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IgA nephropathy is the most common glomerulonephritis worldwide; the global incidence is 2.5/100,000 per year amongst adults. [1] Aggressive Berger's disease is on the NORD list of rare diseases. [2] Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus.
IgA nephropathy, also known as Berger's disease, is the most common type of glomerulonephritis, and generally presents with isolated visible or occult hematuria, occasionally combined with low grade proteinuria, and rarely causes a nephritic syndrome characterised by proteinuria, and visible blood in the urine. IgA nephropathy is classically ...
The drug, Fabhalta, is already approved to treat adults with paroxysmal nocturnal hemoglobinuria, a rare blood disorder. With the expanded approval, Novartis' drug entered the IgA nephropathy ...
English: What is IgA nephropathy? IgA nephropathy, sometimes called Berger disease, is a type of nephritic syndrome where abnormal IgA antibody is formed, which results in immune complex deposition in the glomerular mesangium, leading to kidney disease. This video covers the pathophysiology, signs and symptoms, and treatment of IgA nephropathy.
As with IgA nephropathy, serum levels of IgA are high in HSP and there are identical findings on renal biopsy; however, IgA nephropathy has a predilection for young adults while HSP is more predominant among children. Further, IgA nephropathy typically only affects the kidneys while HSP is a systemic disease.
The symptoms at onset are very similar to IgA nephropathy and include abdominal pain, hematuria, edema, and oliguria. [ 18 ] Henoch–Schönlein purpura (HSP) - Often considered a systemic form of IgA nephropathy , Henoch–Schönlein purpura (HSP) is a systemic small-vessel vasculitis that is characterized by deposition of IgA antibody immune ...
Rapidly progressive glomerulonephritis (RPGN) is a syndrome of the kidney that is characterized by a rapid loss of kidney function, [4] [5] (usually a 50% decline in the glomerular filtration rate (GFR) within 3 months) [5] with glomerular crescent formation seen in at least 50% [5] or 75% [4] of glomeruli seen on kidney biopsies.
Sparsentan was approved in the US under accelerated approval based on reduction of proteinuria. [1] [5] [12] In September 2024, the US FDA converted the conditional approval in the kidney disease IgA nephropathy (IgAN) into a full approval based on positive long-term confirmatory results from the PROTECT Study demonstrating that sparsentan significantly slowed kidney function decline over two ...