Takayasu arteritis

Alternative names
Pulseless disease

Definition
Takayasu arteritis is an inflammmation of the aorta and its major branches.

Causes, incidence, and risk factors

The cause of Takayasu arteritis is unknown. The disease occurs primarily in children and young women younger than 30, many of whom are of Asian or African descent. It appears to be an autoimmune condition, where immune cells are wrongly targeted against the body’s own tissues, and may involve other systems.

Because of changes in the aorta and its branches that feed the head, neck, and upper limb areas, patients may have decreased or absent radial pulses (pulse in the wrist) and pain in the arm and forearm. Inflammation in the carotid arteries (large arteries in the neck that supply blood to the brain) may cause visual problems or neurological problems such as dizziness or Stroke. Decreased blood flow to the kidneys often causes hypertension (high blood pressure). The formation of aneurysms may lead to rupture of the affected vessel.

Systemic complaints may include fever, rash, muscle aches (myalgia), and arthritis. Chest pain can develop due to inflammation of either the lining of the chest cavity (pleuritis) or the sac-like covering of the heart (pericarditis), or due to Heart attack (Myocardial Infarction).

The survival rate is better for adults than for children.

Symptoms

     
  • Fever  
  • Fatigue  
  • Decreased or absent radial pulses  
  • Skin rash  
  • Weight loss  
  • Night sweats

Signs and tests

     
  • High blood pressure (hypertension)  
  • Decreased radial pulses (at the wrist) with normal femoral pulses (groin)  
  • Difference in blood pressure between the two arms

There may also be signs of inflammation (pericarditis or pleuritis).

Tests:

     
  • CBC  
  • ESR (erythrocyte sedimentation rate)  
  • ZSR (zeta sedimentation rate), which is similar to an ESR  
  • Serum immunoelectrophoresis, a blood test  
  • X-ray of the chest  
  • Arteriogram  
  • ECG

Treatment

Treatment of Takayasu arteritis is difficult, but results with appropriate therapy are encouraging. Early detection is important. Steroids and immunosuppressive agents form the cornerstone of treatment, with surgery reserved for complications caused by narrowed arteries.

Surgery to bypass narrowed arteries may be necessary to supply blood beyond the constriction.

Expectations (prognosis)

This disease can be fatal. However, with a combination of aggressive medical and surgical therapy, mortality (death) rates have dropped dramatically. In well-managed cases of Takayasu arteritis, the long-term survival rate is greater than 90%.

Complications

Calling your health care provider

Call your health care provider if you have symptoms of this condition. Weak pulse, chest pain, and breathing difficulty require immediate care.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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