Presentation: Venous Thromboembolic Events in Patients with Different Systemic Necrotizing Vasculitides: Systematic Study on the French Vasculitis Study Group (FVSG) Patient Cohort (2007)

2013 Venous Thromboembolic Events in Patients with Different Systemic Necrotizing Vasculitides: Systematic Study on the French Vasculitis Study Group (FVSG) Patient Cohort

PURPOSE: Venous thromboembolic event (VTE) frequency was reported to be higher for Wegener’s granulomatosis (WG) patients than the general population, but it has not been assessed in other systemic necrotizing vasculitides (SNV). This study aimed to determine VTE frequency in ANCA-associated systemic vasculitides (AASV) and polyateritis nodosa (PAN), and to identify the characteristics of the affected patients.
METHODS: Retrospective systematic analysis of 1130 patients diagnosed between 1985 and 2006 with microscopic polyangiitis (MPA, n=236), Churg-Strauss syndrome (CSS, n=232), WG (n=377) or PAN (HBV-related or not, n=285), satisfying the ACR classification and/or Chapel Hill diagnostic criteria and entered in the FVSG database. Patients’ VTE were considered when they occurred ≤12 mo before SNV diagnosis or thereafter.
RESULTS: During a mean follow-up of 58 ± 46.3 mo, 83 VTE occurred in 75 (6.6%) patients, particularly those affected with AASV (all AASV, 7.6%; GW, 8.0%; CSS, 8.2%; MPA, 7.6%) compared to PAN (2.8%; p=0.003). Mean age at VTE diagnosis was 58.7 ± 21.9 yr and median SNV-diagnosis-VTE interval was 5.7 mo [-3;+139]. Four patients suffered VTE ≤12 mo before SNV diagnosis. The first VTE were diagnosed as phlebitis for 47 (62.7%) patients, pulmonary embolism for 16 (21.3%) and both for 12 (16%). At the time of VTE, mean BVAS was 12.6 ± 10.5 and C-reactive protein 67 ± 80.9 mg/l. VTE patients were predominantly males (M/F 51/24; p=0.03) and, compared to non-VTE patients, had more frequent proteinuria >3 g/24 h (11.1% vs 4%; p=0.02) and/or stroke with motor deficit (4% vs 1%; p=0.04), but less frequent peripheral lower limb neuropathy (24% vs 43.5%; p<0.001). History of cancer or recent surgery was not associated with VTE. Among all the ANCA-positive patients, VTE occurred in 8% of those with MPO-ANCA and 10.1% of those with PR3-ANCA (p=0.45).
CONCLUSIONS: VTE occurred more frequently in AASV than PAN patients, predominantly during the first months after diagnosis, i.e. during active disease, and particularly in those with nephrotic syndrome and/or stroke. Peripheral neuropathy was not a risk factor significantly associated with VTE. These results suggest that VTE is a specific complication of small-vessel SNV, and not only of WG or PR3-ANCA-positive patients.

 Y. Allenbach, None; C. Pagnoux, None; R. Seror, None; L. Teixeira, None; P. Guilpain, None; L. Guillevin, None.