Takayasu’s arteritis, also called TAK, is a rare disease involving inflammation in the walls of the largest arteries in the body: the aorta and its main branches. The disease results from an attack by the body’s own immune system, causing inflammation in the walls of arteries. The inflammation leads to narrowing of the arteries, and this can reduce blood flow to many parts of the body. TAK can result in a weak pulse or loss of pulse in arms, legs and organs. For this reason, people used to refer to the illness as “pulseless disease.”
What is Takayasu’s Arteritis?
This disease is one of many types of vasculitis. Vasculitis refers to inflammation of blood vessels, and arteries are a type of blood vessel. In TAK, this inflammation occurs in the walls of large arteries: the aorta and its main branches. These blood vessels supply blood to the head, arms, legs and internal organs, such as the kidneys. Inflammation may cause the vessels’ walls to thicken. With time, this thickening results in a narrowing inside the artery, called a “stenosis.” If severe enough, such narrowing can reduce blood flow and result in less oxygen sent to the body parts or organs that the artery supplies.
Stenosis can cause symptoms (what you feel) and problems ranging from annoying to dangerous:
- Pain with use of an arm or leg (called “claudication”)
- Dizziness, headaches or fainting
- Weakness and fatigue
- High blood pressure
- Chest pain
- Heart attack
Stenosis occurs slowly with time, and smaller vessels may grow and expand to carry blood around the blockage. These new vessels are called “collateral vessels.” Collateral vessels may help prevent major organ damage.
Sometimes inflammation in the artery weakens the vessel wall, causing vessel expansion rather than narrowing (stenosis). This expansion is called an aneurysm (a bulge in the artery). The aorta as it emerges from the heart is one of the more common areas where an aneurysm can form. An aneurysm in the aorta might lead to heart valve dysfunction or rupture (bursting) of the aorta.
Juvenile Idiopathic Arthritis
What is juvenile idiopathic arthritis (JIA)?
There are many terms used to describe a child with chronic arthritis. These include juvenile rheumatoid arthritis, juvenile chronic arthritis and juvenile idiopathic arthritis. Several types of arthritis, all involving chronic (long-term) joint inflammation, fall under the JIA heading. This inflammation begins before patients reach the age of 16, and symptoms last from 6 weeks to 3 months to be called chronic. JIA may involve one or many joints, and cause other symptoms such as fever, rash and/or eye inflammation.
Systemic onset JIA affects about 10 percent of children with arthritis. It begins with repeating fevers that can be 103°F or higher, often accompanied by a salmon-colored rash that comes and goes. Systemic onset JIA may cause inflammation of the internal organs as well as the joints, though joint swelling may not appear until months or even years after the fevers begin. Anemia (a low red blood cell count) and elevated white blood cell counts are also typical findings in blood tests ordered to evaluate the fevers and ongoing symptoms. Arthritis may persist even after the fevers and other symptoms have disappeared.
Oligoarticular JIA, which involves fewer than five joints in its first stages, affects about half of all children with arthritis. Girls are more at risk than boys. Older children with oligoarticular JIA may develop “extended” arthritis that involves multiple joints and lasts into adulthood. Children who develop the oligoarticular form of JIA when they are younger than 7 years old have the best chance of having their joint disease subsiding with time. They are, however, at increased risk of developing an inflammatory eye problem (iritis or uveitis). Eye inflammation may persist independently of the arthritis. Because iritis usually does not cause symptoms, regular exams by an ophthalmologist (eye doctor) are essential to detect these conditions and identify treatment to prevent vision loss.
Polyarticular JIA affects five or more joints and can begin at any age. Children diagnosed with polyarticular JIA in their teens may actually have the adult form of rheumatoid arthritis at an earlier-than-usual age.
With Psoriatic Arthritis, children have both arthritis and a skin disease called psoriasis or a family history of psoriasis in a parent or sibling. Typical signs of psoriatic arthritis include nail changes and widespread swelling of a toe or finger called dactylitis.
Enthesitis-Related Arthritis is a form of JIA that often involves attachments of ligaments as well as the spine. This form is sometimes called a spondyloarthropathy. These children may have joint pain without obvious swelling and may complain of back pain and stiffness.
Some children with arthritis develop uveitis, an inflammation of the eye.
What causes JIA?
