Background/Purpose: Previous studies have documented an increased risk of venous thromboembolic events in patients with antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV) as compared to healthy subjects. However, only a limited number of studies have analyzed the prevalence of thrombosis in eosinophilic granulomatosis with polyangiitis (EGPA).Aim of the study was to determine the frequency of venous thromboembolic events (VTE) in a single center cohort of patients with EGPA and to describe its relation with disease clinical manifestations and activity.

Methods: Patients diagnosed with EGPA (1990 ACR classification criteria) from 1994 to 2014 were included in the study. Data were retrospectively retrieved from patients’ charts, including gender, demographic data, cumulative clinical features, ANCA status and BVAS at the baseline. In patients with VTE (i.e deep venous thrombosis and/or pulmonary embolism), EGPA  characteristics and disease activity at the time of VTE occurrence were also collected. Categorical variables were compared using Fisher’s exact test; continuous variables were compared using Student’s t-test. A 2-tailed value of p < 0.05 was taken to indicate statistical significance. 

Results: The systematic search of our database identified 89 patients, among whom 51 were included in the study. Median (IQR) age at the EGPA diagnosis was 59 (41-62.2) years and their M:F sex ratio was 1.04. During a median (IQR) follow-up of 72.2 (14.8-135.2) months, 6/51 (11.8%) EGPA patients presented at least one VTE. One patient presented 4 recurrent thromboses despite anticoagulant therapy; overall, then, 9 VTE were recorded. Lower-limb deep venous thromboses (DVTs) were the most common VTE manifestations, representing the 89% (8/9) of all the VTE in our series. One patients developed also a pulmonary embolism, while another presented a cardiac intra-ventricular thrombus. Six (67%) VTE occurred within 6 months before the EGPA diagnosis. The patient with recurrent thromboses presented 2 DVTs before the EGPA diagnosis and 2 further DVTs within 15 months after the EGPA diagnosis. The patient with the pulmonary embolism, developed the VTE 13 years after EGPA diagnosis concomitantly with a femoral neck fracture. According to our analysis, factors associated with the occurrence of VTE were renal involvement (p=0.01), nephrotic range proteinuria >3 g/24 h (p = 0.03) and a FFS>1 (p=0.03). No differences were observed regarding theANCA status and the BVAS score at the diagnosis between the groups.

Conclusion: The results of this study confirm a higher risk of VTE in patients with EGPA. The pathogenesis of thrombosis in EGPA calls for further studies.

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