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Clinical manifestations and diagnosis of rheumatoid vasculitis

Author
Patrick Whelan, MD, PhD
Section Editor
Eric L Matteson, MD, MPH
Deputy Editor
Paul L Romain, MD

INTRODUCTION

Rheumatoid vasculitis (RV) refers to a destructive, inflammatory process that is centered on the blood vessel wall itself. The condition occurs in patients with longstanding, severe rheumatoid arthritis (RA). RV is often associated with substantial potential morbidity, requires intensive immunosuppressive therapy, and leads to a significantly higher mortality than RA itself. Within a given patient with RV, clinical features of both medium- and small-vessel disease may be found, although rare patients can also present with large-vessel disease. RV leads to necrosis, blood vessel occlusion, and tissue ischemia in a manner that resembles other forms of systemic vasculitis, particularly polyarteritis nodosa (medium-vessel disease) and cutaneous small-vessel vasculitis. (See "Overview of and approach to the vasculitides in adults" and "Clinical manifestations and diagnosis of polyarteritis nodosa in adults" and "Overview of cutaneous small vessel vasculitis".)

Understanding of the precipitating factors for these extremes of blood vessel inflammation in RA is limited, and the relationship of RV to severe RA that is not associated with systemic vasculitis remains uncertain. Although the decreased life expectancy and early cardiovascular mortality in RA are well-recognized, any relationship between atherosclerotic vascular disease leading to thrombosis and RV is dubious, despite many common risk factors for these two RA complications. Atherosclerotic coronary heart disease and its relationship to RA are discussed in detail separately. (See "Coronary artery disease in rheumatoid arthritis: Implications for prevention and management".)

The epidemiology, clinical manifestations, and diagnosis of RV are reviewed in this topic. The etiology, pathogenesis, and treatment of this disorder are discussed separately. (See "Etiology and pathogenesis of rheumatoid vasculitis" and "Treatment of rheumatoid vasculitis".)

EPIDEMIOLOGY AND RISK FACTORS

Rheumatoid vasculitis (RV) is uncommon, with an annual incidence among patients with rheumatoid arthritis (RA) that has decreased since the 1980s to less than 1 percent (see 'Incidence' below). These estimates are consistent with an approximate annual incidence of RA of about 400 per million persons, with a prevalence of about 1 percent among Caucasians, and separate estimates of an annual incidence of clinically significant RV of below four per million. (see "Epidemiology of, risk factors for, and possible causes of rheumatoid arthritis")

Prevalence — Autopsy series of patients with RA suggested that RV affects as many as 25 to 31 percent of patients with RA, although one of the two reports was from 1954 and the other dealt with patients known to have RA who died between 1960 and 1990 [1,2]. Clinically evident disease is much less common. The lifetime occurrence of RV in one large retrospective study of patients in northern Italy seen in the late 1980s was 2 percent [3]. A smaller prospective study found cutaneous vasculitis in 5.4 percent of patients during an 18-month period of observation in the early 1990s [4].

                            

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Literature review current through: Jul 2016. | This topic last updated: Aug 16, 2016.
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