Immunoglobulin A nephropathy (IgAN) is the most common form of primary glomerulonephritis in the world today. It was initially considered to be a benign disease with a favorable prognosis, but according to more recent long‐term follow‐up data, the disease progresses to renal failure in 10–15% of pediatric patients. It was also recently noted that the pathogenesis of IgAN may be associated with the circulation of defective forms of IgA1 or the presence of in situ immune complexes, and the onset and progression of inflammation are thought to be associated with complement components, activated macrophages and mesangial cells. With regard to the prognosis of IgAN, clinical predictors of poor outcome include renal insufficiency, heavier degrees of proteinuria during the follow‐up period, and hypertension, while pathologic features of poor prognosis include glomerular sclerosis, interstitial fibrosis and tubular atrophy. There are few useful laboratory markers, however, for predicting treatment response and outcome of IgAN at diagnosis or prior to treatment.With regard to serum IgA level, serum IgA and galactose‐deficient (GD) ‐IgA1 in adult IgAN are higher than in non‐IgAN. Ishiguro et al. reported that the serum IgA/complement factor 3 (IgA/C3) ratio in adult IgAN was higher than in other glomerular disease, and that the
Pediatrics International – Wiley
Published: Jan 1, 2018
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