IgA vasculitis (IgAV) is the most prevalent form of systemic vasculitis in kids and commonly includes the kidney. A minority of individuals with IgA vasculitis nephritis (IgAVN), notably those presenting with high proteinuria and/or renal failure at the beginning, are at risk of chronic end-stage kidney disease. For selecting upon treatment intensity, knowledge of the short-term clinical course of IgAVN is needed to develop therapy algorithms. The medical records of 66 children with biopsy-proven IgAVN were analyzed for this retrospective multicenter investigation. Age, gender, medical history, and therapeutic interventions were documented. Laboratory data included serum creatinine, albumin, urinary protein excretion (UPE), and glomerular filtration rate (eGFR). Threshold values were calculated for each measure, and full remission was defined as no proteinuria and eGFR more than 90 ml/min/1.73m2. The median age at the onset of IgAVN was 8.9 years. About 14.1% of the children presented with nephrotic syndrome, 50% had an eGFR less than 90 ml/min/1.73 m2, and 51.5% had cellular crescents in renal histology. The therapy regimens differed substantially. Around 44 patients were treated with immunosuppression; 17 patients with crescents or nephrotic syndrome were treated with corticosteroid (CS) pulse treatment. After 6 months, UPE had dropped from 3.7 to 0.3 g/g creatinine, and the proportion of patients with a lowered eGFR had fallen from 50.0% to 35.5%. About 13 children (26.5%) got full remission within 6 months. In most individuals with IgAVN proteinuria diminishes slowly, and kidney function improves, but full remission is attained only in the minority after 6 months. Heavy proteinuria that persisted for more than 2 months seldom led to chronic proteinuria. Therefore, the progression of UPE over time should be considered while deciding on a more intensive treatment plan. The number of patients was too low to compare treatments’ efficacies reliably. Clarifying risk factors and the impact of immunosuppressive treatments requires prospective, randomized controlled trials.

Source: bmcpediatr.biomedcentral.com/articles/10.1186/s12887-022-03611-9