OBJECTIVE: To investigate the occurrence of venous thromboembolic events (VTE) in a large cohort of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) across the European Union, Turkey, Russia, UK and North America. METHODS: Patients with a definite diagnosis of AAV who were followed for at least 3 months and had sufficient documentation were included. Data on VTE, including either deep vein thrombosis or pulmonary embolism, were collected retrospectively from tertiary vasculitis centres. Univariate and multivariate regression models were used to estimate odds ratios (ORs) and 95% CIs. RESULTS: Over a median follow-up of 63 (interquartile range: 29, 101) months, VTE occurred in 278 (9.7%) of 2869 AAV patients with a similar frequency across different countries (from 6.3% to 13.7%), and AAV subtype [granulomatosis with polyangiitis: 9.8% (95% CI: 8.3, 11.6%); microscopic polyangiitis: 9.6% (95% CI: 7.9, 11.4%); and eosinophilic granulomatosis with polyangiitis: 9.8% (95% CI: 7.0, 13.3%)]. Most VTE (65.6%) were reported in the first-year post-diagnosis. Multiple factor logistic regression analysis adjusted for sex and age showed that skin (OR 1.71, 95% CI: 1.01, 2.92), pulmonary (OR 1.78, 95% CI: 1.04, 3.14) and kidney [eGFR 15-60 ml/min/1.73 m2, OR 2.86 (95% CI: 1.27, 6.47); eGFR <15 ml/min/1.73 m2, OR 6.71 (95% CI: 2.94, 15.33)] involvement were independent variables associated with a higher occurrence of VTE. CONCLUSION: Two-thirds of VTE occurred during the initial phase of active disease. We confirmed previous findings from smaller studies that a decrease in kidney function, skin involvement and pulmonary disease are independently associated with VTE.

Beaumont Hospital Dublin Ireland

Department of Clinical Sciences Lund Nephrology Lund University Lund Sweden

Department of Internal Medicine 4 Medical University Innsbruck Innsbruck Austria

Department of Internal Medicine Division of Rheumatology Marmara University School of Medicine Istanbul Turkey

Department of Internal Medicine National Referral Center for Rare Systemic and Autoimmune Diseases Hôpital Cochin Paris France

Department of Medicine Centre for Inflammatory Disease Imperial College London London UK

Department of Medicine University of Cambridge Cambridge UK

Department of Nephrology 1st Faculty of Medicine Charles University and General University Hospital Prague Prague Czech Republic

Department of Nephrology and Dialysis Dubrava University Hospital Zagreb Croatia

Department of Pathology Leiden University Medical Center Leiden The Netherlands

Department of Rheumatology Skåne University Hospital Lund Sweden

Division of Nephrology Department of Medicine Johns Hopkins University Baltimore MD USA

Faculty of Medicine Lomonosov Moscow State University Moscow Russia

Irish Centre for Vascular Biology Tallaght University Hospital

Tareev Clinic of Internal Diseases Sechenov 1st Moscow State Medical University

Trinity Health Kidney Centre Trinity Translational Medicine Institute

Vasculitis and Lupus Clinic Addenbrooke's Hospital Cambridge University Hospitals Cambridge UK

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