Giant cell arteritis

Definition

The term temporal arteritis literally means "inflammation of the temporal arteries."As implied by the name, these blood vessels run along the temples after they branch off from the carotid artery in the neck. They provide the blood supply to portions of the scalp, jaw muscles, and salivary glands. Inflammation of these arteries, probably resulting from an abnormal immune reaction, disrupts this blood supply, resulting in a variety of symptoms. They can range from relatively minor—jaw pain or headache—through major—including temporary or permanent blindness.

Temporal arteritis is also called giant cell arteritis or cranial arteritis. It is a rheumatic disease that affects large and medium-sized arteries throughout the body and can occur in a variety of patients. Although the temporal arteries are most commonly affected, other arteries throughout the body may be affected. The disease seems to target arteries containing elastic tissue. Veins are rarely affected. Temporal arteritis is a type of vasculitis.

Description

Temporal arteritis almost always occurs in people over 50, and it becomes more common as people age. About 20 out of 100,000 people over the age of 50 suffer from temporal arteritis. Women are affected twice as often as men. Some authorities say that temporal arteritis is more common in Caucasians (especially Scandinavians) than in people of other races. Close relatives of patients with temporal arteritis may be more likely than others to get the disease.

Patients with temporal arteritis are diagnosed and overlap with a broader disorder called giant cell arteritis. This can affect parts of the body in addition to the scalp, eyes, and jaw. Sometimes the disease can cause restricted circulation to both arms or both legs, producing pain in the affected limbs. With other blood vessels involved, patients with advanced forms of the disease may experience strokes or transient ischemic attacks (TIA). These result in brief episodes of pain caused by decreased blood flow. Even heart attacks are occasionally caused by giant cell arteritis.

— Richard H. Lampert

Definition

Temporal arteritis is a disease that causes inflammation and sometimes blockage of medium and large arteries in the head (often near the side of the head or temples).

Description

The mechanism responsible for temporal arteritis (also called giant cell arteritis) is complex and can affect medium and large size arteries, but commonly strikes the temporal artery causing temporal located headaches. In affected arteries there is an abnormal reaction that causes the infiltration of immune cells, such as lymphocytes, multinucleated giant cells, and plasma cells. Frequently the arteries in the head and neck are involved, but vasopathy can extend to the carotids and aorta. The abnormal mechanism is a cell-mediated immune response that is abnormally directed on an antigen (a foreign protein) near the elastic tissue component of an arterial wall. This immune response causes an infiltration of immune cells in an artery which could damage or even completely block the affected blood vessel. The exact cause of temporal arteritis (TA) is unknown. TA can be serious in cases where there is involvement of blood vessels that supply blood to the affected eye (i.e., posterior ciliary artery a branch of the ophthalmic artery) which can cause visual impairment.

Demographics

The disorder is more commonly observed in persons older than 50 years. TA occurs frequently with the occurrence ranging from 10 in 100,000 to 50 in 100,000. Internationally, there seems to be a higher incidence in countries higher in northern climates. The disorder occurs more frequently in Caucasian persons of northern European descent. TA rarely occurs in Blacks and Asians and it is four to six times more frequent in women than men. The mean age of onset is 70 years and the disorder is rarely seen in persons younger than 50 years. Long term survival is the same as for the general population. Visual loss is the most worrisome complication and can occur in over 50% of persons who are untreated, which could result in blindness for 20–50% of these patients.

Causes and symptoms

The cause of TA is not known. It is thought to be due to an immune cell response that attacks a foreign chemical (called an antigen) in the elastic layer of arteries in the head and neck.

In over 85% of affected persons, the most universal symptom is headache. The headache is severe and tends to be on one side (unilateral), and worsens at night. The pain tends to increase as days go by. Visual impairment may be the first presenting symptom since approximately 50% of patients complain of a sudden and painless visual loss. Loss of vision may be transient or permanent and blindness can occur if the condition is untreated.

In approximately 65% of persons, jaw claudication is prominent when chewing, swallowing or talking. Patients may have low grade fever and the effected arteries may be tender, warm, pulseless, dilated and thickened. Other symptoms that can occur include cough, anorexia, muscle aches, malaise, difficulty hearing, fatigue, fever/sweats and depression.

Diagnosis

Criteria for the diagnosis of TA were established in 1990 by the American College of Rheumatology. A diagnosis based on criteria for giant cell arteritis includes the presence of three of the following five items:

• new onset of headache or localized pain in the head region
• temporal artery tenderness to palpation
• development of symptoms in a person over 50 years of age
• lab result of over 50 for a special test called the Westergren Erythrocyte Sedimentation rate (WESR)
• decreased pulsations in head arteries, which cannot be attributed to arteriosclerotic disease of neck (cervical) arteries

A definitive diagnosis is made by the temporal artery biopsy which can be performed as an outpatient procedure (same day surgery).

