Diagnostic Considerations
Takayasu arteritis is rare and difficult to diagnose. Initially, symptoms are vague; the disease may progress considerably until an angiogram is performed. Conditions to consider in the differential diagnosis of Takayasu arteritis include the following:
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Cerebral aneurysms
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IgG4-related disease [30]
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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
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Intracranial hemorrhage
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Lacunar syndromes
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Neurofibromatosis type 1
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Neurofibromatosis type 2
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Polyarteritis nodosa
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Sarcoidosis and neuropathy
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Subarachnoid hemorrhage
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Syncope and related paroxysmal spells
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Fibromuscular dysplasia
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Spondyloarthropathies
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Thromboembolism
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Traumatic stenosis
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Radiation fibrosis
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Ergotism
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Ehlers-Danlos syndrome
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Marfan syndrome
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Neurofibromatosis
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Syphilis
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Cogan syndrome
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Dilated cardiomyopathy
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Erythema nodosum
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Relapsing polychondritis
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Amaurosis fugax
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Ocular pathology
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Other arteritides
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Other large vessel diseases (eg, neurofibromatosis of the abdominal aorta)
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Carotid disease and stroke
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Reversible posterior leukoencephalopathy syndrome
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Common variable immunodeficiency syndrome
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Keratouveitis
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Posterior scleritis
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Polymyalgia rheumatica
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Subclavian steal syndrome
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Raynaud phenomenon
Case reports describe patients who meet the diagnostic criteria for both Takayasu arteritis and giant cell (temporal) arteritis, although subtle differences exist in the distribution of arterial involvement in the two diseases. [31, 32] Some authors have advanced the notion that the two disorders may represent a spectrum of a single disease, with the manifestations influenced by hormonal, immunologic and vascular factors. [32] Virological and pathological findings and response to antiviral treatment in one patient support a possible etiologic role for varicella-zoster virus (VZV). [31]
Differential Diagnoses
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Atherosclerosis
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Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis)
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IgG4-related disease
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Takayasu arteritis. Complete occlusion of the left common carotid artery in a 48-year-old woman with Takayasu disease. Also note narrowing of the origin of the right subclavian artery and a narrowed small vessel with subsequent aneurysmal dilatation on the right side. Image courtesy of Robert Cirillo, MD.
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Takayasu arteritis. Characteristic long, tapered narrowing of the distal aorta and iliac vessels. Image courtesy of Robert Cirillo, MD.
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Takayasu arteritis. Image obtained in the same patient as in Image 2 reveals narrowing of the proximal descending aorta and right brachiocephalic artery. Image courtesy of Robert Cirillo, MD.
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Takayasu arteritis. Aortogram of a 15-year-old girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery. Image courtesy of Christine Hom, MD.
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Takayasu arteritis. MRI of thorax of 15-year-old girl with Takayasu arteritis. Note aneurysms of descending aorta. Image courtesy of Christine Hom, MD.
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Takayasu arteritis. Coronal MRI of abdomen of 15-year-old girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys. Image courtesy of Christine Hom, MD.
