[Berger's disease in childhood]

Abstract

Berger's disease or IgA nephropathy (NIgA) is the most common form of glomerulonephritis in the world. In children macroscopic haematuria is the first sign in about 80% of the patients. Renal failure appears in 20% of cases after twenty years of follow-up. The most important prognosis indicators are a nephrotic syndrome at the onset, a proteinuria > 1 g/24 hours, diffuse tubulo-interstitial lesions and extracapillary proliferation with crescents in more than 50% of the glomeruli. The pathogenic mechanisms are just emerging and involve a disrupted process of the systemic tolerance to mucosal antigen with abnormal mucosal gamma delta T cell repertoire, abnormally glycosylated IgA1 molecules and a down-regulation of Fc alpha receptors on blood cells. After IgA deposition, the mechanisms of mesangial cell damage and activation involve vascular factors as endothelin/nitric oxide system, cytokines and growth factors such as interleukine-6, platelet derived growth factor and transforming growth factor beta. There is no curative treatment but steroids are useful in diffuse proliferative extracapillary forms, when histological activity score is high with a short delay between diagnosis and treatment, or for moderately severe NIgA with normal renal function.