Knowra IgA nephropathy IgA nephropathy IgA nephropathy is an immune-mediated kidney disease in which immunoglobulin A accumulates in glomeruli, often causing blood in the urine and sometimes progressive loss of kidney function.
Galactose-deficient IgA1 : An IgA1 form with reduced galactose in its hinge-region O-glycans, associated with several immune disorders. This abnormal IgA1 can trigger the antibody responses that initiate deposits in IgA nephropathy.
Hematuria : The presence of red blood cells in urine, visible or detectable only by testing. Episodes of visible hematuria, often around respiratory infections, are a common presentation.
IgA vasculitis : A small-vessel vasculitis involving IgA-rich immune deposits, often affecting skin, joints, gut, and kidneys. Its kidney biopsy can resemble IgA nephropathy, but systemic purpura and other features distinguish it.
MEST-C score : A summary of Oxford biopsy features that records mesangial and endocapillary hypercellularity, segmental sclerosis, tubular atrophy or interstitial fibrosis, and crescents. Its prognostic precision varies, leaving uncertainty about how individual biopsy lesions should guide therapy.
Jean Berger : A French nephrologist who described glomerular IgA deposits with Nicole Hinglais in 1968. Berger’s report established the distinctive biopsy finding that gave the disease its former name, Berger disease.
Anti-glycan antibodies : Antibodies that recognize carbohydrate structures attached to proteins or cells. Some recognize exposed hinge-region structures on galactose-deficient IgA1 and help form immune complexes.
Proteinuria : An abnormally high amount of protein in urine. Persistent proteinuria signals kidney injury and helps estimate progression risk.
Lupus nephritis : Kidney inflammation caused by systemic lupus erythematosus and immune-complex deposition. Unlike IgA nephropathy, it typically shows a broader immune-deposit profile and lupus-associated findings.
Clinical prediction tool for IgA nephropathy : A validated model that estimates the risk of kidney-function decline using clinical and biopsy variables. Risk estimates aid counseling, but do not fully resolve who benefits from immunosuppression.
Nicole Hinglais : A French pathologist who co-described glomerular IgA deposits with Jean Berger in 1968. Her pathology work helped identify the defining mesangial IgA pattern.
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