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Arteritis ACR 2021

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2 Key Points and Definitions ` Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are systemic vasculitides that primarily affect large and medium-sized vessels. • GCA can present with both cranial and extracranial manifestations and is more common in individuals of Northern European descent who are older than 50 years of age. • TAK is more common in younger women and causes granulomatous inflammation of the aorta and its branches. Table 1. Definitions of Selected Terms Used in the Recommendations and Ungraded Position Statements for GCA and TAK Term Definition Disease States Suspected disease Clinical symptoms or signs suggestive of GCA/TAK and not explained by other conditions Active disease New, persistent, or worsening clinical signs and/or symptoms attributed to GCA/TAK and not related to prior damage Severe disease Vasculitis with life-/organ-threatening manifestations (e.g., vision loss, cerebrovascular ischemia, cardiac ischemia, limb ischemia) Non-severe disease Vasculitis without life-/organ-threatening manifestations (e.g., constitutional symptoms, headache, jaw claudication, symptoms of polymyalgia rheumatica) Remission Absence of clinical signs or symptoms attributed to active GCA/TAK, on or off of immunosuppressive therapy Refractory disease Persistent active disease despite an appropriate course of immunosuppressive therapy Relapse Recurrence of active disease following a period of remission Cranial ischemia Visual and neurological involvement including amaurosis fugax, vision loss, and stroke Disease Assessments Clinical monitoring Assessing for clinical signs and symptoms of active disease, obtaining 4 extremity blood pressures, and obtaining clinical labs including inflammatory markers Inflammatory markers Erythrocyte sedimentation rate, C-reactive protein Non-invasive imaging Computed tomography angiogram, magnetic resonance angiogram, positron emission tomography scan, vascular ultrasound, magnetic resonance imaging of temporal and scalp arteries Invasive imaging Conventional catheter-based angiogram

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