femenino , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inducido químicamente , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Antitiroideos/efectos adversos , Antitiroideos/uso terapéutico , Embarazo , Adulto , Enfermedad de Graves/tratamiento farmacológico , Vasculitis por IgA/inducido químicamente , Vasculitis por IgA/diagnóstico , Vasculitis por IgA/inmunología , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Anticuerpos Anticitoplasma de Neutrófilos/inmunología

Asunto(s)

Glomerulonefritis por IGA , Enfermedad de Graves , Vasculitis por IgA , Linfoma de Células del Manto , Humanos , Vasculitis por IgA/complicaciones , Vasculitis por IgA/diagnóstico , Linfoma de Células del Manto/complicaciones , Linfoma de Células del Manto/patología , Enfermedad de Graves/complicaciones , Glomerulonefritis por IGA/complicaciones , Glomerulonefritis por IGA/patología , Masculino , Persona de Mediana Edad

RESUMEN

OBJECTIVE: The objective of this meta-analysis was to compare the efficacy and safety between glucocorticoids combined with mycophenolate mofetil (MMF) versus glucocorticoids combined with cyclophosphamide (CTX) for henoch schonlein purpura nephritis (HSPN) in children. METHODS: Databases including PubMed, EMbase, the Cochrane Library, China National Knowledge Infrastructure, and Wanfang database were searched from the inception to April 5th, 2024. Eligible studies comparing glucocorticoids combined with MMF versus glucocorticoids combined with CTX for HSPN in children were included. Data were analyzed using Review Manager Version 5.3. RESULTS: Ten studies were included in the meta-analysis. Six randomized controlled trials (RCTs) and 4 non-randomized studies involving 675 patients were identified. Compared with CTX therapeutic schedule, MMF therapeutic schedule had a higher complete remission (CR) within the 6 months (OR 1.61, 95%CI 1.16-2.22, P = .004) and CR within the 12 months (OR 1.73, 95%CI 1.00-2.97, P = .05). However, there was no significant difference between MMF and CTX therapeutic schedule concerning total remission (TR) within the 6 months (OR 1.54, 95%CI 0.82-2.92, P = .18) and TR within the 12 months (OR 2.08, 95%CI 0.86-5.01, P = .10). In addition, incidences of gastrointestinal discomfort (OR 0.33, 95%CI 0.19-0.56, P < .0001), liver function injury (OR 0.28, 95%CI 0.09-0.87, P = .03), myelosuppression (OR 0.15, 95%CI 0.06-0.41, P = .0001), alopecia (OR 0.25, 95%CI 0.07-0.91, P = .03) in MMF therapeutic schedule were all lower than CTX therapeutic schedule. There was no statistically significant difference between the 2 therapeutic schedules concerning infection (OR 0.90, 95%CI 0.50-1.61, P = .72), rash (OR 0.38, 95%CI 0.07-2.04, P = .26). CONCLUSION: Glucocorticoids combined with MMF had a higher CR and lower incidence of adverse effects compared with glucocorticoids combined with CTX in the treatment of HSPN in children.

Asunto(s)

Ciclofosfamida , Quimioterapia Combinada , Vasculitis por IgA , Inmunosupresores , Ácido Micofenólico , Nefritis , Humanos , Vasculitis por IgA/tratamiento farmacológico , Vasculitis por IgA/complicaciones , Ciclofosfamida/uso terapéutico , Ciclofosfamida/efectos adversos , Ácido Micofenólico/uso terapéutico , Ácido Micofenólico/efectos adversos , Niño , Inmunosupresores/uso terapéutico , Inmunosupresores/efectos adversos , Inmunosupresores/administración & dosificación , Nefritis/tratamiento farmacológico , Glucocorticoides/uso terapéutico , Glucocorticoides/administración & dosificación , Glucocorticoides/efectos adversos , Resultado del Tratamiento , Ensayos Clínicos Controlados Aleatorios como Asunto , Preescolar