Malfunctioning of the immune system in JIA targets the lining of the joint, known as the synovial membrane. This causes inflammation. When the inflammation persists, joint damage may occur.
It is not known what causes the immune system to malfunction in JIA. These conditions are not considered hereditary and rarely involve more than one family member. Research suggests that some individuals may have a genetic tendency to develop JIA, but develop the condition only after exposure to an infection or other unknown trigger. Dietary and emotional factors do not appear to play a role in the development of JIA.
Who gets JIA?
About 1 child in every 1,000 develops some type of juvenile arthritis. These disorders can affect children at any age, although rarely in the first six months of life.
How is JIA diagnosed?
JIA may be difficult to diagnose because some children may not complain of pain at first, and joint swelling may not be obvious. There is no blood test that can be used to diagnose the condition and adults with rheumatoid arthritis typically have a positive rheumatoid factor blood test, but children with JIA typically have a negative rheumatoid factor blood test. As a result, diagnosis of JIA depends on physical findings, medical history and the exclusion of other diagnoses. Typical symptoms include:
- Stiffness when awakening
- Reluctance to use an arm or leg
- Reduced activity level
- Persistent fever
- Joint swelling
- Difficulty with fine motor activities
Other conditions that can look like JIA, including infections, childhood cancer, bone disorders, Lyme disease and lupus also must be ruled out before a diagnosis of JIA can be confirmed.
How is JIA treated?
Optimal care is tailored for each child with JIA and provided by an experienced team of healthcare providers that should include a pediatric rheumatologist, physical and occupational therapist, social worker and nurse specialist. This core team can coordinate efforts with the child’s pediatrician, adult rheumatologists, other physicians (such as an ophthalmologist or orthopedic surgeon) and other health professionals (dentist, nutritionist or psychologist) as well as reach out to schools and additional community resources as necessary to ensure that the child receives the best care possible.
The overall treatment goal is to control symptoms, prevent joint damage and maintain function. The first line of treatment involves a non-steroidal anti-inflammatory drug or NSAID. Examples of NSAIDs, such as Ibuprofen or Naproxen, administered in a dose appropriate for the child’s weight. Younger children may be given liquid preparations or medications that require less frequent use. Because NSAIDs can cause gastrointestinal distress (gastritis), they should be taken with food.
Disease modifying drugs—commonly called DMARDs—are added as second-line treatment when arthritis does not respond to NSAIDs. DMARDs include Methotrexate, Leflunomide and more recently developed medications known as biologics. The biologics include anti-tumor necrosis factor agents such as Etanercept; Infliximab; Adalimumab; Abatacept; Anakinra; Canakinumab and Tocilizumab. Each of these medications can cause side effects that need to be monitored and discussed with the rheumatologist treating your child. Most of these treatments are approved for use in children as well as adults. In addition, researchers are developing newer and much safer treatments.
When only a single joint is involved, steroid can be injected into the joint before any additional medications are given. Oral steroids such as prednisone may be used in certain situations, but only for a short period of time and at the lowest dose possible. The long-term use of steroids is associated with unacceptable side effects such as weight gain, poor growth, osteoporosis, cataracts, avascular necrosis, hypertension, and risk of infection.
Because the causes of JIA are unknown, no one knows how to prevent these conditions.
Children living with arthritis
Children with JIA should attend school, participate in extra-curricular and family activities, and live life as normally as possible. To foster a healthy transition to adulthood, adolescents with JIA should be allowed to enjoy independent activities, such as taking a part-time job and learning to drive.
A positive outlook and continued physical activity will help. Physical and occupational therapy can increase joint motion, reduce pain, improve function, and increase strength and endurance. Therapists may construct splints to prevent permanent joint tightening or deformities, and work with school-based therapists to address issues at school.
Opportunities for the child to interact with other children who have arthritis may be available in or near your community and should be utilized to make the child participate in group activities, play to ensure proper physical and psychosocial development.
What causes Takayasu’s Arteritis?
As with most types of vasculitis, the cause of TAK is not known. It is rare to see more than one case in a family and the role of genetics is unclear. A link between TAK and an infection has also not been proven.
TAK is thought to be an autoimmune disease, which means that the body comes under attack by its own immune system. In TAK, the immune system is attacking the blood vessels.
Who gets Takayasu’s Arteritis?
