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: vasculitis and ANCA

Session Information

Date: Monday, November 11, 2019

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

« Back to 2019 ACR/ARP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/factors-associated-with-overall-and-first-year-mortality-in-turkish-patients-with-anca-associated-vasculitides-retrospective-multicentre-trial/