IgA肾病英文教学课件

1、Case24-year-old, male Main complaints Repeated gross hematuria for 4 yrs History of Present Illness: The pts found his urine are brown,1 day after upper respiration infection.the gross hematuria could disappear after the treatment with antibiotics.the symptom repeated 12 times a year.第1页，共47页。Case b

2、etween the episode, urine analysis : persistent proteinuria and hematuria ( Pro BLD3) Lab：IgG:576, IgA:142, IgE: femaleOccur at all agespredominate：older children and young adults, 20+30+ y/o：the common cause of CRF第8页，共47页。Etiology第9页，共47页。Most cases are idiopathicSLE ,Schnlein-Henoch SyndromLiver

3、cirrhosis.Gluten enteropathy（非热带性口炎性腹泻）.HIV infection.Wegeners granulomatosis treated with immunosuppressive agents and URI(upper respiratory infection).Familial.Dermatitis herpatiformis（疱疹性皮炎） and seronegative arthritis（血清阴性关节炎）, psoriasis (牛皮癣). Gougerot-Houwe syndrome(干燥综合征) 第10页，共47页。Pathogenesi

4、s第11页，共47页。Unknow: what causes IgA deposits to form in the kidneys . IgA nephropathy may run in families or be related to respiratory infections. No consistent trigger for the disease has been found第12页，共47页。Abnormality of IgA regulation in response to an environmental antigen.Plasma IgA concentrati

5、on increased in 50% of cases.Direct correlation to circulation IgA complexes and disease severity.Increase in IgA- specific B and T Lymphocytes in response to URI(upper respiratory infection) .Decreased mucosal immunity.第13页，共47页。IgA immune complex activates alternate complement pathway thus elimina

6、tion by monocyte-macrophage system is slow and more complexes are available to be deposited on the glomerular membrane.第14页，共47页。Altered structure of IgA complexes and electrostatic charge also slows the clearance mechanism.IgA-immune complex binds to fibronectin which helps in clearance after bindi

7、ng with uteroglobin. Abnormal uteroglobin or impaired production prevents clearance.第15页，共47页。第16页，共47页。Pathology第17页，共47页。Light microscopy finding focal or diffuse mesangial proliferation and matrix expansion.Minor glomerular chang(20%), FSGS(28%),mesangial proliferative nephritis (50%), focal segm

8、ental glomerulonephritis, necrotizing glomerulonephritis,mesangiocapillary glomerulonephritis, crescentic glomerulonephritis, 第18页，共47页。Globular deposits of IgA ( along with C3 and IgG ) as demonstrated by Immunofluorescence microscopy in the mesangium and glomerular capillary wall.Electron microsco

9、py showing electron dense deposits of mesangium.第19页，共47页。第20页，共47页。第21页，共47页。Mesangial electron dense deposits with mesangial matrix & cellularity in IgAN第22页，共47页。Clinical Presentation第23页，共47页。Clinical feature：1. predilection in young men： predominate:1315yr2. many pts with forerunner infection b

10、efore episode or relapse3. hematuria is very common第24页，共47页。一、hematuria ：infection or tired 40 to 50% present with recurrent gross hematuria. 30 to 50% with microscopic hematuria and protienuria. 20 to 25 % progress to gross hematuria.第25页，共47页。二、proteinuria with or without microscopic hematuria th

11、e commonest clinical feature(50%)三、nephrotic syndrome about 34%， a report in China16.7 第26页，共47页。四、Hypertension Adult IgA50%,children 5%。 about 6 yrs before renal failure。some patients malignant hypertension五、Chronic renal failure 1020yrs after dignosis，some pts at the first visit。第27页，共47页。六、 Acute renal failure（1g/day) Pool Prognosis Factors：第46页，共47页。Po