| ObjectiveAntineutrophil cytoplasmic antibody(ANCA)associated vasculitis is a kind of systemic autoimmune disease,leading to multisystem involvement.57-82%of ANCA-associated vasculitis patients have lung abnormalities,of which interstitial lung disease(ILD)is the most common lung abnormalities with poor outcome,leading to huge disease burden.However,current knowledge about ANCA-associated vasculitis combined with ILD is still limited.The prevention,screening,early diagnosis,disease assessment and individualized treatment of ANCA-associated vasculitis combined with ILD are still challenging.In this study,we retrospectively analyzed the clinical data of ANCA-associated vasculitis combined with ILD to:(1)summarize the prevalence and clinical characteristics of the disease;(2)identify the risk factors associated with the presence of ILD;(3)elucidate the risk factors associated with poor outcome;in patients with ANCA-associated vasculitis.Methods486 patients with ANCA-associated vasculitis who had undergone chest CT at the First Affiliated Hospital of Zhengzhou University from January 1,2012 to December 12,2018 were included.Based on the presence of ILD on chest images or not,patients were divided into two groups(patients with ILD versus patients without ILD).Clinical investigations were compared between two groups.And univariate and multivariate Logistic regression analysis were employed to identify the risk factors associated with the presence of ILD in patients with ANCA-associated vasculitis.the possible risk factors of ANCA-associated vasculitis complicated with ILD.Kaplan-Meier curve were conducted to compare the survival time between the two groups by Log-rank test.And multivariate COX proportional hazard regression models were used to investigate the risk factors associated with short death in patients with ILD.Results486 patients with ANCA-associated vasculitis were finally enrolled in this study,The mean age of the included patients was(61.05±13.31)years,and 236 were male(48.55%).428(88.1%)were categorized as microscopic polyangiitis(MPA),and 58(11.9%)were granulomatosis with polyangiitis(GPA).Among of 486 patients with ANCA-associated vasculitis,172 patients(35,39%)were diagnosed ILD.Compared to patients without ILD,patients with ILD were older[(65.04±10.08)years,vs.(58.86 ± 14.33)years,P<0.001],more likely to be male and smokers(62.21%vs.41.08%,P<0.001)and(28.49%vs.16.88%,P<0.001),respectively.The BVAS score and duration of follow-up did not differ between the two groups(P>0.05).Patients with ILD were more likely to have cough and chest tightness than those without ILD,which were(62.21%vs.51.96%,P=0.017)and(36.63%vs.23.89%,P=0.003)respectively.However,there was no significant differences in fever,hemoptysis,weight loss,anemia and other symptoms between the two groups.Regarding laboratory tests,patients with ILD were more likely to be MPO-ANCA positive(93.02%vs.82.80%,P=0.002),and have higher lymphocyte counts[1.50(1.00,2.00)vs.1.22(0.93,1.71),P<0.001]than those without.Patients with ILD had lower rate of PR3-ANCA positive(6.40%vs.16.88%,P=0.001)and titers of PR3-ANCA[55.80(23.01,173.52)RU/ml vs.200.01(99.32,438.40)RU/ml,P=0.009]than those without ILD.By contrast,There were no significant differences in MPO-ANCA titer,the level of CRP,the level of ESR,neutrophil counts and the level of creatinine level between the two groups.Regarding radiological features,106 patients(61.63%)with ILD had reticular pattern(mainly located at the base of the lungs),66 patients(38.37%)had ground glass opacities,and 33 patients(19.19%)showed honeycombing(mainly distributed in subpleural and bilateral lung base).Of 172 patients with ILD,there were 101 patients(58.72%)were categorized as UIP,and 42 patients(24.42%)were categorized as NSIP.Compared to patients with ILD,patients without ILD were more likely to have eyes[20(6.37%)vs.3(1.74%),P=0.022],and ENT[56(17.83%)vs.13(7.56%),P=0.002]involvement.In contrast，there were no significant differences in joint,skin,abdomen,cardiovascular,kidney,central nervous system and peripheral nerve involvement between the two groups(all P>0.05).In patients with ILD,kidney(65.70%),cardiovascular(23.25%),joint(13.37%)involvement were commonly involved organs.Regarding risk factors associated with the presence of ILD in ANCA-associated vasculitis,mala[OR=2.77,95%CI(1.83-4.20),P<0.001],older age[OR=1.04,95%CI(1.02-1.06),P<0.001)],MPO-ANCA positive[OR=2.14,95%CI(1.45-8.23),P=0.037],higher lymphocyte counts[OR=2.11,95%CI(1.56-2.85),P<0.001]were the risk factors associated with the presence of ILD by using multivariate logistic regression analysis.There was no significant difference regarding treatment strategies between the two groups(P>0.05).Among the 486 ANCA-associated vasculitis patients,67(13.79%)were lost during follow-up,419 patients complete the follow up and included the final analysis.The median time of follow-up was[31(19-56)]months During follow-up,128 patients died,with a death rate of 30.55%.The survival rates at 1,3 and 5 years of ANCA-associated vasculitis patients were 97.14%,91.89%and 85.68%,respectively.144 patients with ILD complete the followed up,65 patients died(35 patients died of respiratory failure),the death rate was 45.14%.The survival rates at 1,3 and 5 years of patients in ANCA-associated vasculitis with ILD were 91.68%,76.39%and 58.33%,respectively.275 patients without ILD complete the followed up,and 63 patients died,with a death rate of 22.91%.The survival rates at 1,3 and 5 years in patients without ILD were 95.64%,86.91%,and 78.18%,respectively.In the Kaplan-Meier survival curve,the patients in group of ANCA-associated vasculitis with ILD had lower survival rates compared with those without during follow-up(χ2=18.605,P<0.001).Multivariate Cox survival analysis showed that older age[OR=1.03,95%CI(1.00-1.06),P=:0.044],higher BVAS score[OR=1.96,95%CI(1.01-3.79),P=0.045],and higher level of creatinine[OR=1.001,95%CI(1.0011.002),P=0.006]were the risk factors associated with poor prognosis.Conclusion1.ILD is very common in patients with ANCA-associated vasculitis.2.In the patients of ANCA-associated vasculitis,people who are older age,male and MPO-ANCA positive tend to combine with ILD.3.Patients with ILD have higher death rate than those without.Older age,higher BVAS score and high level of creatinine are the independent risk factors associated with poor outcome in patients with ILD. |
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