Abstract

Objective To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Methods Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN) and granulomatosis with polyangiitis (GPA). Incident acute myocardial infarctions (AMIs) and cerebrovascular accidents (CVAs) after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data. These were compared to rates of a general population comparator group. The incidences of comorbidities (type 2 diabetes mellitus, dyslipidemia, and hypertension) were also collected. Results Among the 626 patients identified with vasculitis, 19.7% had PAN, 2.9% had Kawasaki disease, 23.8% had GPA, 52.4% had GCA, and 1.3% had Takayasu arteritis. The AMI rate was substantially higher in males aged 18–44 with PAN, with rates up to 268.1 events per 10,000 patient years [95% CI 67.1–1070.2], approximately 30 times that in the age- and sex-matched control group. The CVA rate was also substantially higher, particularly in adults aged 45–65. Patients with vasculitis had elevated incidences of diabetes, dyslipidemia, and hypertension versus the general population. Conclusion Atherothrombotic rates were elevated in patients identified as having primary systemic vasculitis. While incident rates of cardiovascular comorbidities were also increased, the substantial elevation in AMIs seen in young adults suggests a disease-specific component which requires further investigation.

Highlights

  • Primary systemic vasculitis encompasses a group of disorders characterized by inflammation and necrosis of blood vessels which leads to end-organ damage

  • Of importance is the potentially heightened degree of atherosclerotic disease seen in several types of systemic vasculitis including giant cell arteritis [1, 2], Kawasaki’s disease [3], granulomatosis with polyangiitis (GPA) [4,5,6,7,8], microscopic polyangiitis (MPA) [6, 7], and polyarteritis nodosa (PAN) [7, 9,10,11,12,13]

  • 626 patients diagnosed with primary systemic vasculitis were identified within the observation period, of which 19.7% had PAN and 23.8% had GPA, 52.4% had GCA, 2.9% had Kawasaki disease, and 1.3% had Takayasu arteritis

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Summary

Introduction

Primary systemic vasculitis encompasses a group of disorders characterized by inflammation and necrosis of blood vessels which leads to end-organ damage. The low incidence of systemic vasculitis in the general population makes the creation of large prospective clinical cohorts difficult. Among these patients, investigating relatively rare outcomes such as acute myocardial infarction (AMI) and cerebrovascular accidents (CVA) poses an even greater challenge. Administrative health databases have been used to estimate incidence of several rheumatologic diseases and monitor a variety of associated health outcomes. We estimate the incidence of clinically apparent AMI and CVA from a large administrative database in subjects with systemic vasculitis, further divided into PAN and GPA. We evaluate the incidence of cardiovascular risk factors within these groups in the International Journal of Rheumatology period after being diagnosed with vasculitis, compared to the incidence in the general population

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