Analysis Of Epidemiology And Clinical Characteristics Of Anti-glomerular Basement Membrane Disease

Objective:To explore the epidemiological and clinical characteristics of anti-glomerular basement membrane(GBM)disease by retrospective analysis,so as to provide more data and deepen the comprehension of anti-glomerular basement membrane(GBM)disease in ChinaMethods:The clinical data of 21 patients diagnosed with Anti-GBM disease in our hospital during the past 10 years(2010-2020)were retrospectively analyzed.Including general clinical information,laboratory tests,pathological results and prognosis of patients.Result:1.The average age of 21 patients in this study was 48.0±20.02 years(age range 17-86 years),most of them were women,the ratio of men to women was 8:13.7 patients(33%)were aged 20 to 40 years,and the ratio of men to women was 4:3;8 patients(38%)were between 50-70 years old,with a male to female ratio of 1:3.There was no significant difference in gender between the two age groups(P=0.315).Of the 21 patients,8(38%)developed the disease in the spring and 9(43%)developed the disease in the winter.There were 15 patients(71.4%)with a history of respiratory tract infection before the onset,and 6 patients(28.6%)had urinary tract infections.2.The clinical manifestations of the patients varied,and all 21 patients presented with rapidly progressive glomerulonephritis,of which 2 cases were accompanied by alveolar hemorrhage.19 patients had hematuria,16 had proteinuria,12 had fever,and 2 had hemoptysis.3.Increased serum creatinine levels were observed in all patients,including 16 patients with serum creatinine ?442umol/L at admission.Serum anti-GBM antibody was positive in 21 patients,among which 15 patients were only positive for anti-GBM antibody,and 4 patients were both positive for anti-GBM antibody and ANCA,with a male-female ratio of 1:3.2 patients had both anti-GBM antibodies and ANA positive.Compared with patients in the anti-GBM antibody single positive group,the average age of patients in the group of both anti-GBM antibodies and ANCA positive was significantly older(69.50±4.80),the creatinine level at admission was higher,with an average value of(1142.18±406.03),both of which have statistical significance.However,there was no significant difference in the efficacy and the level of anti-GBM antibody at admission between the two groups.4.8 patients had complete pathological results,and they all found crescentic glomerulus.75%of the patients had more than 50%of glomerulus affected by crescents In 75%of patients,immunofluorescence showed typical linear deposition of IgG and C3 along the glomerular capillary wall.Among them,3 patients had IgA deposition and 1 patient had fibrinogen linear deposition.25%of patients had atypical immunofluorescence,and no obvious antibody deposits were seen.5.Of the 21 patients,10 patients received a combination of plasma exchange,corticosteroids,and cyclophosphamide.16 cases(76.2%)required renal replacement therapy immediately after admission,and only 1 case improved;Five patients(23.8%)did not require renal replacement therapy at the time of admission,and one patient needed replacement therapy due to the progress of the disease,but did not receive dialysis due to personal reasons and was discharged automatically.The remaining 4 patients did not require renal replacement therapy,among which 1 patient had normal renal function and achieved clinical remission.The serum creatinine level of 2 patients was lower than before,but did not return to normal level.The renal function of another patient was not significantly relieved after treatment,and the treatment was ineffective.At the time of admission,12 patients(57.14%)had creatinine greater than or equal to 550 umol/L,and all of them were ineffective after treatment;9 patients(42.86%)had creatinine less than 550 umol/L,of which 5 were ineffective and 4 were effective.Patients with serum creatinine greater than 550 umol/L at admission had poor efficacy(P=0.21).There was no significant difference in the level of initial anti-GBM antibodies among patients with different efficacy.Conclusion:1.There are more women in anti-GBM disease,more common in spring and winter,and most(71.4%)patients have pre-infection before the onset of disease.The age of onset is bimodal distribution,respectively 20-40 years old and 50-70 years old.2.Patients with anti-GBM disease have different clinical manifestations.In addition to varying degrees of hematuria and proteinuria,fever is also more common.3.A small number of patients combined with ANCA-positive who had a relatively older age,a higher creatinine levels at admission.but the gender and prognosis of patients were not significantly different from those of single anti-GBM antibodies positive patients.4.Immunofluorescence examination showed that typical IgG and C3 were deposited linearly along the glomerular capillary wall.In some patients,immunofluorescence examination was not typical.5.The prognosis of anti-GBM disease is poor,and the prognosis of the patients is related to the dependence on renal replacement therapy and serum creatinine level at the time of admission,but there is no significant correlation with the anti-gbm level at the time of onset.