Blood tests may reveal a high white blood cell count (leukocytosis), mild anemia or an increase in platelet cells (thrombocytosis), which are responsible for blood coagulation. Approximately 50% of patients affected with temporal arteritis have abnormal liver function tests. Chest radiograph may be useful to detect involvement of a chest (thoracic) artery. Ocular pneumoplethysmography can help make the diagnosis of temporal arteritis. Multiple biopsies may be indicated if initial findings are negative, but the suspicion for this diagnosis remains high.

Treatment team

The condition can be diagnosed by a primary care provider. Consultations may be indicated with an ophthalmologist (if there are visual complications). Generally an internist or rheumatologist directs the general care for systemic symptoms.

Treatment

Oral steroids are effective treatment for TA. Treatment is critical and important to avoid vision loss. Treatment should be initiated based on clinical suspicion and should not be delayed for biopsy results. The use of steroid or prednisone can be initially given at a dose of 60 to 100 mg per day. The dose can be tapered down in an individualized manner at a rate of approximately 10% per week, while concurrently taking into account symptomatic state and lab result improvement.

Recovery and rehabilitation

Most patients can be treated on an outpatient basis, with steroids, and symptoms usually begin to resolve within one to three days. Patients may require oral steroid medication for up to one year, depending on individual response. Nonsteroidal anti-inflammatory drugs may provide some pain relief.

Clinical trials

A clinical trial sponsored by the Cleveland Clinic Foundation Hospital and the National Institute of Health concerning the treatment of TA. The study is a multi-institutional project which includes medical centers within the United States and in several countries overseas. Full details can be obtained from the website: .

Prognosis

The condition is self-limiting and can last up to two years. Treatment with corticosteroids produces relief of symptoms and can help with visual impairment.

Special concerns

A diagnosis of TA can be missed. The disorder should be suspected in older patients with a high erythrocyte sedimentation rate (ESR), even if other evidence is absent.

Resources

BOOKS

Goetz, Christopher G., et al., eds. Textbook of Clinical Neurology. 1st ed. Philadelphia: W. B. Saunders Company, 1999.

Goldman, Lee, et al. Cecil's Textbook of Medicine. 21st ed. Philadelphia: W. B. Saunders Company, 2000.

Noble, John., et al., eds. Textbook of Primary Care Medicine. 3rd ed. St. Louis: Mosby, Inc., 2001.

WEBSITES

American Heart Association. http://www.americanheart.org.

ORGANIZATIONS

National Heart, Lung, and Blood Institute, Building 31, Room 5A52. 31 Center Drive MSC 2486, Bethesda, MD 20892. (301) 592-8573; Fax: (301) 592-8563. nhlbiinfo@nhlbi.nih.gov. http://www.nhlbi.nih.gov.

Laith Farid Gulli, MD

Robert Ramirez, DO

Alfredo Mori, MBBS

A progressive inflammatory disorder of cranial blood vessels, principally the temporal artery, occurring most frequently in women over 70 years of age. The temporal artery is typically tender, swollen, and pulseless. Symptoms are intractable headache, difficulty in chewing, weakness, rheumatoid pain, and loss of vision if the central retinal artery becomes occluded.

Giant cell arteritis
Classification and external resources
The arteries of the face and scalp.
ICD-10	M31.5-M31.6
ICD-9	446.5
OMIM	187360
DiseasesDB	12938
eMedicine	neuro/592
MeSH	D013700

Giant cell arteritis (GCA) is an inflammatory disease of blood vessels (most commonly large and medium arteries of the head). It is a form of vasculitis.

The name (giant cell arteritis) reflects the type of inflammatory cell that is involved^[1] (as seen on biopsy).

The terms "Giant cell arteritis" and "temporal arteritis" are sometimes used interchangeably, because of the frequent involvement of the temporal artery. However, it can involve other large vessels (such as the aorta in "giant cell aortitis".^[2] Giant cell arteritis of the temporal artery is referred to as "temporal arteritis," and is also known as "Cranial arteritis" and "Horton's disease."^[3]:840

Contents 1 Associated conditions 2 Symptoms 3 Diagnosis 3.1 Physical exam 3.2 Laboratory tests 3.3 Biopsy 3.4 Imaging studies 4 Treatment 5 References 6 External links

Associated conditions

The disorder may coexist (in one quarter of cases) with polymyalgia rheumatica (PMR), which is characterized by sudden onset of pain and stiffness in muscles (pelvis, shoulder) of the body and is seen in the elderly. GCA and PMR are so closely linked that they are often considered to be different manifestations of the same disease process. Other diseases related with temporal arteritis are systemic lupus erythematosus, rheumatoid arthritis and severe infections.

Symptoms

It is more common in females than males by a ratio of 3:1. The mean age of onset is about 70 years, and it is rare in those less than 50 years of age.

