Rapidly Progressive IgA Nephropathy in One of a Pair of Identical Twins

Abstract

Introduction: IgA nephropathy (IgAN) is the most common cause of primary glomerulonephritis worldwide. It was considered a benign condition for many years but long term follow up showed that it might progress to end stage renal disease (ESRD). The cause of primary IgAN is unknown and no consistent genetic abnormalities that predict the development or progression of IgAN have been identified. A variety of observations suggest an as-sociation to an unknown environmental antigen, familial clustering, or infectious agent. The patient presented here is the first report of a child with IgAN in Sudan. Case report: a seven years old boy was referred to our center for further evaluation and management of sudden
onset macroscopic hematuria and renal impairment. He was born after an uneventful pregnancy and breast fed. He developed normally and was healthy before this illness. He had eight siblings who were all healthy, including his identical twin brother. Physical examination and laboratory tests led to the diagnosis of IgAN. The patient was treated with peritoneal dialysis and pulses of methylprednisolone for three consecutive days. He showed a remarkable response and regained normal
renal function. He was then kept on alternate day’s steroids, ACE inhibitors, and Azathioprine. The other twin is being closely monitored.
Conclusion: This report provides an indirect support for the hypothesis that environmental factors play a role in the pathogenesis of IgAN. However, follow up of the currently healthy twin is necessary, since affection with the disease may be expressed at a later time.

Key words: IgA nephropathy, identical twins, macroscopic
hematuria