Background/Purpose:

The incidence of first-time venous thromboembolic events (VTE) is high in granulomatosis with polyangiitis (GPA). The incidence of recurrent VTE in these patients has not been studied. The risk of VTE recurrence has been reported in other groups of patients and it is important as it affects recommendations for extended anticoagulation therapy. The aim of this study is to determine the incidence rate of recurrent VTE in GPA.

Methods:

A retrospective chart review was performed in patients with GPA and at least one VTE from a single Vasculitis Center from 2002 to 2016. Our inclusion criteria: 1) 1990 ACR criteria or 2012 Revised International Chapel Hill nomenclature for GPA, 2) ≥ 2 follow-up visits in our center, 3) ≥ 1 VTE documented during the study period, 4) VTE occurrence after diagnosis of GPA or within three months before GPA diagnosis. Kaplan-Meier was used to estimate second VTE event-free survival rates. Disease (GPA) activity was defined as BVAS/WG score of ≥ 1.

Results:

84 out of 137 patients with GPA and at least one VTE met inclusion criteria. Median age at the time of GPA and first VTE was 56 and 57 respectively. Majority were Caucasian (97.6%) and male gender (56%). Incidence rate for second VTE was 8.4 events per 100 patient years (95% CI 5.7-12.3) over a median duration of 2.4 years (0.6-5.5 years) observation period. The cumulative recurrence rates at 3, 6, 12 months, 3 and 5 years were 9.7%, 13.8%, 15.1%, 27.1 %, and 27.1% respectively. Median time-to-second event was 10.6 years, shorter for patients with BVAS at diagnosis ≥ 15 when compared to BVAS < 15 group (7.5 vs 11.3 years -p=0.0042 by Log-Rank test-). 89.3% of first VTE and 57.7% of second VTE occurred when disease was active (p< 0.001). Median BVAS at first event was 8.5 vs 4.0 at second event (p=0.030). Male and upper extremity deep vein thrombosis were associated with higher risk of recurrence (Odds ratios: 2.9 [95% CI: 1.1-8.0] and 6.0 [95%CI: 1.6-22.3] respectively). Lung involvement at GPA diagnosis was associated with lower risk for recurrence (odds ratio: 0.33 [95%CI: 0.12 – 0.95]). ANCA positivity was not associated with an increased risk of recurrence.

Conclusion:

GPA patients have a high rate of VTE recurrence compared to the general population with unprovoked VTE (8.4 vs 3.9 recurrent events per 100 patient years) (Kyrle PA, et al. Lancet. 2010). Recurrent events were not always related to clinically apparent active disease. Our results suggest that VTE in GPA is a recurrent disease, more so during the first three years after first event. Prospective studies are needed to address adequate duration of anticoagulation in this population.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/recurrent-venous-thromboembolic-events-in-granulomatosis-with-polyangiitis-patients/