The American College of Rheumatology classification criteria underperforms in classifying patients with LV-GCA. A retrospective study by Muratore et al [1] demonstrated that American College of Rheumatology criteria are inadequate to classify patients with LV-GCA. Therefore, patients with suspected GCA require supplemental imaging studies in addition to temporal artery US or biopsy [2]. In this regard, the trial of tocilizumab for GCA, which is a large randomized controlled trial in GCA, included patients with imaging-confirmed LV-GCA who did not meet the American College of Rheumatology criteria [3]. Interestingly, patients with LV-GCA compared to other patients with GCA, present less frequently with jaw claudication or ischemic symptoms and have a higher incidence of relapse, have greater cumulative glucocorticoid exposure, and are more frequently treated with steroid-sparing agents, such as the interleukin-6-receptor blocker tocilizumab [1]. Polymyalgia rheumatica (PMR) is a disorder in the same disease spectrum as GCA and can be found in association with GCA or as an isolated phenomenon [4]. Reprint requests to: publications@acr.org Noncerebral Vasculitis from asymptomatic presentations to nonspecific constitutional symptoms and major ischemic events. Although the histopathology of TAK shares similarities with that of GCA, biopsy material from the large arteries is rarely obtained in TAK. The diagnosis of TAK typically requires a combination of physical examination, laboratory findings, and imaging findings. Variable-vessel vasculitis subtypes such as Cogan or Behcet disease can involve the large vessels, particularly the aorta. Although the literature is limited on imaging features of Cogan or Behcet vasculitis, case reports and series have demonstrated similarities with LVV. Because of the limitation of the peer-reviewed literature, the focus of this manuscript will be on LVV. MVV predominantly involves medium-sized arteries, although arteries of any size can be involved.
Noncerebral Vasculitis. The American College of Rheumatology classification criteria underperforms in classifying patients with LV-GCA. A retrospective study by Muratore et al [1] demonstrated that American College of Rheumatology criteria are inadequate to classify patients with LV-GCA. Therefore, patients with suspected GCA require supplemental imaging studies in addition to temporal artery US or biopsy [2]. In this regard, the trial of tocilizumab for GCA, which is a large randomized controlled trial in GCA, included patients with imaging-confirmed LV-GCA who did not meet the American College of Rheumatology criteria [3]. Interestingly, patients with LV-GCA compared to other patients with GCA, present less frequently with jaw claudication or ischemic symptoms and have a higher incidence of relapse, have greater cumulative glucocorticoid exposure, and are more frequently treated with steroid-sparing agents, such as the interleukin-6-receptor blocker tocilizumab [1]. Polymyalgia rheumatica (PMR) is a disorder in the same disease spectrum as GCA and can be found in association with GCA or as an isolated phenomenon [4]. Reprint requests to: publications@acr.org Noncerebral Vasculitis from asymptomatic presentations to nonspecific constitutional symptoms and major ischemic events. Although the histopathology of TAK shares similarities with that of GCA, biopsy material from the large arteries is rarely obtained in TAK. The diagnosis of TAK typically requires a combination of physical examination, laboratory findings, and imaging findings. Variable-vessel vasculitis subtypes such as Cogan or Behcet disease can involve the large vessels, particularly the aorta. Although the literature is limited on imaging features of Cogan or Behcet vasculitis, case reports and series have demonstrated similarities with LVV. Because of the limitation of the peer-reviewed literature, the focus of this manuscript will be on LVV. MVV predominantly involves medium-sized arteries, although arteries of any size can be involved.