Clinical And Pathological Features Of ANCA-associated Vasculitis And Corrective Factors Of Prognosis: A Single-center Study

Purpose:The purpose of this study was to investigate clinical and pathological features of ANCA-associated vasculitis,explore the predictors of ANCA-associated vasculitis with renal damage and analyze the corrective factors of prognosis.Methods:A total of 259 patients diagnosed as ANCA-associated vasculitis in the first hospital of Jilin University from January 2010 to October 2020 were retrospectively analyzed.After screening by exclusion criteria,all patients met the inclusion criteria and could obtain complete clinical data.The laboratory examination,imaging examination and renal biopsy pathological results of follow-up from January 2010 to October 2020 were investigated.Results:There were 237 cases in MPO-ANCA group and 22 cases in PR3-ANCA group.MPO-ANCA group was older,with an average age of 64.60 years.PR3-ANCA group was53.77 years old.MPO-ANCA group was more prone to anemia,with a total of 216 cases(91.5%).PR3-ANCA group was more likely to involve eyes and ears(9 cases,40.9%).There were 236 cases(91.1%)in the renal damage group and 23 cases(8.9%)in the non-renal damage group.Serositis,blood system and gastrointestinal tract involvement were more common in renal damage group,and anemia,hyperuricemia,hypoproteinemia and pleural effusion were more common in renal damage group than in non-renal damage group.In terms of treatment plan,the proportion of hormone pulse therapy in renal damage group was higher.The prognosis of renal damage group was worse than that of non-renal damage group.Hemoglobin level(or: 1.070,95% CI: 1.041 ～ 1.099,P < 0.01)was a predictor of AAV with renal damage.181 patients with follow-up data were divided into two groups according to the last follow-up survival,40 cases in death group and 141 cases in survival group.Then 141 patients in survival group were divided into two groups according to the renal survival,105 patients in renal survival group and 36 patients in ESRD group.The death group was older than the survival group,with higher proportion of serositis,pneumonia,hemoptysis,hematological system involvement,lower hemoglobin,albumin and complement,higher white blood cell count,serum creatinine,blood urea nitrogen and CRP levels,and more common pleural effusion and pericardial effusion.Compared with the renal survival group,the ESRD group had a higher proportion of patients with renal damage and renal replacement therapy,more serositis,gastrointestinal and blood system involvement,lower hemoglobin and complement C3 levels,and more common pleural effusion.The common types of renal pathology were crescent type,mixed type,focal type and sclerotic type.There was no significant correlation between different types and prognosis.Infection was the most common complication during follow-up,and respiratory system involvement was the most common cause of death.The 1-month survival rate was 85.6%.The 3-month,6-month and2-year cumulative survival rates were 79.4%,75.5% and 73.4%,respectively.Age(or:1.048,95% CI: 1.009-1.088,P < 0.05)and renal damage at diagnosis(or: 0.161,95% CI:0.041-0.633,P < 0.01)were independent risk factors for death.The median renal survival time was 3.63 years.The 1-month renal survival rate was 87.3%.The 3-month,9-month and2-year renal survival rates were 81.2%,75.8% and 70.4%,respectively.Renal replacement therapy(or: 0.057,95% CI: 0.022-0.144,P < 0.05)was an independent risk factor for ESRD in AAV patients.Conclusions:(1)AAV patients with different ANCA serotypes show different clinical characteristics.MPO-ANCA patients are older than PR3-ANCA patients,and anemia is more common.PR3-ANCA patients have more eye and ear involvement.(2)Most patients with AAV were diagnosed with different degrees of renal damage.The patients with renal damage were more likely to have multiple system involvement than those without renal damage,and the proportion of using hormone pulse therapy was higher.The prognosis of patients with renal damage was poor.Hemoglobin level was a predictor of AAV with renal damage.(3)The renal function of patients at the time of diagnosis has an impact on the prognosis of AAV.Age and renal damage are the risk factors for death of AAV patients.Renal replacement therapy is the risk factor for ESRD of AAV patients at the time of diagnosis.