Immunoglobulin A vasculitis(IgAV)is the most common form of childhood vasculitis.The disease has the characteristic of IgA1 immunoreactivity deposition,neutral cell and complement factors infiltration,and the pathogenesis is not yet clear.IgAV often affects small vessels of skin,gastrointestinal tract,joints,and kidney.The symptoms are limited,but some patients develop nephritis,hematuria and proteinuria,similar to IgA nephropathy,which develop to the end-stage renal disease.Immune complexes containing galactose-deficient IgA1(Gd-IgA1)play a key role in the pathophysiology of IgAV;through Fc receptor(CD89),the inflammation of the neutral granule cells in the peripheral blood vessel and the proliferation and inflammation of the renal glomerular mesangial are caused.In this review,we discuss the putative role of IgA,IgA receptors,neutrophils and other factors such as the complement system,genetics,and infections in the pathogenesis of IgA vasculitis,as well as the discovery of new therapies.
关键词
过敏性紫癜/IgA血管炎/半乳糖缺乏IgA1/IgA受体/抗内皮细胞抗体
Key words
henoch schonlein purpura/immunoglobulin A vasculitis/galactose-deficient immunoglobulin A1/IgA receptor/anti-endothelial cell antibodies
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