Takayasu arteritis, or pulseless disease, causes inflammation and narrowing of the aorta and arteries. This can lead to blockages and restrict blood flow to vital organs.

Takayasu arteritis is a rare, chronic inflammatory disease that primarily affects the aorta and its main branches. It is a form of vasculitis, a group of disorders that cause inflammation of the blood vessels.

The condition gets its name from Japanese physician Mikito Takayasu, who first described it in 1908. Understanding the symptoms and risk factors is crucial for early diagnosis and effective management, as the disease can lead to serious complications, such as a stroke, if individuals do not seek treatment.

In this article, we delve into Takayasu arteritis, its symptoms, when to contact a doctor, causes, risk factors, diagnosis, treatment, and outlook.

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Takayasu arteritis, or pulseless disease, is a condition in which the body’s immune system attacks the medium and large arteries, causing inflammation and damage in the affected area. Takayasu arteritis is a type of vasculitis.

It often targets the aorta and its main branches, such as the arteries supplying the kidneys, neck, and arms. Takayasu arteritis causes the walls of these blood vessels to thicken, meaning they become blocked or narrow. This can also restrict blood flow to various organs and tissues in the body.

Over time, Takayasu arteritis can cause serious health complications.

Takayasu arteritis is a rare disease, affecting approximately 1 to 2 people per million. The condition mostly occurs in people of Asian descent, with the highest known prevalence in Japan, with 40 cases per million people.

The signs and symptoms of Takayasu arteritis can differ according to the stage:

Early stage

This is also known as the pre-pulseless stage.

Symptoms may include:

As these symptoms are not specific to Takayasu arteritis, many individuals do not seek medical help right away. Also, some people may not experience symptoms in the early stages of Takayasu arteritis, which can delay diagnosis.

Late stage

This is also known as the pulseless stage. Approximately 85% of people experience symptoms in their limbs.

Signs and symptoms may include:

Individuals experiencing symptoms such as shortness of breath, lightheadedness, or sudden chest pain should contact a doctor immediately. This may be a warning sign of a heart attack and is a medical emergency.

If individuals experience any of the symptoms of a stroke, they should seek urgent medical attention. Symptoms include:

  • face drooping on one side
  • weakness in one or both arms
  • difficulty speaking
  • numbness or weakness of the face, arm, or leg, especially on one side of the body
  • confusion
  • difficulty understanding other people
  • loss of coordination
  • trouble seeing in one or both eyes
  • loss of balance or coordination
  • severe headache with no known cause

Early intervention, diagnosis, and treatment are vital to managing Takayasu arteritis effectively and preventing severe complications.

The exact cause of Takayasu arteritis remains unknown, but doctors believe it involves an autoimmune response, where the immune system mistakenly attacks the body’s own arteries.

Genetic factors, environmental triggers, and infections may be possible contributors.

Risk factors for Takayasu arteritis include:

  • Age: While the disease can affect anyone, it tends to affect those under the age of 50 years.
  • Sex: Takayasu arteritis primarily affects females, with up to 9 in 10 cases among adults occurring in females.
  • Ethnicity: Takayasu arteritis is more common among people of Asian descent.

There is no single test for Takayasu arteritis. Diagnosis involves a combination of tests, including:

Treatment aims to:

  • reduce inflammation
  • prevent disease progression
  • manage symptoms

Some people may achieve remission and reduce or stop treatment, while others may require long-term treatment.

Options include:

Immunosuppressant drugs

Often, corticosteroids are the first line of treatment to manage inflammation. Doctors may prescribe prednisone, starting at a high dose and then gradually reducing it to minimize side effects.

A doctor may prescribe other immunosuppressant medications in combination with corticosteroids. This can include the following drugs:

  • methotrexate
  • azathioprine
  • mycophenolate mofetil

Biologic agents

Newer treatments, such as tocilizumab or infliximab, target specific components of the immune system. They can be effective for more complicated cases of Takayasu arteritis.

Surgery

Vascular surgery may be necessary in severe cases or to prevent relapse. It can repair blocked, narrowed, or damaged arteries and increase blood flow.

Learn more about vascular surgery.

Without treatment, the chronic inflammation and arterial wall damage from Takayasu arteritis can lead to various complications, including:

  • Aneurysms: This refers to an atypical bulging of the artery walls, which can rupture and cause life threatening bleeding.
  • Stroke: Reduced blood flow to the brain can result in a stroke.
  • Heart failure: Inflammation of the blood vessels and damage to the heart and its valves can lead to heart failure.
  • Hypertension: Also known as high blood pressure, this can occur particularly when Takayasu arteritis affects the blood vessels to the kidney.
  • Vision loss: The involvement of blood vessels in the eyes can lead to visual disturbances or vision loss.

There is currently no cure for Takayasu arteritis.

Takayasu arteritis is a chronic condition. Therefore, some individuals may require lifelong monitoring and treatment. However, with the right measures in place, many individuals can manage their symptoms and lead healthy lives.

What is the life expectancy of a person with Takayasu arteritis?

Life expectancy with Takayasu arteritis can vary, but it is significantly lower in high risk patients. This underscores the importance of early diagnosis, continuous monitoring, and individualized treatment plans to improve outcomes and quality of life.

A 2019 study looking at 318 people with Takayasu arteritis found that the 5-year mortality rate is 1.9% and the 10-year mortality rate is 3.9%.

Without treatment, individuals with Takayasu arteritis can experience serious complications that may significantly affect quality of life and longevity.

Living with Takayasu arteritis can be challenging. Pain, emotional stress, and medication side effects can affect well-being and other aspects of daily life.

Several strategies can help individuals cope and maintain a good quality of life, including:

  • Regular medical follow-ups: Consistent monitoring by healthcare professionals can help manage disease activity and adjust treatments as necessary.
  • Healthy lifestyle: Eating a balanced diet, exercising regularly, and avoiding smoking can improve overall health and reduce the risk of complications.
  • Stress management: Techniques such as yoga, meditation, or counseling can help manage stress and improve mental well-being.
  • Support: Connecting with others who have Takayasu arteritis through support groups or online communities can provide emotional support and practical advice.

Takayasu arteritis is a rare, chronic inflammatory disease that affects the aorta and its main branches. The cause is largely unknown, but doctors suspect it may occur due to an autoimmune response. Genetic factors, environmental triggers, and infections may also contribute.

Takayasu arteritis can ultimately restrict blood flow to various organs and tissues. Without treatment, this can lead to serious complications such as stroke or heart failure.

Early diagnosis and treatment are crucial to improving outcomes and managing the disease. Individuals with Takayasu arteritis can lead fulfilling lives with proper medical care, lifestyle modifications, and support.