RESUMEN

BACKGROUND: There have been some shifts in the frequency and distribution of biopsy-proven renal diseases in China over recent years. The aim of the study was to investigate the changing spectrum of renal diseases from the view of kidney biopsy data in a single center of China. METHODS AND RESULTS: A total of 10,996 cases of native renal biopsies from patients aged ≥15 years old in Huashan Hospital, Fudan University, between 2008 and 2018 were analyzed retrospectively. The results showed that primary glomerular nephropathy (PGN) remained the most common biopsy-proven renal disease (69.42% of total), with IgA nephropathy (IgAN) accounting for 44.40% of PGN, membranous nephropathy (MN) for 28.55%, minimal change disease (MCD) for 13.26% and focal segmental glomerulosclerosis (FSGS) for 8.00%. During the study period, the proportion of MN in PGN appeared an increasing tendency, while that of IgAN and MCD remained stable and that of FSGS showed a decline. Secondary glomerular nephropathy (SGN) constituted 21.54% of total cases, among which the leading two diseases were lupus nephritis (LN) and Henoch-Schonlein purpura nephritis (HSN) which accounted for 41.08% and 19.11% respectively. CONCLUSIONS: The 11-year retrospective study revealed that PGN was the predominant histologic diagnosis among patients undergoing renal biopsy and the most frequent type of PGN remained to be IgAN, followed by MN which increased dramatically.

Asunto(s)

Glomerulonefritis por IGA , Glomerulonefritis Membranosa , Glomeruloesclerosis Focal y Segmentaria , Riñón , Nefrosis Lipoidea , Humanos , China/epidemiología , Masculino , Estudios Retrospectivos , Adulto , Femenino , Persona de Mediana Edad , Biopsia/estadística & datos numéricos , Glomerulonefritis Membranosa/patología , Glomerulonefritis Membranosa/epidemiología , Glomerulonefritis por IGA/patología , Glomerulonefritis por IGA/epidemiología , Glomeruloesclerosis Focal y Segmentaria/patología , Glomeruloesclerosis Focal y Segmentaria/epidemiología , Adulto Joven , Nefrosis Lipoidea/patología , Nefrosis Lipoidea/epidemiología , Riñón/patología , Adolescente , Nefritis Lúpica/patología , Nefritis Lúpica/epidemiología , Anciano , Vasculitis por IgA/patología , Vasculitis por IgA/epidemiología , Vasculitis por IgA/diagnóstico , Glomerulonefritis/patología , Glomerulonefritis/epidemiología , Enfermedades Renales/patología , Enfermedades Renales/epidemiología , Enfermedades Renales/diagnóstico

RESUMEN

Henoch-Schönlein purpura (HSP) is an immunoglobulin A (IgA)-mediated systemic vasculitis, which is one of the rare adverse reactions to hepatitis B vaccination. Low vitamin D levels were found to be present in the majority of HSP patients.A 19-year-old woman was admitted with a purpuric rash on bilateral lower limbs and joint pain on her left index finger in January 2020. A previous history of rash occurred one week after the patient received her first dose of recombinant hepatitis-B vaccination. Routine hematological examination, creatinine, urinalysis, C3, and C4 showed normal results. HBsAg, Anti-HCV, and ANA tests were negative, and anti-HBs were elevated. Vitamin D is very low. The patient was diagnosed with HSP and given mycophenolate mofetil, methylprednisolone, vitamin D3, and folic acid. Within 1 month of therapy, the rash still occurred frequently, so mycophenolate mofetil was changed to mycophenolic acid, the dose of methylprednisolone was increased and fexofenadine was administered. In the next 3 months, the rash has improved. However, patients reported knee joint pain and hair loss. In May 2021, the patient underwent tonsillectomy due to acute exacerbation of chronic tonsillitis. Thereafter, the patient reported that the rash had completely resolved and never worsened, and the vitamin D assay was normal.Hepatitis B vaccination is one of the etiologies of HSP, although it is rare, so it is important to ask about the vaccination history in patients with suspected HSP. Correction of vitamin D and performing tonsillectomy provide better treatment results in HSP cases in this patient.

