Takayasu’s arteritis

Currently, Takayasu’s arteritis (TAK) is one of the most relevant cardiovascular diseases, but it’s also one
of the hardest diseases to spot or diagnose, due to the fact that it could be confused with other diseases
of the kind, such as arteriosclerosis or tuberculosis. The article that was used as a foundation to make this
review explains how this vasculitis behaves and what it does to the aorta artery and its ramifications, as
well as its different aspects at an etiological level, diagnose and treatment.

It starts with a detailed description of the disease. We are informed that Takayasu’s arteritis is a type of
vasculitis that affects the aorta artery as well as its ramifications. It is believed that it’s caused by an
infiltration of type T gamma/delta, T CD4, NK and B lymphocytes. It starts in the outer layer (adventitia)
but it will mainly affect the outer muscular layer of the aorta. These immune cells will stimulate the release
of pro-inflammatory cytokines, such as IL-2, 4, 6, 8, 10, 12, 18 and tumor necrosis factor alpha (TNF alpha)
in which, after an antigen activates the major histocompatibility complex, provoking the activation of
macrophages and IL-18 (all of this happening at the vasa vasorum in the adventitia), IL-1 and 6 will be
released and they will degrade the elastin layer and the smooth muscle cells of the outer muscular layer,
which will cause arterial stenosis or, more specifically, inner-layer hyperplasia, which is the earliest stage
of the disease. There’s also a hypothesis stating that this disease might have a trombo-genetical
predisposition, in which P-selectin increase will lead to increased production of thrombin and and
antithrombin III.

At a symptomatic level, it’s not unusual to find discrepancies, which might affect the correct diagnosis of
this pathology. The following are the most common early symptoms:

• Fever of unknown origin

• Weight loss
• Arthalgia (joint stiffness)
• Myalgia (muscle pain)

It was also possible to identify that patients with TAK have an abnormal pulse in their extremities due to
the inflammatory processes happening at a vascular level. Nevertheless, there are more variables that we
could take into account for the detection of TAK in its two phases.

1. Inflammatory phase, in which we might encounter symptoms, such as:

• Anorexia
• Night sweats
• granular proderma
• Pseudochorythema nodosum

Occlusive phase ( TAK is often detected in this stage):

• Varied arterial stenosis

• Neurological, cardiac, renal and intestinal disorders
Aiming to get a correct diagnosis, there are multiple invasive and non-invasive alternatives. However, it is
important to note that one must have many classification criteria, such as Ishkawa’s, in order to have a
clearer vision of what one might detect.

Techniques Advantages Disadvantages

Angiography Pressure measurement Invasive

No information about the

Ultrasound
magnetic resonance imaging
vessel’s wall
Non-invasive It’s not possible to measure the
central aortic pressure
Non-invasive It’s not possible to measure the
central aortic pressure

Poor correlation with IMR to

determine edema in the vessels
positron emission tomography Non-invasive Expensive

Metabolic activity measurement It could be confused with

atherosclerosis

It’s not possible to measure the

central aortic pressure
electron beam computed Non-invasive Radiation exposure
tomography

Two drugs, which are still in review, were talked about in the article. These drugs have shown some
efficiency in TAK’s treatment. They are abatacept and tocilzumab, which are involved in
immunosuppressive therapy.

AGC-tocilzumab Immunosuppressive therapy showed a great efficacy rate by achieving the inhibition of
IL-6 and TNF-a with their respective antagonists. The study was performed with 38 patients, from which
18 of them had a disease relapse. It was also evidenced that the time period before relapse was quite
positive. Nonetheless, the treatment with abatacept was performed with 26 patients, from which 18
relapsed, and 3 of the relapsed patients had new vascular injuries. Even though the data is positive, a
large-scale study is required, along with a better evaluation criteria.

Endovascular surgery is a good option that must be considered, since it’s benefits usually remain in the
long run. It’s recommended to keep the immunosuppressive therapy before and after surgery.