Giant cell arteritis

Alternative names
Arteritis - temporal; Cranial arteritis; Temporal arteritis

Definition
Temporal arteritis is a disorder involving inflammation and damage to blood vessels, particularly the large or medium arteries that branch from the external carotid artery of the neck.

Causes, incidence, and risk factors
Giant cell, cranial, or temporal arteritis occurs when there is inflammation and necrosis (death of the tissues) of one or more arteries. It most commonly occurs in the head, especially in the temporal arteries that branch from the carotid artery of the neck. However, it can be systemic, affecting multiple medium-to-large sized arteries anywhere in the body.

The cause is unknown but is assumed to be, at least in part, an effect of the immune response. The disorder has been associated with severe infections and high doses of antibiotics. The symptoms occur because of inflammation.

The disorder may exist independently or may coexist with or follow polymyalgia rheumatica (a disorder characterized by abrupt development of pain and stiffness in the pelvis and shoulder muscles). About 25% of people with giant cell arteritis also experience polymyalgia rheumatica.

Giant cell arteritis is seen almost exclusively in those over 50 years old, but may occasionally occur in younger people. It is rare in people of African descent. There is some evidence that it runs in families.

Symptoms

     
  • fever  
  • a throbbing headache on one side of the head or the back of the head  
  • scalp sensitivity, tenderness when touching the scalp  
  • jaw pain, intermittent or when chewing  
  • vision difficulties       o blurred vision, double vision       o reduced vision, blindness in one or both eyes  
  • weakness, excessive tiredness  
  • a general ill feeling  
  • a loss of appetite  
  • weight loss (more than 5% of total body weight)  
  • muscle aches  
  • excessive sweating

Additional symptoms that may be associated with this disease:

     
  • mouth sores  
  • joint stiffness  
  • joint pain  
  • hearing loss  
  • bleeding gums  
  • face pain

Signs and tests
When the doctor feels (palpates) the head, the scalp is sensitive and often shows a tender, thick artery on one side of the head. The affected artery may have a weakened pulse or no pulse. About 40% of people will have other nonspecific symptoms such as respiratory complaints (most frequently dry cough) or mononeuritis multiplex (weakness and/or pain of multiple individual nerve groups). Rarely, paralysis of eye muscles) may occur. A persistant fever may be the only symptom.

Blood tests are nonspecific.

     
  • A sedimentation rate and C-reactive protein are almost always very high.  
  • A hemoglobin or hematocrit may be normal or low.  
  • Liver function tests may be abnormal, including elevated alkaline phosphatase, if the disorder is systemic.

A biopsy and analysis of tissue from the affected artery show changes that confirm the diagnosis of temporal vasculitis in most cases.

Treatment
The goal of treatment is to minimize irreversible tissue damage that may occur because of lack of blood flow (ischemia).

Corticosteroids such as Prednisone are commonly prescribed to reduce inflammation.Corticosteroid treatment may be started even before a biopsy confirms the diagnosis (to reduce the risk of complications). Aspirin may be recommended in addition to corticosteroids. Medications that suppress the immune system (such as cyclophosphamide or methotrexate) are occasionally prescribed to try to minimize the dose of steroids needed.

Expectations (prognosis)
Most people make a full recovery, but prolonged treatment, for 1 to 2 years, may be necessary. Recurrence is possible for several years.

Complications

Possible complications, especially if not treated properly or promptly, include:

     
  • obstruction of the arteries of the eye, leading to blindness or ophthalmoplegia (weakness or paralysis of one or more eye muscles)  
  • TIA or stroke  
  • damage to other blood vessels in the body  
  • side effects of therapy

Calling your health care provider
Call your health care provider if persistent throbbing headache and other symptoms that indicate giant cell arteritis occur.

Prevention
There is no known prevention.

Johns Hopkins patient information

Last revised: December 8, 2012
by Brenda A. Kuper, M.D.

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