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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.
Clinical features may include constitutional symptoms like fever, arthralgia, myalgia, loss of appetite, weight loss and fatigue.A variety of organs can be affected, which causes a wide range of symptoms such as cough, shortness of breath, hemoptysis (coughing up of blood), symptoms of kidney failure, skin manifestations (palpable purpura and livedo racemosa [1]), seizures or peripheral ...
Rapidly progressive glomerulonephritis, also known as crescentic GN, is characterised by a rapid, progressive deterioration in kidney function. People with rapidly progressive glomerulonephritis may present with a nephritic syndrome. In management, steroid therapy is sometimes used, although the prognosis remains poor. [9]
Diffuse proliferative glomerulonephritis (DPGN) is a type of glomerulonephritis that is the most serious form of renal lesions in SLE and is also the most common, occurring in 35% to 60% of patients. [1] In absence of SLE, DPGN pathology looks more like Membranoproliferative glomerulonephritis [citation needed]
Presentation of nephrotic syndrome in the context of mesangial proliferative glomerulonephritis have been treated with immunosuppressants, such as steroids and cyclophosphamide. [2] Presentation with nephrotic syndrome can resolve with treatment, but can also progress. [10]
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Immunoglobulin A (IgA) vasculitis, formerly referred to as Henoch–Schönlein purpura, is a type of immune complex vasculitis that primarily affects IgA deposits in small vessels. Acute enteritis, glomerulonephritis, arthralgias and/or arthritis, and cutaneous purpura are the most common clinical manifestations. Children are more likely than ...