Takayasu arteritis in Adult
No definitive cause of Takayasu arteritis has been identified, but it is thought to be an immunoglobulin G (IgG)-mediated autoimmune vasculitis, perhaps triggered by a cross-reacting infectious agent.
The disease typically presents in the second and third decade of life in females (10:1) of Asian descent. It is most prevalent in Japan, Southeast Asia, India, and Mexico but has been found worldwide and in both sexes. Incidence is approximately 2.5 per million.
The disease has 2 phases that may overlap: a "pre-pulseless" and a pulseless phase. In the first phase, a constellation of nonspecific constitutional symptoms and signs including fever, night sweats, malaise, weight loss, arthralgia, myalgia, and mild anemia may be seen. In the second phase, the characteristic sequelae of large-vessel stenosis occur: upper extremity claudication, diminished brachial pulses, and/or differences in blood pressure between contralateral or ipsilateral extremities.
For diagnosis, the American College of Rheumatology requires 3 of 6 criteria:
- Age at disease onset is 40 years or younger
- Claudication of extremities
- Decreased brachial artery pulse
- Blood pressure difference of > 10 mm Hg between arms
- Bruit over subclavian artery or aorta
- Arteriogram abnormality
Diagnosis during the pre-pulseless phase is difficult because of the nonspecific nature of symptoms and laboratory abnormalities. Diagnosis is usually made during the pulseless phase when clinical criteria, as per above, are met. Angiography is essential to confirm diagnosis.
Constitutional symptoms are typically intermittent, and vascular complications are generally progressive, but the prognosis is generally good, with 5-year survival reports of 90%-94%.
Pediatric patient considerations: Takayasu arteritis in children rarely presents with pulselessness, claudication, or bruits. It is most frequently identified during evaluation for hypertension, heart failure, and neurologic symptoms.
M31.4 – Aortic arch syndrome [Takayasu]
359789008 – Takayasu's disease
- Giant cell arteritis – typically seen in older patients (mean age 72), rarely causes hypertension, claudication, or bruits; ocular involvement is common
- Sarcoidosis – look for perihilar lymphadenopathy and pulmonary granulomas
- Behçet disease – look for aphthous ulcers
- Buerger disease – look for digital ulcers and ischemic changes in a patient with a history of tobacco use
- Sweet syndrome
- Mycotic aneurism – rule out sepsis or endocarditis with blood culture
- Tuberculosis – check purified protein derivative (PPD) status
- Tertiary syphilis – check for fluorescent treponemal antibody (about one-fourth of rapid plasma reagin [RPR] is false negative in tertiary syphilis)
- Leprosy – look for hypopigmented or erythematous macules with loss of sensation, thickened peripheral nerves, and acid-fast bacilli on skin smear or biopsy
- Congenital malformation – aortic coarctation or middle aortic syndrome; unlikely to have constitutional symptoms
- Marfan syndrome – look for arachnodactyly, pectus excavatum or carinatum, and arm span greater than height; family history
- Neurofibromatosis – look for neurofibromas, café au lait spots, ocular Lisch nodules; family history (autosomal dominant)
- Ehlers-Danlos syndrome – look for fragile skin, easy bruising, joint hyperextendability, frequent dislocations
- Fibromuscular dysplasia – look for "string of beads" with angiography; family history (autosomal dominant)
- Postradiation therapy – may cause large-vessel stenosis
giant cell arteritis may be indistinguishable from Takayasu arteritis; however, initial treatment for both is nearly identical.