Early clinical course of biopsy-proven IgA vasculitis nephritis

Abstract

Background IgA vasculitis (IgAV) is the most common form of systemic vasculitis in childhood and frequently involves the kidney. A minority of patients with IgA vasculitis nephritis (IgAVN), especially those presenting with heavy proteinuria and/or kidney failure at onset, are at risk of chronic end-stage kidney disease. For deciding upon treatment intensity, knowledge of the short-term clinical course of IgAVN is needed to improve treatment algorithms. Methods For this retrospective multicenter study, the medical records of 66 children with biopsy-proven IgAVN were reviewed. Age, gender, medical history and therapeutic interventions were recorded. Laboratory data included serum creatinine, albumin, urinary protein excretion (UPE) and glomerular filtration rate (eGFR). Threshold values were determined for each parameter, full remission was defined as no proteinuria and eGFR > 90 ml/min/1.73m2. Results Median age at onset of IgAVN was 8.9 years. 14.1% of the children presented with nephrotic syndrome, 50% had an eGFR below 90 ml/min/1.73 m2 and 51.5% showed cellular crescents in renal histology. The treatment regimens varied notably. Forty-four patients were treated with immunosuppression; 17 patients with crescents or nephrotic syndrome were treated with corticosteroid (CS) pulse therapy. After 6 months, UPE had decreased from 3.7 to 0.3 g/g creatinine and the proportion of patients with a decreased eGFR had fallen from 50.0% to 35.5%. Thirteen children (26.5%) achieved full remission within 6 months. Conclusions In most patients with IgAVN proteinuria decreases slowly and kidney function improves, but full remission is reached only in a minority after 6 months. Persistent heavy proteinuria in the first two months rarely developed into long-term proteinuria. Therefore, decisions for more intense treatment should take into account the course of UPE over time. For a comparison of treatment effects, patient numbers were too small. Prospective, randomized controlled trials are necessary to clarify risk factors and the effect of immunosuppressive therapies.

Comparison of clinical characteristics and complication predictors of IgA vasculitis between pediatric and adult patients in China

“…A retrospective study of arthralgia in adults with IgAV [29] reported that joint involvement occurred in 62% IgAV patients, with knee and ankle involvement being the most common, and arthralgia occurred in younger patients, which is consistent with our nding that GI involvement was signi cantly associated with joint manifestations. Other retrospective studies [9,30] reported that 37-77.7% IgAV patients developed arthralgia in childhood.…”

Immunoglobulin A vasculitis nephritis: Current understanding of pathogenesis and treatment

“…In this regard, the German Society of Pediatric Nephrology has recently suggested an early treatment approach for patients with important kidney involvement. Following this treatment suggestion, IgAVN patients with nephritic syndrome, nephrotic syndrome, or glomerular cellular growths will receive an initial standardized corticosteroid- based treatment regimen for 2 mo, followed by an additional immunosuppression in patients with inadequate response after 3-6 mo from the start of treatment[ 58 ].…”