Asunto(s)

Colecalciferol , Vacunas contra Hepatitis B , Vasculitis por IgA , Tonsilectomía , Humanos , Femenino , Vasculitis por IgA/inducido químicamente , Colecalciferol/administración & dosificación , Colecalciferol/efectos adversos , Vacunas contra Hepatitis B/efectos adversos , Vacunas contra Hepatitis B/administración & dosificación , Adulto Joven , Tonsilectomía/efectos adversos

RESUMEN

BACKGROUND: Immumoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP), is a self-limiting systemic vasculitis in children. Kidney involvement is associated with a long-term unfavorable outcome and can lead to significant morbidity. This study was conducted to describe the clinical and laboratory characteristics of childhood IgAV with kidney involvement and to identify risk factors associated with IgAV nephritis (IgAVN). METHODS: This was an ambidirectional descriptive study of 77 children with IgAV. All demographic data, clinical features, and laboratory tests were collected from electronic medical records from January 2010 to December 2022. Risk factors for kidney involvement in IgAV were assessed using multivariate logistic regression. Kaplan-Meier survival analysis was used to calculate the time to commencement of kidney involvement. RESULTS: Twenty-five children (32.4% of the IgAV patients) developed IgAVN. The common findings in IgAV with kidney involvement were microscopic hematuria (100%), nephrotic range proteinuria (44%), and non-nephrotic range proteinuria (40%). Multivariate logistic regression showed that age greater than 10 years (adjusted hazard ratio, AHR 4.66; 95% confidence interval, CI, 1.91-11.41; p = 0.001), obesity (body mass index, BMI, z-score ≥ +2 standard deviations, SDs) (AHR 3.59; 95% CI 1.41-9.17; p = 0.007), and hypertension at onset (AHR 4.78; 95% CI 1.76-12.95; p = 0.002) were associated significantly with kidney involvement. During follow up, most IgAV patients developed nephritis within the first 9 months. CONCLUSION: Age greater than 10 years, obesity, and hypertension at presentation were predictive factors for IgAVN. Our study emphasized that IgAV patients with risk factors should be closely monitored for at least 1 year after the onset of the disease.

Asunto(s)

Vasculitis por IgA , Humanos , Masculino , Femenino , Niño , Factores de Riesgo , Vasculitis por IgA/complicaciones , Vasculitis por IgA/epidemiología , Vasculitis por IgA/diagnóstico , Preescolar , Adolescente , Estudios Retrospectivos , Proteinuria/etiología , Proteinuria/epidemiología , Estimación de Kaplan-Meier , Hematuria/etiología , Hematuria/epidemiología , Modelos Logísticos , Riñón/patología , Glomerulonefritis por IGA/complicaciones , Glomerulonefritis por IGA/epidemiología

RESUMEN

BACKGROUND: Children with IgA Vasculitis (IgAV) may develop renal complications, which can impact their long-term prognosis. This study aimed to build a machine learning model to predict renal damage in children with IgAV and analyze risk factors for IgA Vasculitis with Nephritis (IgAVN). METHODS: 50 clinical indicators were collected from 217 inpatients at our hospital. Six machine learning algorithms-Logistic Regression, Linear Discriminant Analysis, K-Nearest Neighbor, Support Vector Machine, Decision Trees, and Random Forest-were utilized to select the model with the highest predictive performance. A simplified model was developed through feature importance ranking and validated by an additional cohort with 46 patients. RESULTS: The random forest model had the highest accuracy, precision, recall, F1 score, and area under the curve, with values of 0.91, 0.98, 0.70, 0.79 and 0.94, respectively. The top 11 features according to the importance ranking were anti-streptolysin O, corticosteroids therapy, antihistamine therapy, absolute eosinophil count, immunoglobulin E, anticoagulant therapy, C-reactive protein, prothrombin time, age at onset, D-dimer, recurrence of rash ≥ 3 times. A simplified model using these features demonstrated optimal performance with an accuracy of 84.2%, a sensitivity of 89.4%, and a specificity of 82.5% in external validation. Finally, we provided a web tool based on the simplified model, whose code was published on https://github.com/mulanruo/IgAVN_Prediction . CONCLUSION: The model based on the random forest algorithm demonstrates good performance in predicting renal damage in children with IgAV, providing a basis for early clinical diagnosis and decision-making.

