Clinicopathological Characteristic Of Anti-glomerular Basement Membrane Disease Combine With IgA Nephropathy Or Membranous Nephropathy

Objective:To investigated the differences in clinical characteristics,pathological features and prognosis of anti-glomerular basement membrane(GBM)disease with IgA nephropathy,anti-GBM disease with membranous nephropathy and anti-GBM disease.Methodology:From January 2004 to December 2015,75patients had been hospitalized in Nanjing Jinling Hospital of Nanjing Military Command and performed renal biopsy.According to the pathological diagnosis,the patients were divided into three group,including 10 cases of anti-GBM disease with IgA nephropathy(with IgAN group),5 cases of anti-GBM disease with membranous nephropathy(with MN group)and 60 cases of pure anti-GBM disease(pure anti-GBM disease group).The clinical,laboratory and pathological indices were compared between the three groups.Kaplan-Meier method was used to calculate the renal survival rate of patients.Results:1)Baseline data:The mean age of the anti-GBM disease with IgAN group was similar to the anti-GBM disease group.The age of onset of anti-GBM disease with MN group was relatively younger and the proportion of males was higher,but there was no statistical significance difference.2)Clinical manifestation:the incidence of upper respiratory tract infection was high in patients with pure anti-GBM disease,and hypertension was high in the course of disease(0%/20%vs 51.7%,p=0.002).The initial symptoms of the patients of the anti-GBM disease with IgAN group were mainly hematuria,the first symptom of the patients of the anti-GBM disease with MN group were edema,and the patients of pure anti-GBM disease group were mainly hematuria and edema,but there was no statistical significance difference.All three groups had some patients with lung involvement,the proportion of patients of anti-GBM disease with IgAN group with pulmonary hemorrhage was relatively lower.Anuria was found in 28.3%of patients with pure anti-GBM disease group,while no patients with anuria in the patients with anti-GBM disease with IgAN or MN group.The main manifestations of anti-GBM disease with IgAN group were rapidly progressive glomerulonephritis(RPGN),but 40%of patients presented with acute nephritic syndrome.The patients of the anti-GBM disease with MN group with nephrotic syndrome(NS)were relatively high.The main feature of patients with pure anti-GBM diease was RPGN,and percentage of RPGN were statistically significant difference(60%vs 60%vs 93.3%,P=0.004)among three groups.3)Laboratory test results:The level of proteinuria in patients with anti-GBM disease with MN group were significantly higher than that in the other two groups,but there was no statistical significance difference.The urinary red blood cell(RBCs)of urinary sediments were highest in patients of anti-GBM disease with IgAN group,but there was no statistical significance difference amount the three groups.Furthmore,the patients in pure anti-GBM disease group were more serious in renal dysfunctions,which represented as highest levels of serum creatinine(Scr,4.61±2.47 vs 4.78±3.06 mg/dl vs 8.84±3.85 mg/dl,P<0.001],a more serious degree of anemia(94.40±15.44 vs 120.4±27.90g/L vs 84.19±18.39g/L,P<0.001),and a highest level of anti-GBM anibodies.4)Renal pathology:the patients in pure anti-GBM disease group were observed to have the highest percentage of crescents in glomeruli(51.68±19.39 vs 50.14±15.67%vs72.69±24.20%,P=0.008)and Bowman’s wall fracture(50%vs 0%vs70%,P=0.005).5)Treatment and prognosis:The patients of pure anti-GBM disease group had the highest rate of progression to end stage renal disease(ESRD)in 1 years during follow-up(50%vs 0%vs86.7%,P<0.001).Conclusion:Anti-GBM disease with IgA nephropathy or membranous nephropathy is different from pure anti-GBM disease in clinical manifestations,laboratory indices,pathology and prognosis.When the patient’s clinical symptoms were atypical,for example,the clinical manifestation of patients was nephrotic syndrome with gross hematuria and rapid deterioration of renal function,or a large amount of macroscopic hematuria and renal function deteriorated faster than typical IgAN,it is appropriate to check the anti-GBM antibody in the early stage,kidney biopsy should be performed as soon as possible.For anti-GBM disease,when massive proteinuria occurs,repeat renal biopsy should be done.Not only does it help with treatment,it also helps to judge the prognosis.