Chapter 49: Vasculitis and Other Arteriopathies

INTRODUCTION

Vasculitis is a group of inflammatory diseases affecting blood vessels of all calibers. Vasculitis is classified based on the involved-vessel size: small, medium, and large vessels vasculitis. The clinical presentation of vasculitis is variable, and it helps to differentiate between the different vasculitis conditions. Also age, race, organ system involvement, histopathology, and serology can facilitate further classification of vasculitis. Management of vasculitis frequently requires a multispecialty approach involving rheumatology, nephrology, neurology, vascular surgery, and cardiology making coordination of care essential. Historically, vasculitis has evolved from an illness with high morbidity and mortality to one of challenging management with recurrent flares and a goal of remission.

For the purposes of this chapter, we will focus on large and medium vasculitis which have surgical applications and means of intervention. Large vessels function as transportation highways delivering oxygen-rich blood and carrying deoxygenated waste to and from vital organs. Medium vessels are utilized in organs where blood flow is a key element to homeostasis and overall function. Small vessel, while acting as part of the delivery system, have few surgical interventions and are best managed with medical therapy via immunosuppression, but it is important for vascular surgery to identify these conditions as often the vasculitis lesions may present with skin ulceration or palpable, nonblanching purpuric skin lesions (Figure 49-1).

It is imperative to differentiate inflammatory conditions of the vascular wall from infectious conditions, as treatment and outcome of management is completely different. For instance, aortic aneurysms have been linked to infections related to syphilis, aspergillus, or rickettsial.¹ Emergent endovascular repairs are more likely to be complicated by graft infection than elective procedures, but similar rates of postoperative graft infection were noted in both thoracic and abdominal aneurysm location.² Stenting of infectious aneurysms is controversial and beyond the scope of this chapter. On the other hand, some infection can trigger systemic vasculitis. For instance, hepatitis C infection is often associated with cryoglobulinemia. Endocarditis can trigger a leukocytoclastic small vessel vasculitis disease that is similar in histopathology and clinical appearance of systemic vasculitis (Figure 49-2). Identifying these vasculitis mimickers is imperative as it will have significant implications in terms of treatment strategies (antibiotics vs immunosuppressive therapy). Other vasculitis mimickers include drug-induced vasculopathy, such as in the case of cocaine-induced vasculitis, or the result of malignant, atherosclerotic/ischemic, or thromboembolic disease. This should be considered in clinical context based on individual patient risk factors that guide laboratory and differential diagnosis testing to determine the root concern.