Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Nephrol. Jan 6, 2016; 5(1): 6-19
Published online Jan 6, 2016. doi: 10.5527/wjn.v5.i1.6
Update on immunoglobulin a nephropathy. Part II: Clinical, diagnostic and therapeutical aspects
Maurizio Salvadori, Giuseppina Rosso
Maurizio Salvadori, Department of Renal Transplantation and Renal Diseases, Careggi University Hospital, 50139 Florence, Italy
Giuseppina Rosso, Division of Nephrology, San Luca Hospital, 55100 Lucca, Italy
Author contributions: Salvadori M designed the research and wrote the manuscript; Rosso G collected the literature data, helped in writing and revised the manuscript.
Conflict-of-interest statement: The authors do not have any conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Maurizio Salvadori, MD, Department of Renal Transplantation and Renal Diseases, Careggi University Hospital, viale Pieraccini 18, 50139 Florence, Italy.
Telephone: +39-55-597151 Fax: +39-55-597151
Received: October 20, 2015
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.

Keywords: IgA nephropathy prevention and control, IgA nephropathy, IgA nephropathy diagnosis, IgA nephropathy prognosis, IgA nephropathy classification, IgA nephropathy therapy

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.