IgA nephropathy (IgA nephropathy, IgAN) , also known as Berger 's disease, is
a glomerular mesangial IgA deposition in IgA or dominated , with or without
other immunoglobulins in the glomerular mesangial deposition of primary
glomerular disease . The clinical manifestations : recurrent episodes of gross
hematuria or microscopic hematuria may be associated with varying degrees of
proteinuria, severe hypertension can occur in some patients or renal
insufficiency.
First : causes :
The exact pathogenesis of IgA nephropathy has not yet entirely clear, a
variety of factors and pathogenesis. Currently more consistent view is IgAN is
caused by glomerular immune complex disease.
1 the relationship between the immune system : IgA nephropathy mainly
multimeric IgA (PIgA) glomerular deposition , indicating that the immune system
has led PIgA IgA molecules occur in the circulatory system and the deposition of
mesangial region .
(2) the relationship with the bone marrow : glomerular mesangial deposition
of IgA1, and is present in the blood similar to the hinge region of IgA1
glycosylation abnormalities, suggesting deposition of IgA nephropathy in IgA
mesangial area of bone marrow-derived IgA.
3 Relationship with cytokines : IgA nephropathy mesangial IgA1 deposition
causes the secretion of inflammatory cytokines in mesangial cells .
Second: pathophysiology
IgA nephropathy iconic pathological changes in glomerular mesangial IgA
deposition . Most patients with concomitant C3, IgG, IgM deposition .
LEE Rating:
Ⅰ level: glomerular lesions: the vast majority of normal , with occasional
mild mesangial widening ( segment ) , with or without cell proliferation ;
tubulointerstitial changes: normal .
Ⅱ level: glomerular lesions: focal glomerular mesangial proliferation and
sclerosis ( 50% ) , rare small crescents ; tubulointerstitial changes: normal
.
Ⅲ grade : glomerular lesions : diffuse mesangial proliferation and broadening
( and occasionally focal stage ) , and occasionally small crescent , balloon
adhesions ; tubulointerstitial changes: Bureau Zaojian interstitial edema,
occasional cell infiltration, rare tubular atrophy .
Ⅳ grade : glomerular lesions : severe diffuse mesangial proliferation,
accompanied by sclerosis, some or all of glomerular sclerosis, visible crescent
( < 45% ) ; tubulointerstitial changes: tubular atrophy , interstitial
infiltration, even see interstitial foam cells .
Ⅴ grade : glomerular lesions: Ⅳ grade lesions similar , but more serious ,
visible crescent ( > 45% ) ; tubulointerstitial changes: grade Ⅳ similar ,
but more severe .
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