[IgA nephropathy. A frequent disease in the Mediterranean area]

Primary IgA nephropathy (IgAN) is a worldwide disease characterized by the recurrence or macroscopic hematuria episodes followed by continuous or intermittent microscopic hematuria. This disease is more frequent in men than in women and the prevalence is higher in the Asian and European areas than Australia and North-America. However the prevalence of IgA is strictly correlated with the biopsy policy and health screening urinary tests. The renal biopsy implicates the diagnostic and prognostic aspects since various histological degrees of renal lesions show different behaviour of the renal function. Even though the disease is progressive in a minority of patients various investigators have attempted treatment with various drugs. Corticosteroids seem to have effect on the course of IgAN in those patients with heavy proteinuria or nephrotic syndrome and normal renal function. Since Berger's original report the immunological mechanisms implicated in the pathogenesis of IgAN have been extensively described. Increased serum levels of IgA, mainly in the polymeric form, and the occurrence of circulating IgA immune complexes which localize in the glomeruli may be explained by an abnormal regulation of the IgA system. The disregulation of IgM-IgA switch may be implicated in this IgA disorder. Recent data have evidenced similar abnormalities in family members.