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Pauci-immune (pauci- Latin: few, little) vasculitis is a form of vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescent staining for IgG. [1] Often, this is discovered in the setting of the kidney.
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.
Immunsuppressive treatment is the gold standard management in order to achieve remission of the blood vessel inflammation that occurs in active microscopic polyangitis. The current immunosuppressive protocols consists of a combination of high dose of glucocorticoids in combination with either cyclophosphamide or Rituximab . [ 8 ]
Glomerulonephritis (GN) is a term used to refer to several kidney diseases (usually affecting both kidneys). Many of the diseases are characterised by inflammation either of the glomeruli or of the small blood vessels in the kidneys, hence the name, [1] but not all diseases necessarily have an inflammatory component.
In 2014 a study was completed to diagnose glomerulonephritis based on etiology. Using immunofluorescence and light microscopy, the investigators were determining the classification for the disease based on if the disease was immune complex mediated, pauci- immune, or complement mediated.
Pathogenesis of post-streptococcal glomerulonephritis includes injury to the glomerulus by immune complexes (IgG) passively trapped in the glomerulus, which leads to an inflammatory response from recruited immune cells, cytokines, chemical mediators, and complement and coagulation cascade activation. [5]
IgA nephropathy (IgAN), also known as Berger's disease (/ b ɛər ˈ ʒ eɪ /) (and variations), or synpharyngitic glomerulonephritis, is a disease of the kidney (or nephropathy) and the immune system; specifically it is a form of glomerulonephritis or an inflammation of the glomeruli of the kidney.
[5] [6] PR3 directed c-ANCA is present in 80-90% of granulomatosis with polyangiitis, 20-40% of microscopic polyangiitis, 20-40% of pauci-immune crescentic glomerulonephritis and 35% of eosinophilic granulomatosis with polyangiitis. c-ANCA (atypical) is present in 80% of cystic fibrosis (with BPI as the target antigen) and also in inflammatory ...