How is Takayasu arteritis staged?

Updated: Nov 14, 2018
  • Author: Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond, MD  more...
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Takayasu arteritis progresses through 3 stages. Thus, symptoms that clinicians encounter depend on how soon the patient presents; most patients present late, delaying the diagnosis. In actual practice, however, most patients do not fall readily into such groupings, and this 3-stage scheme is an oversimplification of the complex clinical presentation. Symptoms encountered can occur early or late in the course of the disease. In the aforementioned NIH series, which included 60 patients observed over 20 years, only 33% of patients had constitutional symptoms (corresponding to stage 1); 18% of patients never progressed to the third stage.

The first stage is an early systemic stage during which the patient may complain of constitutional symptoms (eg, fatigue, malaise, giddiness, fever). This stage is considered to be prevasculitic.

The second stage is the vascular inflammatory stage when stenosis, aneurysms, and vascular pain (carotidynia) tend to occur.

Signs and symptoms characterizing the vascular inflammatory stage include fatigue, fevers, malaise, pain in extremities and joints, dyspnea, palpitations, headaches, rash (erythema nodosum or a lupuslike butterfly rash, which can be photosensitive), hemoptysis, ulceration, and weight loss. A single case of thoracic and lumbar spine pain has been reported. Symptoms of vascular insufficiency include arm numbness, claudication in the legs (rare), blurry vision, double vision (which can be posture dependent), amaurosis fugax, stroke, transient ischemic attacks, hemiplegia, seizures, and paraplegia.

The constitutional systemic symptoms and vascular symptoms may occur at the same time, rendering the classification into stages practically impossible.

The third stage is the burned-out stage, when fibrosis sets in, and generally is associated with remission. This stage does not occur in all patients, and even in patients who are in remission, relapses can occur. Presumably, the burned-out stage manifests with minimal symptoms, but little supportive evidence is found in the literature.

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