Natural history of idiopathic IgA nephropathy and factors predictive of disease outcome

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Abstract

Among the numerous studies published in the last 20 years that have calculated the actuarial renal survival and tried to individuate the prognostic role of the clinical and histologic features present at the onset of the disease or the time of biopsy, we chose to critically analyze the results of the most valid (23 studies). Actuarial renal survival at 10 years in adults was between 80% and 85% in most of the European, Asian, and Australian studies, but was lower than this in studies from the United States and exceeded 90% in the few studies on children. Concordance existed in this selected literature on the fact that impairment of renal function, severe proteinuria, and arterial hypertension are the strongest and more reliable clinical predictors of an unfavorable outcome. Extent of proteinuria during follow up was an even stronger predictor. In adult patients, a high score of the glomerular and tubulointerstitial lesions predicted a more rapid progression. When the single lesions were analyzed separately, glomerular sclerosis and interstitial fibrosis appeared to be the strongest, most reliable predictors of unfavorable prognosis. More controversial was the role of crescents and capsular adhesions.

Section snippets

Outcome: actuarial renal survival

Despite the relative paucity of relevant symptoms during the clinical course, the outcome is extremely variable and difficult to predict, as we1 and other2 have already emphasized many years ago. Some patients who presented with an episode of macroscopic hematuria, after 40 years and dozens of recurrences of the macroscopic hematuria still have a normal renal function and in repeat biopsies, after the four decades, show only mesangial proliferation with scarce segmental glomerular sclerosis or

Clinical, histologic, and genetic prognostic factors in adults and children

Among the numerous studies on the clinical and histologic predictors of an unfavorable outcome in adult patients with IgAN, we selected, according to the criteria previously mentioned, 23 studies in which an univariate analysis of the various risk factors, each independently considered, has been performed, usually comparing renal survival rates calculated according to the method of Kaplan and Meyer. A multivariate survivorship analysis of the prognostic factors that had appeared more

Can we predict the ultimate outcome for the single patients?

All nephrologists with extensive experience will agree that some patients with rather marked proteinuria and/or severe histologic lesions at the time of biopsy could have rather favorable courses, and some patients with moderate proteinuria and/or mild histologic lesions could have progressive courses in the following years.

Even when some impairment of renal function has already developed and glomerular sclerosis is severe, the speed of progression to ESRF is quite variable. Before the time of

Conclusion

Three clinical features, when present at the time of diagnosis, are strong predictors of an unfavorable outcome: marked proteinuria, arterial hypertension, and impairment of renal function. The latter is in most instances more a marker of advanced, already progressing renal disease than a true marker of bad prognosis of IgAN. However, even marked proteinuria and arterial hypertension are rather nonspecific markers of severity, valid for all glomerular diseases, as already stressed by us.89

It

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    Supported in part by EU Grant OLG1-CT-2002-01215 and by the Associazione per la Ricerca in Nefrologia.

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