Abstract

ObjectiveEvidences indicate that the risk of stroke is increased in autoimmune rheumatic diseases. This study aimed to investigate the incidence of stroke in patients with systemic necrotizing vasculitis (SNV) using the national health database.MethodsData were obtained from the Korean National Claims database between 2010 and 2018 to identify incident SNV [anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN)] cases. The standardized incidence ratio (SIR) and incidence rate ratio (IRR) were calculated to estimate the risk of stroke in patients with SNV compared to the general population and among disease subgroups. Time-dependent Cox’s regression analysis was performed to identify risk factors for stroke.ResultsAmong 2644 incident SNV cases, 159 patients (6.0%) were affected by stroke. The overall risk of stroke was significantly higher in patients with SNV compared to the general population (SIR 8.42). Stroke event rates were the highest within the first year of SNV diagnosis (67.3%). Among disease subgroups, patients with microscopic polyangiitis (MPA) exhibited higher IRR compared to PAN (adjusted IRR 1.98). In Cox’s hazard analysis, older age and MPA were associated with higher risk of stroke [hazard ratio (HR) 1.05 and 1.88], whereas the administration of cyclophosphamide, azathioprine/mizoribine, methotrexate, and statins were protective in stroke (HR 0.26, 0.34, 0.49, and 0.50, respectively).ConclusionA considerable number of SNV patients experienced stroke, especially in the early phase of disease. Older age and MPA diagnosis were associated with elevated risk of stroke, while the administration of immunosuppressive agents and statins was beneficial in preventing stroke.

Highlights

  • Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare systemic inflammatory disorder causing necrotizing organ injury within small vessels, which is typically associated with myeloperoxidase-ANCA or proteinase 3-ANCA [1]

  • Patients were diagnosed with systemic necrotizing vasculitis (SNV) when they were first registered with the corresponding International Classification of Diseases (ICD)-10 codes for AAV or polyarteritis nodosa (PAN) in a general or tertiary hospital and were prescribed with glucocorticoids during the followup period [19]

  • A total of 2984 AAV and PAN cases were found in the Health Insurance and Review Agency (HIRA) database between 2010 and 2018

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Summary

Introduction

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare systemic inflammatory disorder causing necrotizing organ injury within small vessels, which is typically associated with myeloperoxidase-ANCA or proteinase 3-ANCA [1]. A growing body of evidence has suggested that chronic inflammation and high degree of inflammation are crucial factors associated with increased vascular thrombosis in autoimmune rheumatic diseases by triggering the coagulation cascade [11, 12]. In this context, previous studies have reported an increased risk of stroke in patients with large vessel vasculitis, such as Takayasu arteritis (TA) and giant cell arteritis (GCA). This study was conducted to investigate the incidence of stroke in SNV using a nationwide database

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