Spectrum of clinico-pathological profile and treatment response in children with nephrotic immunoglobulin a nephropathy.

Immunoglobulin A nephropathy (IgAN) is one of the most common types of primary glomerulonephritis in the world. Nephrotic syndrome is an uncommon presentation of IgAN. To evaluate the clinico-pathological features and treatment response of nephrotic IgAN, we prospectively studied 20 nephrotic children with biopsy-proven IgAN at our center from August 2009 to December 2012. The histopathological characterization of IgAN was carried out with the HAAS classification. The demographic profile, clinical presentation, initial laboratory, biopsy findings and treatment response were analyzed. The mean age was 6.7 years. The most common indication of renal biopsy was steroid-dependent nephrotic syndrome associated with microscopic hematuria (65%) and hypertension (25%). The majority of cases were classified as HAAS-III stage. Fifteen cases responded to oral cyclosporine-A, four cases to oral cyclophosphamide and one to mycophenolate mofetil. Complete remission of the nephrotic syndrome was achieved in 90% (18/20) cases within 3 months of initiation of therapy. Two cases that had partial remission were in the HASS-II and III stages. We conclude that the majority of children with nephrotic IgAN responded to oral cyclosporine-A. However, a larger cohort and longer duration follow-up are required to confirm our results.