Takayasu arteritis (TAK) is a rare condition that involves inflammation of the lining of large blood vessels called arteries. It is sometimes called aortic arch syndrome.
This inflammation caused by Takayasu Arteritis can seriously damage important arteries including those that supply blood to the heart and lungs.
Takayasu Arteritis belongs to a family of arthritic diseases called vasculitis. The word vasculitis means inflammation of blood vessels.
Some people who get the disease go into remission after successful treatment and eventually no longer require medication. In others, the condition behaves more like a chronic (long-term) disease that must be controlled with medication on an ongoing basis.
Takayasu Arteritis is an autoimmune disease, which means it occurs when the body’s immune system attacks its own healthy cells and tissues. The reason why it does this is not well understood.
Importance of Treatment
It is very important to treat Takayasu arteritis quickly and aggressively to control inflammation that can damage the arteries.
Takayasu arteritis is considered a medical emergency. If left untreated it can lead to serious complications including loss of vision and stroke.
Who Gets Takayasu Arteritis
Takayasu arteritis mostly affects young people, primarily between the ages of 10 and 40 years. It is 8 times more common in young women than young men, and slightly more common in people of Asian or African descent.
Understanding Takayasu Arteritis
The first signs and symptoms of Takayasu arteritis are often hard to pinpoint. People with the condition usually start feeling fatigued and generally unwell.
Many people with Takayasu arteritis think that they have a viral illness like a cold or flu.
Symptoms can include: fever, headache, and muscles that may feel sore and achy. Loss of appetite and unintended weight loss can also occur.
Inflamed Blood Vessels
As Takayasu arteritis progresses, the blood vessels become inflamed and tender. This is especially common over the carotid arteries in the neck – the blood vessels that supply the brain with blood – which are close to the surface of the skin.
Other possible symptoms of Takayasu arteritis depend on which arteries are affected by the disease:
- Heart or Aorta: If the heart’s blood vessels are involved, patients may feel chest pain. Eventually, inflammation can cause swelling of the blood vessel walls and blockage of arteries. If the aorta, the body’s main artery, is affected, the heart can have trouble properly pumping blood.
- Carotid arteries (neck): a stroke or seizure might occur. The carotid arteries supply blood to the brain.
- Vertebral arteries (vessels that supply the head): dizziness and visual problems might occur.
- Limbs (arms or legs): a person could lose the pulse in their limb, feel weakness or notice a colour change in the affected limb.
- Kidneys: If arteries near the kidney are affected, high blood pressure is possible.
Takayasu Arteritis is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease.
To diagnose the disease, they will take a careful and complete history and perform a thorough physical examination. Based on this information, the doctor will likely order tests like blood tests and scans to confirm the diagnosis.
A type of scan called an angiogram is considered to be the gold standard test to diagnose Takayasu arteritis.
Common Tests to Diagnose Takayasu Arteritis
Scans that show blood vessels (angiograms): A dye is injected and pumped through the arteries and then pictures are taken using magnetic resonance imaging (MRI), a computed tomography (CT) scan, or a regular x-ray.
MRI is more common because it requires a lower load of dye and shows the vessel wall in greater detail.
Angiograms give doctors a picture of what’s going inside the arteries and can help detect Takayasu arteritis as early as possible.
Blood tests that look for inflammation: Takayasu arteritis is an inflammatory disease so these tests are expected to show abnormal results. Common tests include a Complete Blood Count (CBC), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP).
Tests that look at kidney function: Creatinine is a common blood test that looks at how well the kidneys are working. Abnormally high levels of creatinine can indicate a problem. A urinalysis is also important to look for protein or blood in the urine. This may be an indicator that the kidneys are affected.
Scientists don’t fully understand what causes Takayasu arteritis but it’s thought to be an autoimmune disease: it is caused by the body’s immune system attacking its own healthy cells and tissues. In Takayasu arteritis, the targets of the attack are large blood vessels.
Takayasu arteritis is considered a medical emergency that should be treated early and aggressively to avoid serious consequences like blindness and stroke. People with the disease can lead active and productive lives with the right kinds of treatment.
Medications for Takayasu arteritis are aimed at controlling the inflammation that can eventually damage the arteries. In some people, once inflammation is brought under control the disease can go into remission. When that happens, doses of medicines can sometimes be tapered down and eventually stopped.
In other people, the disease behaves more like a chronic (long-term) disease that must be controlled with small doses of medicine on an ongoing basis.
Corticosteroid medications like prednisone are the primary treatment for Takayasu arteritis. They can help control inflammation and may also prevent long-term damage in some people.
Prednisone is usually started at a high dose to quickly bring inflammation under control. Most people start to feel better within a few days.
Once symptoms have improved and markers of inflammation have come back to normal, the dose can start to be tapered down. In some patients small doses of prednisone might be required on an ongoing basis to keep the disease under control.
Disease Modifying Anti-Rheumatic Drugs (DMARDs)
In severe cases, Cytoxan (cyclophosphamide) may be used to control inflammation. This medication carries a few more side effects including an effect on fertility in both men and women. If cyclophosphamide is contemplated then family planning must be discussed.
Biologic medications such as Actemra (tocilizumab) that blocks an important immune signaling protein called interleukin-6 have been used successfully to treat some cases of Takayasu arteritis but more studies are needed before they can be widely used.
Other biologic medications used to treat Takayasu arteritis include Tumor Necrosis Factor Inhibitors such as Etanercept (Enbrel/Brenzys/Erelzi) or Inflximab (Remicade/Inflectra/Remsima). Other biologics such as Abatacept (Orencia), Rituximab (Rituxan), and Ustekinumab (Stelara) have been successful in some case reports.
Surgery may be required to improve the flow of blood in cases where the arteries have become severely narrowed. There are different surgeries that can be performed including bypass, angioplasty, and stenting.