Clinical Characteristics of Concomitant Nephrotic IgA Nephropathy and Minimal Change Disease in Children

Abstract

Background: IgA nephropathy (IgAN) is the most common primary glomerulonephritis in children and adolescents worldwide. Meanwhile, minimal change disease (MCD) is the most common cause of nephrotic syndrome in children. Recently, several case reports have revealed that IgAN can be complicated by MCD in adults. Here, we report our experience concerning the features of such patients in pediatrics. Methods: The clinical manifestations and pathological features of 197 children who presented with IgAN from December 2007 to November 2013 were analyzed retrospectively based on the criteria for nephrotic syndrome and MCD. Results: Among the 197 children diagnosed with primary IgAN, 25 (12.7%) patients presented with nephrotic syndrome, and 7 patients (2.8%) presented with MCD-like pathological features and nephrotic syndrome simultaneously. The cohort of 7 patients included 5 boys, and the median age was 8.9. All of the patients who were diagnosed with primary nephrotic syndrome were treated initially with corticosteroids. Except for 1 patient with steroid resistance who was lost to follow-up, the other 6 cases presented were steroid sensitive and remained in complete remission for the last follow-up, with median and mean follow-up durations of 30.5 and 34.5 months, respectively (range 10-65 months). Conclusions: This study revealed that IgAN and MCD may also coexist in children. Moreover, most of these patients who presented with nephrotic syndrome responded well to steroids and had a favorable prognosis. Large-scale studies are required, and careful attention should be paid to such complicated cases.

Characterization of patients with IgA nephropathy with and without associated minimal change disease

“…However, some studies have reported that patients with MCD-IgAN respond well and safely to corticosteroid therapy (24,25), MCD-IgAN experience a higher recurrence rate than non-MCD-IgAN. (25) IgAN and MCD may also coexist in children, and most of these patients who presented with nephrotic syndrome responded well to corticosteroids and had a satisfying prognosis (26). The pathophysiology of IgAN is considered to be a multi-hit mechanism (9).…”