TAK is rare, affecting perhaps one in 200,000 people. It most often occurs in people ages 15–40 years, but sometimes affects younger children or middle-aged adults. Nine of 10 patients are female.
How is Takayasu’s Arteritis diagnosed?
Doctors most often find TAK on an angiogram, a test that shows how well blood flows in arteries. A doctor often orders an angiogram when a patient has symptoms and abnormal results of the physical exam. These include loss of pulse or low blood pressure in an arm, or abnormal sounds (“bruits”) heard over large arteries with a stethoscope.
There are various types of angiograms, including standard ones that involve injection of dye directly into an artery while X-ray test are taken. Less invasive types of angiography use another imaging technique such as computed tomography, and this is CT angiography or CTA. When MRI—magnetic resonance imaging— is used, it is called magnetic resonance angiography or MRA.
Angiograms may show narrowing of one or more large arteries. It is important for the doctor to try to distinguish between narrowing due to vasculitis (inflammation of arteries) and narrowing due to atherosclerosis (“hardening” of the arteries). At times, this can be challenging. There are other causes of arterial narrowing as well, including fibromuscular dysplasia, another rare disease that mainly affects women.
Large arteries can also become inflamed in a few other diseases. Examples include other types of vasculitis: Giant cell arteritis (a disease of older adults), relapsing Polychondritis, Cogan’s syndrome and Behçet’s disease. Some infections can also cause inflammation in large arteries.
Blood tests for inflammation include measurements of the erythrocyte sedimentation rate (ESR) and C-reactive protein (often called CRP). Results of these tests are often, but not always, high in patients with active TAK. However, these tests are also abnormal in a large number of other inflammatory diseases. Patients with TAK may also have anemia due to chronic (long-term) inflammation. Anemia is also tested for with a blood test. None of these blood tests can tell you for sure if you have TAK, and these blood tests may be abnormal in many other diseases.
Patients with TAK may have no symptoms, and the disease is so rare that doctors may not easily recognize it. Thus, there is often a delay in detecting it, sometimes several years.
How is Takayasu’s Arteritis treated?
TAK most often needs treatment to prevent further narrowing of affected arteries. Yet, the narrowing that has already occurred often does not improve, even with drug treatment.
Glucocorticoids (prednisone, prednisolone or others), often referred to as “steroids,” are an important part of treatment. The dose and length of treatment depend on how bad the disease is and how long the patient has had it. However, these drugs can have long-term side effects.
Doctors sometimes prescribe immune-suppressing drugs because their side effects may be less serious than those of glucocorticoids. This is called “steroid-sparing” treatment. These medicines include Methotrexate, Azathioprine, Mycophenolate mofetil, Cyclophosphamide and drugs that block tumor necrosis factor (such as Etanercept, Adalimumab or Infliximab). Doctors frequently prescribe these drugs to treat other rheumatic diseases, but they also use them to treat TAK. There is not enough proof that these drugs are definitely effective in treating TAK. Research studies are ongoing to find new drugs to treat TAK.
Some experts advise routine use of low-dose aspirin. The thought is that it will help prevent blood clots from forming in damaged arteries.
Therapy for TAK also includes screening for high blood pressure and high cholesterol, and treatment if these problems are present.
Lasting damage to arteries sometimes needs a vascular procedure or surgical treatment. This may involve angioplasty (widening a narrowed or blocked blood vessel), with or without placement of a stent, to prop open the vessel. Another treatment option is bypass grafting, a surgery to redirect blood flow around a blockage in a blood vessel.
What is the broader health impact of Takayasu’s Arteritis?
Because TAK can cause heart problems, high blood pressure and stroke, patients with TAK should talk to their doctor about ways to lower the risk of these serious problems.
Living with Takayasu’s Arteritis
TAK is a chronic disease and may need long-term treatment. Some patients have no symptoms or only mild symptoms, but others are disabled or need surgery more than once. Side effects from medicines, mainly glucocorticoids, can be troubling. Patients taking immunosuppressants are at risk of infections.
In patients with TAK due to arterial stenosis, the Blood pressure measurement often may not be correct (falsely low due to blocked arteries) in the arms. So, your health care provider may need to measure your blood pressure in a leg.
The disease can recur after treatment or can silently get worse. It is often very hard to know whether TAK is active again. Thus, most patients need frequent doctor visits and angiograms.