IgA vasculitis nephritis clinical course and kidney biopsy \u2013 national study in children

Małgorzata Mizerska-Wasiak,Jadwiga Małdyk,Karolina Cichoń-Kawa,Monika Miklaszewska,Agnieszka Turczyn,Małgorzata Pańczyk-Tomaszewska,Maria Szczepańska,Beata Bieniaś,Magdalena Drożyńska-Duklas,Dorota Drożdż,Aleksandra Żurowska,Przemysław Sikora

doi:10.1186/s12969-021-00616-z

Abstract

The aim of the study was to investigate the relationship between the severity of typical clinical symptoms, severity of histopathological lesions in kidney biopsies in IgA vasculitis nephritis (IgAVN) and to propose indications for kidney biopsy in children.Material and methodsThis retrospective study enrolled 106 patients, included in the IgAVN registry of Polish children, diagnosed by kidney biopsy. Renal and extrarenal symptoms at onset of the disease were analyzed. Biopsy results were assessed using Oxford classifications (MEST-C). The patients were divided into 3 groups depending on the severity of proteinuria: A-nephrotic proteinuria with hematuria; B-non-nephrotic proteinuria with hematuria; C-isolated hematuria.ResultsThe first symptoms of nephropathy were observed at the 0.7 (1–128.4) months from the onset of extrarenal symptoms. Kidney biopsy was performed on 39 (6–782) days after the onset of nephropathy symptoms. MEST-C score 4 or 5 was significantly more frequent in children from group A than in groups B and C. Significantly higher mean MEST-C score was found in patients with abdominal symptoms than without.In group A: S0 and T0 we found in significantly shorter time to kidney biopsy than in S1, T1–2 p < 0.05) and in group B the significantly shorter time in T0 compare to T1–2 p < 0.05). The ROC analysis shows that S1 changes appear in kidney biopsies in group A with cut off 21 days (AUC 0,702, p = 0.004, sensitivity 0.895 specificity 0.444) T1–2 changes after 35 days (AUC 0.685, p = 0.022, sensitivity 0.750, specificity 0.615), and in goupn B T1–2 cut off is 74 days (AUC 0,738, p = 0.002, sensitivity 0.667, specificity 0.833).ConclusionsIn childhood IgAVN, the severity of changes in the urine is clearly reflected in the result of a kidney biopsy. The biopsy should be performed in patients with nephrotic proteinuria no later than 3 weeks after the onset of this symptom in order to promptly apply appropriate treatment and prevent disease progression. Accompanying abdominal symptoms predispose to higher MESTC score.

Highlights

IgA vasculitis (IgAV, Schönlein-Henoch purpura, HSP) is an autoimmune vasculitis characterized by the formation of IgA-dominant immune deposits in blood vessels wall [1, 2]
In group A: S0 and T0 we found in significantly shorter time to kidney biopsy than in S1, T1–2 p < 0.05) and in group B the significantly shorter time in T0 compare to T1–2 p < 0.05)
In childhood IgA vasculitis nephritis (IgAVN), the severity of changes in the urine is clearly reflected in the result of a kidney biopsy

Summary

Introduction

IgA vasculitis (IgAV, Schönlein-Henoch purpura, HSP) is an autoimmune vasculitis characterized by the formation of IgA-dominant immune deposits in blood vessels wall [1, 2]. It occurs in both previously healthy children (90%) and, much less frequently, in adults [3]. In children, it is the most common form of vasculitis and in most cases the course is self-limiting [1, 3,4,5]. The incidence in children is 10–30/100000 cases per year and is the highest in autumn and winter. According to Mizerska-Wasiak et al Pediatric Rheumatology (2021) 19:150 most studies, the disease is more frequently observed in boys [1,2,3, 6,7,8]

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