IgA nephropathy (Berger's disease) has become recognized worldwide as one of the most common of the primary glomerulonephritis. The mesangial granular deposits suggested an immune complex disease. The available data evidence that the IgA circulating immune complexes in these patients are heterogeneous. Recent analysis, performed after dissociating the complexes, found both IgA1 and IgG. In fact, high serum levels of IgA rheumatoid factor and shared antibody idiotypes were found in a large proportion of those patients. A close relationship was noted between the presence of cross-reactive idiotypes on mesangial immunoglobulins and the existence of increase levels of serum idiotypes and many patients have increased rates of IgA synthesis either spontaneously or after stimulation of the peripheral blood mononuclear cells by various mitogens. A lot of abnormalities on B and T lymphocytes, related with the IgA immune regulation, have been described. Most of deposited IgA in the mesangium is polymeric and belongs to the IgA1 class. Patients with IgA nephropathy have very often antibodies against exogenous and endogenous antigens. Among the most frequently found are the antibodies against dietary, viral and bacterial antigens as well as against the Fc and Fab portions of immunoglobulins, nuclear and glomerular antigens. The mechanisms of mesangial damage in IgA nephropathy are not well known. Mesangial cells are capable of producing and releasing various lipidic and proteic mediators. The stimulation of mesangial cells, cultured in vitro by IgA or IgG immune complexes induced the release of PAF, PGE2 and superoxide anion. A better knowledge of the mechanism implicated in the abnormality of IgA immune regulation, as well as of the glomerular inflammation response could afford a new therapeutic approach to this nephropathy.
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Case Rep Nephrol
January 2025
Division of Nephrology, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan.
A 63-year-old Japanese housewife was admitted to our hospital because of hematuria and proteinuria lasting for 3 months. At the age of 59 years, she was diagnosed with neurosarcoidosis at another hospital, and she received oral glucocorticoid therapy for 1 year. Her serum angiotensin-converting enzyme (ACE) and 1, 25-dihydroxyvitamin D levels were elevated.
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January 2025
Centre for Public Health, Queen's University of Belfast, Belfast, BT12 6AB, Northern Ireland.
Background: IgA nephropathy (IgAN) is the most common primary glomerulonephritis in the world and is an important cause of chronic kidney disease (CKD) and kidney failure. Outcomes are heterogeneous, and accurate risk stratification is important to identify the highest risk individuals for treatment and to help prevent disease progression. The Oxford classification (OC) is an internationally adopted standard for renal biopsy reporting in IgAN, which measures the degree of histological abnormalities and predicts prognosis.
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January 2025
Shanxi Genetic Engineering Center for Experimental Animal Models, The Fifth Hospital (Shanxi Provincial People's Hospital) of Shanxi Medical University, Taiyuan, Shanxi, China.
Phospholipase A2 receptor 1 (PLA2R1) exists in many animals and plays an important role in membranous nephropathy. In this study, we aimed to evaluate a PLA2R1 knock-in rat model with repaired kidney function to study the molecular mechanisms of membranous nephropathy. We constructed the PLA2R1 knockout [PLA2R1(-)] model and PLA2R1 knock in [PLA2R1(+)] model in rats.
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January 2025
Department of Cardiovascular Sciences, University of Leicester, Leicester, Leicestershire, UK.
Introduction: Endothelin A (ETA) receptor activation is a driver of proteinuria, kidney inflammation, and fibrosis in IgA nephropathy (IgAN). Atrasentan, a selective ETA receptor antagonist, has potential to reduce proteinuria and preserve kidney function in IgAN. ALIGN (NCT04573478) is a phase 3, randomized, double-blind, placebo-controlled clinical trial of atrasentan in patients with IgAN at high risk of kidney function loss.
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January 2025
Renal Department and Nephrology Institute, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, 610072, China.
Background: The factors influencing diffuse crescentic glomerulonephritis renal survival and prognosis remain uncertain. Additionally, there's no literature on the clinical outcomes of IgA nephropathy, lupus nephritis, and IgA vasculitis nephritis in type II patients.
Methods: This study retrospectively examined 107 patients diagnosed with diffuse crescentic glomerulonephritis through biopsy.
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