Abstract
Immunoglobulin A nephropathy (IgAN) is the most frequent glomerular disease worldwide and a leading cause of end-stage renal disease. Particularly challenging to the clinician is the early identification of patients at high risk of progression, an estimation of the decline in renal function, and the selection of only those that would benefit from additional immunosuppressive therapies. Nevertheless, the pathway to a better prognostication and to the development of targeted therapies in IgAN has been paved by recent understanding of the genetic and molecular basis of this disease. Merging the data from the Oxford Classification validation studies and prospective treatment studies has suggested that a disease-stratifying algorithm would be appropriate for disease management, although it awaits validation in a prospective setting. The emergence of potential noninvasive biomarkers may assist traditional markers (proteinuria, hematuria) in monitoring disease activity and treatment response. The recent landmark trials of IgAN treatment (STOP-IgAN and TESTING trials) have suggested that the risks associated with immunosuppressive therapy outweigh the benefits, which may shift the treatment paradigm of this disease. While awaiting the approval of the first therapies for IgAN, more targeted and less toxic immunotherapies are warranted. Accordingly, the targeting of complement activation, the modulation of mucosal immunity, the antagonism of B-cell activating factors, and proteasomal inhibition are currently being evaluated in pilot studies for IgAN treatment.
Highlights
Immunoglobulin A nephropathy (IgAN), first described in 1968 by Jacques Berger [1], is the most frequent primary glomerular disease worldwide diagnosed by kidney biopsy [2]
It is important to note that in the majority of cases, the disease starts at an early age with few manifestations and follows a relentlessly progressive clinical course [10], which is an important challenge in countries that lack screening programs where IgAN may remain undiagnosed or is captured at an advanced stage of disease when a particular treatment will not be of benefit [1,10]
The scope of this review is to provide an update of the current view of pathogenesis, prognosis, and treatment options of IgAN
Summary
Immunoglobulin A nephropathy (IgAN), first described in 1968 by Jacques Berger [1], is the most frequent primary glomerular disease worldwide diagnosed by kidney biopsy [2]. Current clinical guidelines [12] assess the risk of disease progression based on classical risk factors (proteinuria, renal function at renal biopsy and during follow-up) and recommend corticosteroids only in patients with persistent proteinuria and relatively preserved renal function, albeit with a low quality of evidence (2C). These recommendations are based on previous randomized clinical trials, which were criticized for the inconsistent use of a renin-angiotensin system (RAS) blockade and the inadequate reporting of adverse events [13,14,15,16]. Despite current treatment strategies that merely slow disease progression, by transitioning from pathogenesis to bedside, the future perspective of IgAN is targeted toward the individualized management of patients
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