Vasculite associada à IgA em criança: relato de caso / IgA vasculitis in a child: case report

A Vasculite associada à imunoglobulina A (VIgA), também conhecida como púrpura de Henoch-Schonlein, púrpura anafilactóide ou púrpura reumática é uma vasculite de pequenos vasos associada a deposição de imunocomplexos IgA, de etiologia ainda desconhecida e que acomete principalmente crianças. Em grande parte dos casos pediátricos, é uma doença autolimitada com manifestações cutâneas, articulares, gastrintestinais e renais. O diagnóstico diferencial inclui outras vasculites, como lúpus eritematoso sistêmico, meningococcemia, coagulação intravascular disseminada e síndrome hemolítica urêmica. Neste artigo abordam-se os principais aspectos da VIgA nas crianças, salientando-se a importância do diagnóstico diferencial precoce. É apresentado o caso clínico de uma paciente do sexo feminino de 5 anos com lesões purpúricas tratada numa primeira abordagem como infecção bacteriana grave. Após reavaliação médica houve alteração terapêutica com uso de glicocorticóides resultando em melhora expressiva dos sintomas. [au]

Vasculitis associated with immunoglobulin A (VIgA), also known as Henoch-Schonlein purpura, anaphylactoid purpura or rheumatic purpura is a small vessel vasculitis associated with deposition of IgA immune complexes, of unknown etiology and affecting mainly children. In most pediatric cases, it is a self-limited disease with cutaneous, joint, gastrointestinal and renal manifestations. The differential diagnosis includes other vasculitis, such as systemic lupus erythematosus, meningococcemia, disseminated intravascular coagulation and uremic hemolytic syndrome. In this article, the main aspects of HSP in children are addressed, highlighting the importance of early differential diagnosis. The clinical case of a 5-year-old female patient with purpuric lesions treated in a first approach as a severe bacterial infection is presented. After medical re-evaluation, there was a therapeutic change with the use of glucocorticoids resulting in a significant improvement of symptoms. [au]

A five-year follow-up study on the clinicopathology of 130 children with Henoch-Sch?nlein purpura nephritis / 中华肾脏病杂志

Objective:To analyze the clinicopathologic features and prognosis of children with Henoch-Sch?nlein purpura nephritis (HSPN).Methods:The clinicopathological data of children with HSPN who were followed up for more than 5 years and underwent renal biopsy in Jinling Hospital affiliated to Medical School of Nanjing University from January 2001 to June 2015 were retrospectively analyzed. The follow-up endpoint event was defined as estimated glomerular filtration rate (eGFR)<90 ml·min -1·(1.73 m 2) -1. Participants were divided into two groups according to whether the children had reached the primary endpoint event or not. Cox proportional hazards model was used to analyze the influencing factors of renal poor prognosis in children with HSPN. Kaplan-Meier survival curve method was used for survival analysis, and log-rank test was used to compare the difference of renal cumulative survival rate between segmental sclerosis/adhesion (S1) group and non-segmental sclerosis/adhesion (S0) group. Receiver operating characteristic curve (ROC curve) and area under the curve ( AUC) were used to evaluate the diagnostic value. Results:A total of 130 children with HSPN were enrolled in the study. The median onset age was 11.7(8.6, 13.3) years old, of whom 71 cases were males (54.6%). At a median follow-up time of 100.0(75.8, 119.0) months, 12 cases (9.23%) with HSPN reached the primary endpoint event. Compared with the non-endpoint event group, the endpoint event group had higher proportion of hypertension, higher levels of 24-hour urinary protein, serum cholesterol, serum uric acid, and serum creatinine, and lower levels of serum albumin (all P<0.05). There was no statistical difference in treatment between the two groups (all P>0.05). In terms of pathological features, compared with the non-endpoint event group, the endpoint event group had higher proportion of mesangial hyperplasia (M1), S1, tubular atrophy/interstitial fibrosis (T1/T2) and Glomerulus-Bowman's capsule adhesion (all P<0.05). Multivariate Cox regression model showed that S1 was significantly correlated with renal poor prognosis ( HR=7.739, 95% CI 1.422-42.114, P=0.018). As was revealed in a Kaplan-Meier plot, renal cumulative survival rate in the S1 group was significantly lower than that in the S0 group (log-rank χ2=17.069, P<0.001). The ROC curve showed S1 accurately predicted the outcome ( AUC=0.710, 95% CI 0.549-0.872) with specificity of 0.667(95% CI 0.349-0.901) and specificity of 0.754(95% CI 0.667-0.829). Conclusions:S1 is an independent risk factor affecting renal poor prognosis and has a diagnostic value.

