IgA Nephropathy: Emerging Mechanisms of Disease

被引:4
作者
Roberts, Lydia E. [1 ,2 ,3 ]
Williams, Chloe E. C. [4 ,5 ]
Oni, Louise [5 ,6 ]
Barratt, Jonathan [1 ,2 ,3 ]
Selvaskandan, Haresh [1 ,2 ,3 ]
机构
[1] Univ Hosp Leicester NHS Trust, John Walls Renal Unit, Leicester, England
[2] Univ Leicester, Leicester, England
[3] Univ Leicester, Dept Cardiovasc Sci, Leicester, England
[4] Royal Liverpool & Broadgreen Univ Hosp Trusts, Liverpool, England
[5] Univ Liverpool, Inst Life Course & Med Sci, Dept Womens & Childrens Hlth, Liverpool, England
[6] Alder Hey Childrens NHS Fdn Trust Hosp, Dept Paediat Nephrol, Liverpool, England
关键词
Four-hit hypothesis; IgA nephropathy; inflammation; pathogenesis; treatments; FECAL MICROBIOTA TRANSPLANTATION; GENOME-WIDE ASSOCIATION; SPLEEN TYROSINE KINASE; LONG-TERM OUTCOMES; IMMUNE-COMPLEXES; ENDOTHELIN RECEPTOR; ALTERNATIVE PATHWAY; GLOMERULAR DEPOSITS; EPITHELIAL-CELLS; O-GLYCOSYLATION;
D O I
10.25259/ijn_425_23
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis reported across the world and is characterized by immunoglobulin A (IgA) dominant mesangial deposits, which are poorly O-glycosylated. This deposition leads to a cascade of glomerular and tubulointerstitial inflammation and fibrosis, which can progress to chronic kidney disease. The variability in rate of progression reflects the many genetic and environmental factors that drive IgAN. Here, we summarize the contemporary understanding of the disease mechanisms that drive IgAN and provide an overview of new and emerging therapies, which target these mechanisms.
引用
收藏
页码:297 / 309
页数:13
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