Patients present with:

• fever
• headache^[4]
• tenderness and sensitivity on the scalp
• jaw claudication (pain in jaw when chewing)
• tongue claudication (pain in tongue when chewing) and necrosis^[5]
• reduced visual acuity (blurred vision)
• acute visual loss (sudden blindness)
• diplopia (double vision)
• acute tinnitus (ringing in the ears)

The inflammation may affect blood supply to the eye and blurred vision or sudden blindness may occur. In 76% of cases involving the eye, the ophthalmic artery is involved causing anterior ischemic optic neuropathy.^[6] Loss of vision in both eyes may occur very abruptly and this disease is therefore a medical emergency.

Diagnosis

Physical exam

• Palpation of the head reveals prominent temporal arteries with or without pulsation.
• The temporal area may be tender.
• Decreased pulses may be found throughout the body.
• Evidence of ischemia may be noted on fundal exam.

Laboratory tests

• Erythrocyte sedimentation rate, an inflammatory marker, >60 mm/hour (normal 10-40 mm/hour), but may be normal in approximately 20% of cases.
• C-reactive protein, another inflammatory marker, is also commonly elevated.
• Platelets may also be elevated.

Biopsy

The gold standard for diagnosing temporal arteritis is biopsy, which involves removing a small part of the vessel and examining it microscopically for giant cells infiltrating the tissue. Since the blood vessels are involved in a patchy pattern, there may be unaffected areas on the vessel and the biopsy might have been taken from these parts. Unilateral biopsy of a 1.5-3 cm length is 85-90% sensitive. So, a negative result does not definitely rule out the diagnosis.

Imaging studies

Radiological examination of the temporal artery with ultrasound yields a halo sign. Contrast enhanced brain MRI and CT is generally negative in this disorder. Recent studies have shown that 3T MRI using super high resolution imaging and contrast injection can non-invasively diagnose this disorder with high specificity and sensitivity.^[7]

Treatment

Corticosteroids, typically high-dose prednisone (40–60 mg bd), must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy) to prevent irreversible blindness secondary to ophthalmic artery occlusion. Steroids do not prevent the diagnosis from later being confirmed by biopsy, although certain changes in the histology may be observed towards the end of the first week of treatment and are more difficult to identify after a couple of months.^[8] The dose of prednisone is lowered after a 2–4 weeks, and slowly tapered over the course of 9–12 months. Oral steroids are at least as effective as intra venous steroids,^[9] except in the treatment of acute visual loss where intravenous steroids appear to offer significant benefit over oral steroids ^[10]

References

1. ^ giant cell arteritis at Dorland's Medical Dictionary
2. ^ Walter MA, Melzer RA, Graf M, Tyndall A, Müller-Brand J, Nitzsche EU (May 2005). "[18FFDG-PET of giant-cell aortitis]". Rheumatology (Oxford) 44 (5): 690–1. doi:10.1093/rheumatology/keh551. PMID 15728420. http://rheumatology.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=15728420. 
3. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
4. ^ Moutray TN, Williams MA, Best JL (August 2008). "Suspected giant cell arteritis: a study of referrals for temporal artery biopsy" (PDF). Can. J. Ophthalmol. 43 (4): 445–8. doi:10.1139/i08-070. PMID 18711459. http://pubs.nrc-cnrc.gc.ca/cjo/cjo43/i08-070.pdf. 
5. ^ Sainuddin S, Saeed NR (December 2008). "Acute bilateral tongue necrosis – a case report". Br J Oral Maxillofac Surg 46 (8): 671-2. PMID 18499311. 
6. ^ Hayreh (April 3, 2003). "Ocular Manifestations of GCA". http://webeye.ophth.uiowa.edu/dept/GCA/04-ocular.htm. Retrieved 2007-10-15. 
7. ^ Bley TA, Uhl M, Carew J, et al. (October 2007). "Diagnostic value of high-resolution MR imaging in giant cell arteritis". AJNR Am J Neuroradiol 28 (9): 1722–7. doi:10.3174/ajnr.A0638. PMID 17885247. http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=17885247. 
8. ^ Font RL, Prabhakaran VC (2007). "Histological parameters helpful in recognising steroid-treated temporal arteritis: an analysis of 35 cases". The British journal of ophthalmology 91 (2): 204–9. doi:10.1136/bjo.2006.101725. PMID 16987903. 
9. ^ "BestBets: Steroids and Temporal Arteritis". http://www.bestbets.org/bets/bet.php?id=708. 
10. ^ Chan CC, Paine M, O'Day J.Br J Ophthalmol. 2001 Sep;85(9):1061-4.

External links

• Giant Cell Arteritis article at University of Iowa
• Polymyalgia rheumatica article from National Institute of Arthritis and Musculoskeletal and Skin Diseases

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