泌尿系统课件：IgAN 2014.6.24.ppt

1、Casev24-year-old, malev Main complaints Repeated gross hematuria for 4 yrs v 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.Case betwee

2、n the episode, urine analysis : persistent proteinuria and hematuria ( Pro BLD3) Lab：IgG:576, IgA:142, IgE: femalevOccur at all agesvpredominate：older children and young adults, 20+30+ y/o：vthe common cause of CRFEtiologyvMost cases are idiopathicvSLE ,Schnlein-Henoch SyndromvLiver cirrhosis.vGluten

3、 enteropathy（非热带性口炎性腹泻）.vHIV infection.vWegeners granulomatosis treated with immunosuppressive agents and URI(upper respiratory infection).vFamilial.vDermatitis herpatiformis（疱疹性皮炎）（疱疹性皮炎） and seronegative arthritis（血清阴性关节炎）（血清阴性关节炎）, psoriasis (牛皮癣). Gougerot-Houwe syndrome(干燥综合征) PathogenesisvUnkn

4、ow: 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 foundPathogenesisvAbnormality of IgA regulation in response to an environmental antigen.vPlasma IgA concentration increase

5、d in 50% of cases.vDirect correlation to circulation IgA complexes and disease severity.vIncrease in IgA- specific B and T Lymphocytes in response to URI(upper respiratory infection) .vDecreased mucosal immunity.PathologyvLight microscopy finding focal or diffuse mesangial proliferation and matrix e

6、xpansion.Minor glomerular chang(20%), FSGS(28%),mesangial proliferative nephritis (50%), focal segmental glomerulonephritis, necrotizing glomerulonephritis,mesangiocapillary glomerulonephritis, crescentic glomerulonephritis, PathologyvGlobular deposits of IgA ( along with C3 and IgG ) as demonstrate

7、d by Immunofluorescence microscopy in the mesangium and glomerular capillary wall.vElectron microscopy showing electron dense deposits of mesangium.vMesangial electron dense deposits with mesangial matrix & cellularity in IgANClinical PresentationClinical feature：1. 1. predilection in young men： pre

8、dominate:1315yr2. many pts with forerunner infection before episode or relapse3. hematuria is very commonClinical Presentationv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 hematuri

9、a.Clinical Presentation二、二、proteinuria with or without microscopic hematuria the commonest clinical feature(50%)三、三、nephrotic syndrome about 34%， a report in China16.7 Clinical Presentation四、Hypertension Adult IgA50%,children 5%。 about 6 yrs before renal failure, some patients malignant hypertension

10、五、Chronic renal failure 1020yrs after dignosis，some pts at the first visit。Clinical Presentation六、 Acute renal failure（1g/day) Pool Prognosis Factors：Poor Prognosis FactorsvPathological expression： 1. Diffuse mesangial proliferation 2. Extensive glomerular crescents 3. Glomerulosclerosis and tubulointerstitial change Thanks for your attention!