Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ KEY FEATURES +++ ESSENTIALS OF DIAGNOSIS ++ A severe inflammatory vascular disorder involving the aorta, its major branches, and the pulmonary artery Predominantly found in Asian women < 40 years old Evidence of diffuse vascular disease (coronary, cerebral, peripheral), especially involving the upper extremities (pulseless disease) Elevated erythrocyte sedimentation rate +++ GENERAL CONSIDERATIONS ++ An uncommon vasculitis of the thoracic aorta and its major branches Aneurysms and occlusions found Half of those affected have a systemic illness More common in Asian women aged 25–50 years +++ CLINICAL PRESENTATION +++ SYMPTOMS AND SIGNS ++ Fever, lymphadenopathy, arthralgias, or arthritis Weakness of the arms or, less commonly, the legs Chest or abdominal pain Many are asymptomatic +++ PHYSICAL EXAM FINDINGS ++ Absent or diminished pulses in the upper extremities +++ DIFFERENTIAL DIAGNOSIS ++ Diffuse atherosclerosis Other vasculitides (eg, Buerger’s disease) +++ DIAGNOSTIC EVALUATION +++ LABORATORY TESTS ++ Elevated erythrocyte sedimentation rate +++ IMAGING STUDIES ++ Magnetic resonance or CT angiography is usually diagnostic +++ DIAGNOSTIC PROCEDURES ++ Invasive angiography is the reference standard +++ TREATMENT +++ CARDIOLOGY REFERRAL ++ Suspected cardiac disease Severe hypertension +++ HOSPITALIZATION CRITERIA ++ Acute coronary syndromes +++ MEDICATIONS ++ Corticosteroid: prednisone 5–60 mg PO daily +++ THERAPEUTIC PROCEDURES ++ Percutaneous transluminal revascularization procedures +++ SURGERY ++ Vascular surgery for occlusion not amenable to angioplasty +++ MONITORING ++ ECG monitoring in hospital as appropriate +++ DIET AND ACTIVITY ++ Restricted activity until disease controlled +++ ONGOING MANAGEMENT +++ HOSPITAL DISCHARGE CRITERIA ++ Resolution of problem Successful revascularization +++ FOLLOW-UP ++ Depends on the severity and extent of disease +++ COMPLICATIONS ++ Hypertension due to renal artery involvement Myocardial infarction Stroke Intestinal ischemia Critical limb ischemia +++ PROGNOSIS ++ Generally good with treatment Over 10 years, half of patients will have a relapse or a vascular complication Mortality rate is low +++ RESOURCES +++ PRACTICE GUIDELINES ++ The diagnosis of this disease is difficult because the clinical presentation depends on the location and severity of the aortic branch lesions Once the diagnosis is made, many respond well to anti-inflammatory therapy or revascularization +++ REFERENCE + +Comarmond C, Biard ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.