Asunto(s)

Vasculitis por IgA , Aprendizaje Automático , Humanos , Masculino , Femenino , Niño , Vasculitis por IgA/diagnóstico , Vasculitis por IgA/sangre , Vasculitis por IgA/complicaciones , Vasculitis por IgA/inmunología , Pronóstico , Adolescente , Estudios Retrospectivos , Factores de Riesgo , Riñón/patología , Riñón/inmunología , Preescolar

RESUMEN

Palpable purpura, gastrointestinal symptoms, joint involvement, and renal disease characterize immunoglobulin A vasculitis (IgAV). Renal involvement ranging from mild proteinuria to severe nephritic or nephrotic syndrome highlights the importance of monitoring kidney function in patients with IgAV. Recognizing these key features is crucial for early diagnosis and appropriate management to prevent long-term complications related to kidney disease. However, the pathogenesis of IgAV remains unclear. Disease mechanisms involve various factors, including the interplay of aberrantly glycosylated IgA, anti-endothelial cell antibodies, and neutrophils following infection triggers, which are the main pathogenic mechanisms of IgAV. Insights from cases of IgAV related to Coronavirus disease 2019 have offered additional understanding of the connection between infection and IgAV pathogenesis. This review provides a valuable resource for healthcare professionals and rheumatology researchers seeking a better understanding of the clinical features and pathophysiology of IgAV.

Asunto(s)

COVID-19 , Inmunoglobulina A , Humanos , Inmunoglobulina A/inmunología , COVID-19/inmunología , COVID-19/fisiopatología , COVID-19/virología , COVID-19/complicaciones , Vasculitis/inmunología , Vasculitis/fisiopatología , SARS-CoV-2/inmunología , Vasculitis por IgA/inmunología , Vasculitis por IgA/fisiopatología , Vasculitis por IgA/diagnóstico , Autoanticuerpos/inmunología , Neutrófilos/inmunología

RESUMEN

IgA nephropathy (IgAN) is a fairly common association with alcoholic liver disease. However, IgA vasculitis (IgAV) is quite an uncommon association with alcoholic liver cirrhosis and only a handful of cases have been reported in literature. Secondary IgAN usually presents in a docile manner, progressing slowly in about 5-25 years. It is usually responsive to steroid therapy, very rarely progressing to End-Stage Renal Disease. Here, we present a man in his late 50s, a known hypertensive and alcohol related liver-cirrhotic, who presented to our hospital with rash and rapidly progressive renal failure (RPRF). He was diagnosed with IgA nephritis with IgA vasculitis (IgAVN). His diagnosis was confirmed with skin and renal biopsy. He was started on renal replacement therapy for his renal failure and began oral steroid therapy. After administration of steroid therapy for 6 months, the patient recovered and was dialysis independent with stable renal parameters.

Asunto(s)

Glomerulonefritis por IGA , Humanos , Masculino , Glomerulonefritis por IGA/complicaciones , Glomerulonefritis por IGA/diagnóstico , Persona de Mediana Edad , Progresión de la Enfermedad , Hepatopatías Alcohólicas/complicaciones , Vasculitis por IgA/complicaciones , Vasculitis por IgA/diagnóstico , Vasculitis por IgA/tratamiento farmacológico , Vasculitis/complicaciones , Vasculitis/etiología , Vasculitis/diagnóstico , Vasculitis/tratamiento farmacológico