Aortitis Differential Diagnoses

Updated: Dec 18, 2014
  • Author: Masato Okada, MD, FACP, FACR, FAAAAI; Chief Editor: Uchechukwu Sampson, MBBS, MPH, MBA, MSc  more...
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DDx

Diagnostic Considerations

Scalenus anticus syndrome and chronic regional pain syndrome/sympathetic dysfunction can cause asymmetry of pulses. With scalenus anticus, the neck muscles have focal tenderness, and position of neck and arm can change the pulse strength. With sympathetic dysfunction, a thermal map may identify >1°C difference between the arms exacerbated by exposure to cold or wind, and bone scan may show focal activity in small bones of the wrist. In advanced cases, asymmetry of nail and hair growth may be noted.

To make the diagnosis of arteritis at the early stage, a high index of suspicion for vasculitis is necessary, especially in patients with nonspecific inflammatory manifestations such as fatigue, malaise, joint aches, and low-grade fever but no specific clinical picture of other autoimmune diseases or infections. High index of suspicion is essential, not just in young women—recently, a highly esteemed senior American rabbi died from severe vasculitis because the correct diagnosis was not considered in time. Carotidynia presenting as neck pain can be an important clue. C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR) often are elevated, which supports the presence of an ongoing inflammatory process.

In the prepulseless phase, vascular changes may be too subtle to cause obvious extremity ischemia or arm claudication. With awareness of the disease, however, careful examination of the arteries at this stage may lead to the detection of reduction in one or more pulses; differences in blood pressure between the arms; or bruits over the neck, supraclavicular areas, axillae, or abdomen.

American College of Rheumatology published the classification criteria of Takayasu arteritis as follows: (1) age 40 years or younger at disease onset; (2) claudication of extremities; (3) decreased brachial artery pulse; (4) systolic blood pressure difference of greater than 10 mm Hg between arms; (5) bruit over subclavian arteries or abdominal aorta; and (6) arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities that is not caused by arteriosclerosis, fibromuscular dysplasia, or similar causes.

The presence of at least 3 criteria reportedly yields a sensitivity of 90.5% and a specificity of 97.8%, although, being a clinical syndrome, the estimate of sensitivity is questionable.

Fibromuscular dysplasia, Ehlers-Danlos syndrome, and Marfan syndrome are associated with noninflammatory lesions that can mimic arteritis in angiographic findings. Fibromuscular dysplasia most commonly affects the renal arteries and leads to stenotic changes. Other sites, including carotid arteries and mesenteric arteries, can be involved, but the lesion usually is more focal. Ehlers-Danlos syndrome and Marfan syndrome also cause aortic aneurysm and aortic root dilatation, respectively. Their systemic manifestations and lack of typical clinical symptoms of Takayasu arteritis may help differentiation.

Infectious diseases, including syphilis, tuberculosis, and mycosis, should be excluded as causes of aortic aneurysms. The progression of these infections can be indolent and, unless they are considered as differential diagnoses, missing them is easy. The clinician should keep in mind that, after the infection is stopped, he or she still may have to treat consequent inflammatory processes.

Other systemic vasculitides and granulomatous diseases, such as giant cell (temporal) arteritis, sarcoidosis, SLE, and Behçet disease, can manifest with aortic lesions. Age younger than 40 years at onset of disease is the single most discriminatory variable between Takayasu arteritis and giant cell (temporal) arteritis.

Important considerations

Elevations of acute phase reactants, such as ESR and CRP level, not only are nonspecific but also are insensitive markers of the activity of Takayasu arteritis. False reassurance to patients based solely on these values can lead to medicolegal troubles. Careful monitoring of the disease with regular follow-up and appropriate imaging studies (including CT scan or MRI if necessary) is recommended.

Patients should be informed about the possibility of late complications (eg, aortic aneurysms), even in cases of successful treatment of patients whose disease is in early stages.

Failure to diagnose aortitis often arises from the fact that the clinician fails to consider it in the differential diagnosis.

Pregnancy has not been shown to alter prognosis significantly, although hypertension and heart failure can worsen during the third trimester. The disease does not seem to be associated with increased incidence of neonatal death.

Differential Diagnoses