| Background and Objective:Immunoglobulin A nephropathy(IgAN)is the most common glomerulonephritis worldwide,affects almost 1%of the population.It accounts for 30%of primary glomerulonephritis in our country,also is the most common chronic renal disease.It is the fist primary cause responsible for end-stage renal disease(ESRD)in China.More and more nephrologists think IgAN is virtually a syndrome,characterized by deposits of IgA in the renal mesangium and capillary loop.It is reported that serum IgA1 molecules of IgA nephropathy are deficient in galactose(Gal)residues on O-linked glycan chains, and aberrantly glycosylated IgA1 play a very important role on the mechanism of IgAN.A renal biopsy is required for an accurate diagnosis of IgA nephropathy, and the degree of renal lesions is very important for treatment and prognosis. However,IgAN has the potential in slowly progressive chronic renal impairment, It is very hard for patients to accept renal biopsy more times to estimate progression of the reanl lesions.This study is to analysis the clinical and pathological chatacteristics of 2204 patients with IgAN,and the relations between them.There were other two aims in the study reported here,to investigate the clinical risk factors associated with the renal pathologic lesions of IgAN patients with nephrotic syndrome;to evaluate the clinical value of detecting serum underglycosylated IgA1 in diagnosis and differentiation of IgAN.Subjects and Methods:(1)The clinical and pathological data of 2204 cases with IgAN were analyzed respectively;(2)The clinical index of different severity of renal pathologic lesions were compared of 118 IgAN patients with nephrotic syndrome,as well as the relationship between renal pathologic lesions and clinical factors were studied,and the predicted factors were analyzed using step-wise multiple variance regression analysis;(3).Serum underglycosylated IgA1 levels were test in 130 cases with IgA nephropathy(IgAN)and 50 cases who suffered from other primary glomerulonephritis,Sera from 40 health examination persons were used as control group.And the diference of serum underglycosylated IgA1 levels were compared.Results:(1)IgAN account for 34.6%in all patients who underwent renal biopsy in the same stage,and 42.9%in primary glomerular disease.Tthe ratio of male to female was 1.49:1,the apparent peak incidence age was 20~40 years old (59%),the apparent age of male was younger than female(P＜0.05).Gross hematuria is more commom in younger patients,incidience of hypertention,renal failure was 40.1%and 22.1%,respectively.The incidience risk of mass proteinuria,hypertention,renal failure was increased by aging.The sex ratio of different age stage was different.Asymptomatic abnormal urinalysis was the most common present(35.4%)of IgAN,the incidence of age and sex ratio were different among different clinical stypes.Renal lesions of patients with isolated microscopic hematuria,recurrent macroscopic hematuria,asymptomatic abnormal urinalysis were more slightly than those with clinical type of mass proteinuria, hypertention,rapidly progressive renal failure(P＜0.05).(2)The incidence of hypertension,renal failure,full field of microscopic hematuria is lower in group A than in group B(11.8%vs 63.1%,15%vs 41.7%, 14.7%vs 50%,respectively.P＜0.01).MAP,UP＜6g/d,microscopic hematuria＞5×10~4/ml were independent predictors of the severity of renal pathologic lesion in IgA nephropathy patients with nephrotic syndrome(OR=1.048,3.227,6.578, P=0.034,0.047,0.002.respectively.).Meanwhile,with the decrease of hemoglobin,the severity of hypertension and microscopic hematuria,the renal pathologic lesion are more severe.(3)The level of serum underglycosylated IgA1 in patients with IgAN was significantly hingher than both control group(median,0.40 vs 0.14,P＜0.001)and those patients with other primary glomerular diseases(median,0.40 vs 0.17, P＜0.01).Non-IgA mesangial proliferative glomerulonephritis account for 48% among other primary disease.Conclusions:(1)IgAN is the most common glomerulonephritis in China,the incidience is 34.6%,and account for 41.9%in primary glomerular disease.The ratio of male to female is 1.49:1,the apparent peak incidence age is 20～40 years old(59%),Gross hematuria is more commom in younger patients,incidience of hypertention,renal failure is 40.1%and 22.1%,respectively.The incidience risk of mass proteinuria,hypertention,renal failure was increased by aging.Different clinical phenotype have different pathologic renal lesions,and may imply different prognosis.(2)IgA nephropaty patients with nephrotic syndrome have different clinical manifestations and pathologic lesions,patients with decreased hemoglobin, accompanied by hypertension and mass microscopic hematuria have more severe renal lesions.(3)Detection of serum underglycosylated IgA1 level with ELISA assay seems to be of significant clinical value in diagnosis and differentiation of IgAN.
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