Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The rare disease that we will be learning about today is:

Takayasu’s Arteritis

Sometimes called aortic arch syndrome.

What is Takayasu’s Arteritis?

• Takayasu’s arteritis is a type of granulomatous vasculitis that impacts the large blood vessels, typically the aorta and its branches, such as the subclavian artery, common carotid artery, and brachiocephalic artery
  • Sometimes the pulmonary arteries can be affected as well
• Symptoms tend to begin between 15 and 30 years old
• If the renal arteries are affected, renovascular hypertension may appear
• Impaired blood flow can lead to damage to the heart or other organs
• Takayasu’s arteritis is named after Mikito Takayasu, a Japanese ophthalmologist who first described the disease in 1908

How Do You Get It?

• The precise cause of Takayasu’s arteritis is unknown, but the mechanism of the disease is believed to be autoimmune
• Certain genetic variants appear to contribute to the disease, but have not been identified as the direct cause
• Women are eight to nine times more likely to be affected than men
• Women of Asian descent are most likely to have Takayasu’s arteritis, but the disease can potentially affect anyone

What Are the Symptoms?

• Symptoms of Takayasu’s arteritis include:
  • Lack of pulse in the wrist
  • Fever
  • Malaise
  • Fainting
  • Fatigue
  • Joint pain
  • Weight loss
  • Night sweats
  • Narrowing of the affected arteries
  • Heart valve abnormalities
  • Hypertensive crisis

How Is It Treated?

• Steroids such as prednisone are a common treatment for Takayasu’s arteritis
  • The dose is usually tapered over time to minimize side effects while maintaining benefit
• Tocilizumab and mycophenolate can also be effective treatments
• Unfortunately, a small number of patients may not respond to these treatments
  • In these cases, revascularization procedures, such as angioplasty and stenting or vascular bypass, may be necessary
    • Outcomes of surgery can vary depending on disease severity

Where Can I Learn More???

• Learn more about this disease from the Vasculitis Foundation.
• Check out our cornerstone on this disease here.