A 30-Year-Old Diagnosis Never Treated: A Case

Stephen Paget, MD


June 08, 2017

Editorial Collaboration

Medscape &


Takayasu arteritis is a systemic granulomatous large vessel arteritis that is also known as Takayasu disease, aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease. Takayasu arteritis can cause massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-aged women of Asian descent, though anyone can be affected.

The first case of Takayasu arteritis was described in 1908 by Japanese ophthalmologist Mikito Takayasu.[1] He described a peculiar "wreathlike" appearance of the blood vessels in the retina that could cause hemorrhage and is seen mainly in Japanese patients. It primarily affects the aorta and its branches, as well as the pulmonary arteries. Women are about eight to nine times more likely to be affected than men.

Some believe that a continuum of granulomatous vasculitides exists between younger patients who develop Takayasu arteritis and older patients who have giant cell arteritis and that any clinical differences reflect the vascular impact of older patients' comorbidities, including diabetes, lipid disorders, and hypertension. The tremendous overlap in angiography-defined vascular bed involvement supports this concept. There are many clinical overlaps including systemic manifestations and the clinical aftermath of vascular occlusions; common treatment options include steroids, immunosuppressive drugs, and interleukin (IL)-6 blockade.

Does this patient have Takayasu arteritis? Yes, supported by the early onset of vascular occlusions, eye hemorrhage, Japanese ethnicity, and the vascular abnormalities found on imaging and supported by the clinical examination.

Does everyone with Takayasu arteritis need to be treated with steroids at some time during the course of the disease? Steroids are commonly used in patients with Takayasu arteritis, usually in the setting of active disease manifested by one or more of the following: advancing, critical, and widespread vascular occlusion with visceral or limb ischemia; constitutional symptoms such as fatigue, renovascular hypertension, and elevated ESR; or elevated C-reactive protein with anemia and thrombocytosis.

Typically, high-dose steroids are used initially, but their cumulative side effects must be appreciated, and it is not uncommon that immunosuppressive drugs are used as disease-controlling and steroid-sparing medications. Biologic drugs that block IL-6 are being studied in Takayasu arteritis and giant cell arteritis with impressive results, and they will likely become the initial go-to drugs with or without steroids.

If this patient had been treated at initial diagnosis, perhaps her current vascular changes and eye hemorrhage could have been avoided. However, it does not make sense to treat someone who has shown no interval vascular changes defined by imaging; has no systemic complaints, limb symptoms or findings, or visceral dysfunction; and now has a normal ESR and C-reactive protein.

What about the patient's immune abnormalities of anti-Ro and antithyroid antibodies? Does that change the diagnostic possibilities or therapeutic options? No. They reflect immune activation. Certainly this patient has no clinical support for a diagnosis of Sjogren syndrome or lupus, diseases that may affect smaller vessels but do not present in this fashion.


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