| Purpose:This study was conducted to investigate the correlation between clinical manifestations and pathological features and patient prognosis by analyzing the clinicopathological features of patients with ANCA-associated glomerulonephritis(AAGN)in our region,and to provide a theoretical basis for improving the kidney survival rate of patients.Method:Retrospective analysis of patients with pathologically confirmed AAGN by renal puncture biopsy from January 2013 to October 2022 at the First Hospital of Jilin University,clinical data of 62 patients who met the screening criteria were collected and divided into four groups according to Berden’s staging criteria,namely,focal group(11 cases),crescentic group(27 cases),mixed group(18 cases),and sclerotic group(6 cases),comparing the general data,laboratory tests,pulmonary imaging,differences in treatment options and the relationship with prognosis among patients in the four groups.The mean follow-up of patients in this study was 31 - 27 months to observe renal survival,i.e.,whether they progressed to ESRD or died.SPSS 29.0statistical software was applied and the data were analyzed and discussed by the appropriate statistical methods.Result:1.62 patients with AAGN were included,24(38.7%)males and 38(61.3%)females,with a male to female ratio of 1:1.58.The age of males was 49-71 years and that of females was 31-74 years,with an overall mean age of 58.3 - 9.2 years,and no statistically significant differences in the age distribution of different genders were compared.The percentage of MPO-ANCA in the four pathological groups was90.9%,81.5%,100% and 83.3%,and the percentage of PR3-ANCA was 9.1%,8.5%,0% and 16.7%,in that order,and there was no statistical difference in ANCA serological typing distribution among the four groups.2.The first clinical manifestations of AAGN were commonly cough and sputum(60%),edema(45%),malaise(40%),nausea and vomiting(40%),renal function abnormalities(37%)and fever(32%).The median time interval between the first clinical presentation and the diagnosis of AAGN was 35 days,and there was no statistical difference between the pathology groups for comparison.The lung was the most commonly involved extrarenal organ with 100% involvement,followed by the gastrointestinal tract(56%),heart(27%),joint muscle(13%)and skin(12%),but there was no statistically significant difference in the comparison of extrarenal involvement between the four groups.3.Lung imaging showed that the most common imaging manifestation in pulmonary involvement was inflammatory solid changes(71%)with the proportions of 82%,67%,72% and 67% in the four groups,respectively,while interstitial changes were less common(19%)with the proportions of 9%,26%,17% and 17% among the four groups,respectively,and the above differences were not statistically significant when compared among the four groups.4.The median BVAS scores were 16.0,18.0,18.5 and 17.5 for focal,crescentic,mixed and sclerotic types,respectively,with statistically significant differences among the four groups(P < 0.05),and the BVAS scores were lower in focal patients than in the mixed group(P < 0.05).5.The median glomerular filtration rate at admission was 25.8 ml/min/1.73m2,11.9 ml/min/1.73m2,14.9 ml/min/1.73m2 and 11.8 ml/min/1.73m2 in the four groups,respectively,and the differences in e GFR at admission were statistically significant(P< 0.05)when comparing patients between the groups,with focal type e GFR was higher than that of crescentic type(P < 0.05),and the rest of the comparison between groups was not statistically significant.6.Of the 62 patients,8(13%)received hormone therapy alone,48(77%)received hormone therapy combined with cyclophosphamide,5(8%)received hormone therapy combined with other immunosuppressive agents(rituximab,mortemacrolide),5(8%)were treated with plasma exchange,and a total of 24(39%)patients were treated with hemodialysis after admission.7.There was no statistically significant difference in kidney survival between the four groups,but the Kruskal-Wallis survival curve showed an overall trend that the best kidney prognosis among the four groups was the focal type,followed by the crescentic,mixed type,and the worst sclerotic type.8.Cox multifactorial regression analysis showed that e GFR <5 ml/min/1.73 m2 at admission was an independent predictor of patient progression to ESRD or death.Conclusion:1.AAGN is mostly seen in middle-aged and elderly women,with a predominance of MPO positivity.2.lung was the most common organ involved in AAGN,followed by digestive tract,heart,joint muscle and skin.3.eGFR at admission was highest in the focal group and BVAS score was lowest among the four pathology groups.4.The best overall trend in renal prognosis among the four pathology groups was focal,followed by crescentic,mixed,and the worst sclerotic type.5.eGFR <5 ml/min/1.73 m2 at admission was an independent predictor of progression to ESRD or death in patients with AAGN. |