The diagnosis and treatment analysis of abdominal involvement in 229 children with IgA vasculitis / 中国小儿急救医学

Objective:To summarize the diagnosis and treatment process of abdominal involvement in 229 children with IgA vasculitis and to provide reference for clinic treatment.Methods:A total of 229 pediatric patients, diagnosed as IgA vasculitis with abdominal involvement admitted to the Department of Pediatric Nephrology of Shengjing Hospital, China Medical University from January 1st 2018 to December 31st 2019, were retrospectively analyzed in the study and were divided into three groups according to Numerical Rating Scale to compare indexes in different degrees of abdominal pain.Results:The duration of hospitalization was related with degree of abdominal pain, as the more severe the abdominal pain was, the longer the hospitalization time was( P<0.001). The incidence of bloody stool were also proportionate to the degree of abdominal pain( P<0.001). With the aggravation of abdominal pain, the proportion of intestinal wall edema increased, as the highest proportion was severe group( P<0.001). The proportion of renal involvement in severe group was significantly higher than that in non-severe group( P<0.001). Twenty cases of intestinal wall edema with decreasing of albumin were treated by intravenous hormone therapy after albumin infusion as the results of no intestinal complications occurred.Compared with the mild and moderate groups, the white blood cells of the severe group were higher( P<0.001)and the albumin was lower( P<0.05). It was no significant difference in hemoglobin, serum amylase and serum lipase among three groups.The mean value of CRP had no difference among three groups and was higher than that of normal.Interleukin(IL)-6 in severe group was higher than that in other two groups( P<0.05), but there was no significant difference in IL-2, IL-4, IL-10, IL-17 and tumor necrosis factor.In terms of treatment, 40 cases were treated with immunoglobulin and four cases with hemoperfusion.The average duration of intravenous glucocorticoid application was related to the degree of abdominal pain among three groups.The longest duration was severe group(16.00±6.91)d and the shortest one was mild group(6.71±3.75)d. Conclusion:Pediatric patients diagnosed as IgA vasculitis with severe abdominal pain whose part of inflammatory indexes increased and albumin decreased obviously should complete imaging examinations to evaluate the extent of intestinal wall edema.If diagnosed as hypoalbuminemia and intestinal wall edema distinctly, hormone therapy should be given after albumin infusion to prevent severe complications such as intestinal perforation.For pediatric patients of IgA vasculitis with severe abdominal symptoms, on the basis of hormone therapy, immunoglobulin and hemoperfusion could be used to quickly remove abnormal immune substances to slow down the disease.

The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

A 19-year old man with IgA vasculitis after vaccination

ABSTRACT A 19-year-old patient who mistakenly received two doses of influenza vaccine 10 days before presentation, was admitted with malaise, weakness, and a purpuric non-blanching rash most prominent on the ankles followed by abdominal pain and hematochezia 72 h later. The diagnosis of influenza vaccine-related Henoch-Schonlein vasculitis was made. This complication, although rare, is the most common vasculitis related to immunization.

Analysis of clinical pathology of the IgA nephropathy and purpura nephritis in children from the perspective of IgA vasculitis / 临床儿科杂志

Objectives To analysis clinical pathology of organ speciifc IgA vasculitis (IgA nephropathy) and systemic IgA vasculitis (allergic purpura) of purpura nephritis in children. Methods Clinical and pathological data of hospitalized pediatric patients of IgA nephropathy and purpura nephritis were retrospectively analyzed from June 1993 to November 2014. Results There were 405 patients of IgA nephropathy (256 males and 149 females). The ratio of male to female was 1.7:1. The average age was 10.2±2.8 years. The nephrotic syndrome (31.6%) was the most common clinical type, followed by hematuria and proteinuria (27.9%). There were 548 patients of purpura nephritis, 329 males and 219 females. The ratio of male to female was 1.5:1. The average age was 10.2±3.1 years. The hematuria and proteinuria (61.6%) was the most common clinical type, followed by nephrotic syndrome (21.4%). None of the IgA nephropathy progressed to systemic vasculitis (allergic purpura). Conclusions The causes, onset ages and clinical manifestations of IgA nephropathy and allergic purpura may be consistent or overlap, but none of IgA nephropathy (organ speciifcity IgA vasculitis) progressed to allergic purpura (systemic IgA vasculi-tis). IgA nephropathy might have more renal immune disorder mechanisms involved in its pathogenesis.

Do immunoglobulin A vasculitis and immunoglobulin A nephropathy belong to one desease / 中华实用儿科临床杂志

The immunopathogenesis of IgA vasculitis and IgA nephropathy both demonstrate IgA deposition,and abnormal glycosylation of IgAl molecule is their major pathogenesis.Therefore,it is clinically controversial that they are actually one disease.The present text will delineate their similarities and differences from aspects of epidemiology,clinic,pathology,mechanism and prognosis.