Recent advances in the understanding and management of IgA nephropathy.

Kar Neng Lai,Sydney C.W Tang,Joseph C.K Leung

doi:10.12688/f1000research.7352.1

Abstract

Since its first description in 1968, IgA nephropathy has remained the most common form of primary glomerulonephritis leading to chronic kidney disease in developed countries. The clinical progression varies, and consequent end-stage renal disease occurs in 30% to 40% of patients 20 to 30 years after the first clinical presentation. Current data implicate overproduction of aberrantly glycosylated IgA1 as being pivotal in the induction of renal injury. Effective and specific treatment is still lacking, and new therapeutic approaches will be developed after better understanding the disease pathogenesis.

Highlights

IgA nephropathy (IgAN) remains the most common primary glomerulonephritis worldwide
The kidney is a target of injury in IgAN, yet the primary defect originates from a systemic aberrant glycosylation of O-linked glycans in the hinge region of IgA1, resulting in increased serum levels of galactose-deficient IgA1 (Gd-IgA1)
As the immunochemical abnormality of IgA is not corrected by renal transplantation, not surprisingly IgAN can frequently recur in allograft

Summary

Introduction

IgA nephropathy (IgAN) remains the most common primary glomerulonephritis worldwide. Other than diabetic nephropathy, IgAN remains the important health-care issue in nephrology as it often affects young adults and the nephropathy pursues a slow but relentless clinical course. The KDIGO (Kidney Disease: Improving Global Outcomes) 2012 Clinical Practice Guidelines[29] suggest optional use of fish oil in the treatment of patients with persistent proteinuria of more than 1 g/day, despite 3 to 6 months of optimized supportive care including ACEI or ARBs and blood pressure control. (iii) In another Italian study, a subset of IgAN patients with florid glomerular changes treated with MMF and corticosteroids showed remission of proteinuria and reversal of progressive renal failure[48] Given that these mixed results occurred across different ethnic groups and that none of these studies was adequately powered to provide a definitive answer, the 2012 KDIGO Guidelines[29] suggest not using MMF in IgAN. A single-center, open-label, exploratory study examining the effects of bortezomib (Velcade) in IgAN was started in 2010 in the US

Conclusions

32. Floege J: Glomerular disease

Findings

PubMed Abstract