ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1673

Factors Associated with Overall and First-Year Mortality in Turkish Patients with ANCA-Associated Vasculitides: Retrospective, Multicentre Trial

Onay Gercik1, Emre Bilgin 2, Dilek Solmaz 1, Irfan Ocal 3, Arzu Saglam 4, Riza Kardas 5, Ozge Aybi 5, Gokhan Kabadayi 6, Zeki Soypacaci 7, İdil Kurut Aysin 8, Tolga Yildirim 9, Omer Karadag 10 and Servet Akar 11, 1Izmir Katip Celebi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 2Hacettepe University Vasculitis Centre, Ankara, Turkey, Ankara, Turkey, 3Izmir Katip Celebi University, Faculty of Medicine, Department of Pathology, Izmir, Turkey, 4Hacettepe University, Faculty of Medicine, Department of Pathology, Ankara, Turkey, 5Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Ankara, Turkey, 6Izmir Katip Celebi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 7Izmir Katip Celebi University Faculty of Medicine, Department of Internal Medicine, Division of Nephrology, Izmir, Turkey, 8Izmir Katip Celebi University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 9Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Nephrology, Ankara, Turkey, 10Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, ankara, Turkey, 11Izmir Katip Celebi University, Faculty of Medicine, Division of Rheumatology, İzmir, Turkey

Meeting: 2019 ACR/ARP Annual Meeting

Keywords:

Session Information

Date: Monday, November 11, 2019

Session Title: Vasculitis – ANCA-Associated Poster II

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Overall mortality in ANCA-associated vasculitides (AAVs) over the last two decades has been reported to be decreasing with the use of immunosuppressive therapies. However, despite treatment, mortality rates remain high, particularly in the first year after diagnosis. In this study, we aimed to determine the prevalence of mortality in AAV patients and to investigate the factors that may be associated with first-year and overall mortality.

Methods: AAV patients who were categorized according to the 2012 Chapel Hill consensus nomenclature, were included in this study from two university centers. Diagnostic subgroups were; granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA) and renal-limited vasculitis (RLV). Patients with only clinical renal involvement and diagnosed by renal biopsy were classified as RLV. The clinical and demographic characteristics of the patients were collected retrospectively. Factors predictive of mortality were evaluated by Kaplan-Meier method and the Cox proportional hazard model.

Results: In total 232 (123 [53%] male and mean age at diagnosis51.4±15.75 years) AAV patients (137 [59%] GPA; 40 [17%] MPA; 30 [13%] RLV and 25 [11%] EGPA) were included in the analysis. ANCA positivity was detected in 82% (180/220) patients with IIF and/or ELISA. 167 patients (73%) had renal involvement and among them, one in third patients (52/167) had developed end-stage renal disease (ESRD). Other clinical features are shown in table 1. Overall 44 patients (19%) died during a median 40 months (0-244) of follow-up. First-year and five-year mortality rates were 10% and 30% respectively. Overall mortality was significantly different in AAV subgroups (p< 0.001, Figure). Factors associated with overall and first-year mortalities that were detected with univariate analysis, including disease activity scores (Five Factor Score [FFS] and Birmingham Vasculitis Activity Score [BVAS]), are summarized in table 2. In the multivariate analysis age at diagnosis (Hazard ratio [HR] 1.05, 95 % Confidence interval [CI] 1.01-1.08, p=0.002) and cardiac involvement (HR 6.79, 95 % CI 1.97-23.32, p=0.002) were found to be the independent predictive factors of overall mortality. The same factors were also predictors of the first-year mortality (Age at diagnosis [HR 1.06, 95 % CI 1.02-1.10, p=0.001] and cardiac involvement [HR 5.45, 95 % CI 1.81-16.40, p=0.003]).

Conclusion: Our findings suggest that there could be some survival differences between AAV subgroups and, disease activity scores (both FFS and BVAS at diagnosis) are helpful to predict mortality. However, the age at diagnosis and cardiac involvement seems to be the only significant predictors of first-year and overall mortality in AAV patients.


AAVmortalitytable1

Table 1. Demographic and clinical characteristics of ANCA-associated vasculitis patients


table2AAVmortality

Table 2. The results of Kaplan-Meier survival analysis: Factors associated with overall and first-year mortality


figure_overall_mortality

Figure. The results of the Kaplan-Meier analysis: Overall mortality was significantly different in ANCA-associated vasculitis subgroups

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