La nefropatia idiopatica a depositi mesangiali di IgA. Discussione clinico-patologica

Translated title of the contribution: Idiopathic IgA nephropathy. Clinic pathological conference

R. Coppo, A. Amore, S. N. Emancipator, L. Gesualdo, G. Grandaliano, P. Schena, R. Coppo, G. Maschio, F. Locatelli, D. Roccatello, C. Ponticelli

Research output: Contribution to journalArticlepeer-review

Abstract

Idiopathic immunoglobulin A nephropathy (IgAN) is in Italy the glomerulonephritis most frequently found in renal tissue examined in patients with isolated microscopic hematuria and in those with hematuria associated with non-nephrotic proteinuria. In the Italian Renal Biopsy Registries, which collects data on adults and children, IgAN represents some 20% of all cases included. World wide the frequency of IgAN varies according to the different biopsy policies, which are usually broad in Japan and in France, but much more restricted in the US. Also the natural history varies according to the renal biopsy selection criteria: the renal survival at 10 years is 85% in Italy, 94% in France, 67% in the US. The most important factors associated with an unfavorable outcome are severe proteinuria and hypertension; however, great variability exists among patients. According to the current hypothesis, in IgAN there is a defective immunoregulation, leading to the synthesis of aberrantly glycosylated IgA. These abnormal IgA molecules tend to autoaggregate and have strong reactivity towards the mesangial cells, favoring the synthesis of phlogogenic and pro-sclerotic mediators. The therapy is addressed to the rare patients with a rapidly progressive disease course and to the slowly developing cases presenting high proteinuria. Interesting results with a decrease in proteinuria and in renal insufficiency progression have been reported using corticosteroids, fish oil or ACE inhibitors. Two prospective randomized trials are currently under way enrolling young patients with IgAN and proteinuria between 1 and 3.5 g/day. The first, on going in the US, employ alternate-day prednisone or fish oil for 2 years, and compares the results with a placebo group after 5 years of follow-up. The second, an European Biomed project, tests the hypothesis that ACE-I (benazepril) given for 4 years will slow down the decline in renal function in comparison to placebo. This study focuses on the genetic and immunological factors possibly modulating the clinical effect. The comparison of these and other prospective clinical trials will hopefully give more information on the still controversial issue of the treatment of this common nephritis.

Translated title of the contributionIdiopathic IgA nephropathy. Clinic pathological conference
Original languageItalian
Pages (from-to)79-93
Number of pages15
JournalGiornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
Volume16
Issue number1
Publication statusPublished - 1999

ASJC Scopus subject areas

  • Nephrology

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