Abstract
1. Definition and background IgA nephropathy (IgAN, also known as Berger’s disease) is a disease characterized by urinary findings suggesting glomerulonephritis; predominantly, IgA is deposited in the glomeruli, with no evidence of other underlying disease. Glomerular hematuria and proteinuria are urinary findings that suggest glomerulonephritis. Renal biopsy findings, which are required for confirming the diagnosis of glomerulonephritis, include IgA deposits mainly in the glomerular mesangium and occasionally in the capillary loops. In many cases, C3 is also co-deposited. The rate of progression to end-stage renal disease (ESRD) is approximately 40 % at 20 years after diagnosis. Treatment may include therapy with renin-angiotensin system (RAS) blockers, antiplatelet agents, oral corticosteroids, fish oil, or non-steroidal immunosuppressive agents; steroid pulse therapy; or tonsillectomy. The therapeutic effects of each have been examined, but an effective treatment regimen is yet to be established.
Highlights
Exacerbation in patients with upper respiratory infections has been well known, thereby suggesting changes in mucosal immunity are involved in the pathogenesis
Many other mechanisms are involved in the pathogenesis of IgAN: production and increase of pathogenic IgA1, IgA1 deposition into the glomeruli, proliferation of mesangial cells and matrix expansion from the deposits, and persistent and progressive glomerulonephritis
IgA nephropathy (IgAN, known as Berger’s disease) is a disease characterized by urinary findings suggesting glomerulonephritis; predominantly, IgA is deposited in the glomeruli, with no evidence of other underlying disease
Summary
Immunoglobulin A (IgA) nephropathy (IgAN) is the most common primary glomerulonephritis, and patients typically require dialysis when the disease progresses to end-stage renal failure. In 2011, the Research for Progressive Kidney Diseases of Ministry of Health and Labour and Welfare (MHLW) and the Japanese Society of Nephrology (JSN) established the collaborative clinical guidelines committee, which published JSN and MHLW Evidence-Based Clinical Practice Guidelines for IgA nephropathy 2014 in Nihon Jinzo Gakkai Shi, 2015;57(1):137. In 2011, the Research for Progressive Kidney Diseases of Ministry of Health and Labour and Welfare (MHLW) and the Japanese Society of Nephrology (JSN) established the collaborative clinical guidelines committee, which published JSN and MHLW Evidence-Based Clinical Practice Guidelines for IgA nephropathy 2014 in Nihon Jinzo Gakkai Shi, 2015;57(1):5–137 This is the English version of that report, which was uploaded on JSN website on July 27, 2015. Establishing practice guidelines for IgAN that are adjusted to the situation in Japan is warranted Responding to this need, the Progressive Renal Dysfunction Research Group of MHLW and JSN decided to develop the evidence-based Clinical Guidelines for IgA Nephropathy 2014. It should be stated clearly that these guidelines are not criteria for deciding physician–patient conflicts or medical malpractice lawsuits
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