Peer-review started: October 21, 2015
First decision: November 6, 2015
Revised: November 13, 2015
Accepted: December 3, 2015
Article in press: December 4, 2015
Published online: January 6, 2016
Immunoglobulin A nephropathy (IgAN) is characterized by different clinical manifestations and by long-term different outcomes. Major problem for the physicians is to understanding which patients are at risk of a disease evolution and to prescribe the right therapy to the right patients. Indeed, in addition to patients with a stable disease with no trend to evolution or even with a spontaneous recovery, patients with an active disease and patients with a rapidly evolving glomerulonephritis are described. Several histopathological, biological and clinical markers have been described and are currently used to a better understanding of patients at risk, to suggest the right therapy and to monitor the therapy effect and the IgAN evolution over time. The clinical markers are the most reliable and allow to divide the IgAN patients into three categories: The low risk patients, the intermediate risk patients and the high risk patients. Accordingly, the therapeutic measures range from no therapy with the only need of repeated controls, to supportive therapy eventually associated with low dose immunosuppression, to immunosuppressive treatment in the attempt to avoid the evolution to end stage renal disease. However the current evidence about the different therapies is still matter of discussion. New drugs are in the pipeline and are described. They are object of randomized controlled trials, but studies with a number of patients adequately powered and with a long follow up are needed to evaluate efficacy and safety of these new drugs.
Core tip: Primary immunoglobulin A nephropathy (IgAN) is the most frequent glomerulonephritis. The IgAN is a relatively benign disease however, the long term prognosis should not be considered mild, because, after 20 years of disease evolution, 25% of the patients are going into chronic renal failure. It is essential to find out the risk factors predicting the evolution to end-stage renal disease (ESRD) and to select those patients who may benefit from immunosuppressive treatment. For all patients, it is essential to have a regular clinical control to check any disease evolution, in order to avoid or delay the disease progression to ESRD.