Abstract
Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerulopathies diagnosed in children and adolescents. This study aimed to evaluate the clinical features in and outcomes of pediatric IgAN over the last 30 years. Patients who were diagnosed before age of 18 at 20 centers in Korea were evaluated retrospectively. Of the 1154 patients (768 males, 386 females) with a median follow-up of 5 years, 5.6% (n = 65) progressed to stage 3–5 chronic kidney disease (CKD). The 10- and 20-year CKD-free survival rates were 91.2% and 75.6%, respectively. Outcomes did not differ when comparing those in Korea who were diagnosed prior to versus after the year 2000. On multivariate analysis, combined asymptomatic hematuria and proteinuria as presenting symptoms and decreased renal function at the time of biopsy were associated with progression to CKD, while remission of proteinuria was negatively associated with this outcome. Patients who presented with gross hematuria or nephrotic syndrome tended toward positive outcomes, especially if they ultimately achieved remission. While remission of proteinuria might imply that the disease is inherently less aggressive, it also can be achieved by management. Therefore, more aggressive management might be required for pediatric-onset IgAN.
Highlights
Immunoglobulin A nephropathy (IgAN) is among the most common of the primary glomerulopathies in children and adolescents worldwide, especially in Asian countries [1]
Initial presenting symptoms included gross hematuria (GHU) (583 patients, or 50.5%), nephrotic syndrome (NS) (89 patients, or 7.8%), and asymptomatic urinary abnormalities (AUA) (482 patients, or 41.8%). Among those presented with AUA, 126 patients (10.9%) presented with isolated hematuria (IH), 57 patients (4.9%) presented with isolated proteinuria (IP), and 299 patients (25.9%) presented with combined hematuria and proteinuria (CHP)
We further evaluated the outcomes of patients who presented with CHP, NS, or GHU according to remission status and Estimated glomerular filtration rate (eGFR) at renal biopsy
Summary
Immunoglobulin A nephropathy (IgAN) is among the most common of the primary glomerulopathies in children and adolescents worldwide, especially in Asian countries [1]. In a long-term observational study (mean follow-up of 18.7 years) of pediatric-onset IgAN patients in Finland, Ronkainen et al [5] reported that 6 (11%) of 55 patients had developed ESRD at their last follow-up. In the Validation of the Oxford Classification of IgAN (VALIGA) study that included 174 children and adolescents diagnosed with IgAN from 13 European countries that were followed for a mean of 4.9 years, the survival rate given the combined endpoint of 50% decline in renal function or ESRD at 15 years was 94% [9]. In a Japanese study, Nozawa et al [11] reported that after a mean follow-up of 7.3 years from onset, 7 (3.9%) of 181 pediatric IgAN patients developed ESRD with a predicted survival rate of 92.3% at 10 years and 89.1% at 20 years. Several clinical factors have been identified as risk factors for disease progression including persistence of a high level of proteinuria, higher time-averaged blood pressure during follow-up, and reduced renal function at the time of biopsy; pathologic variables identified as risk factors for IgAN include mesangial hypercellularity, tubular atrophy and interstitial fibrosis, crescents, and segmental glomerulosclerosis [12]
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