Arteritis - temporal; Cranial arteritis; Giant cell arteritis
Temporal arteritis is the inflammatory disease of blood vessels (most commonly large and medium blood vessels of the head). The name comes from the most frequently involved vessel (temporal artery which branches from the external carotid artery of the neck) and it is also named as giant cell vasculitis, indicating the type of cells infiltrating and damaging the vessels.
The disorder may coexist (in one quarter of cases) with another disorder called polymyalgia rheumatica, which is characterized by sudden onset of pain and stiffness in large joints (pelvis, shoulder) of the body and seen in the elderly. Other diseases related with temporal arteritis are systemic lupus erythematosus, rheumatoid arteritis and severe infections.
Temporal arteritis is more common in females and after 50 years of age. Patients present with fever, headache, tenderness and sensitivity on the scalp and recurrent jaw pain evoked especially by chewing. Most importantly, the inflammation may affect blood supply to the structures providing vision, and blurred vision or blindness may occur, accordingly. Palpation of the head reveals sensitive and thick arteries with or without pulsation. Sedimentation rate is very high in most of the patients (may be normal in approximately 20% of cases). The inflammation of the vessel must be demonstrated by removing a small part of the vessel (biopsy) and analyzing it for giant cells infiltrating the tissue. Since the 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. So, a negative result does not definitely rule out the diagnosis.
Loss of vision of both eyes may occur very abruptly and this disease is therefore an emergency. Corticosteroids must be started as soon as the diagnosis is suspected (even before the diagnosis is confirmed by biopsy).
Signs and symptoms of temporal arteritis include:
Pain and tenderness: A dull, persistent pain in the temples is the most common symptom of temporal arteritis. This pain is usually worsened by chewing. The face and scalp may also be painful and tender, and the pain may extend to the jaw and tongue. In addition to facial pain, a patient may experience ear pain or dental pain. Sore throats are common. In some cases, the muscles may ache.
Headache: Dull headaches occur in about two-thirds of all temporal arteritis cases. These may occur on one or both sides of the head and may be moderate to severe. Headaches may be localized to the temporal regions or may be more generalized. They are typically chronic and may get worse over time. Most patients suffering temporal arteritis report that the headache associated with it is markedly different from other headaches they have had.
Jaw claudication (pain when chewing): The chewing (masticatory) muscles may become weak and painful. This occurs in about half of temporal arteritis cases.
Abnormal temporal arteries: In about half of all cases of temporal arteritis, the temporal arteries develop a knobby thickening and become firm. They are generally tender to touch, and it may be difficult to feel a pulse within them.
Vision problems: Blurred or double vision is a frequent complaint in people with temporal arteritis. Patients may experience eye pain, but the visual impairment can be painless. Sudden loss of vision, usually in only one eye but sometimes in both, is a less common but serious risk. The loss of vision may be transient. According to the Arthritis Foundation, this blindness occurs in 10 to 15 percent of cases.
Fever: A fever may accompany temporal arteritis. Although typically low-grade, it may mimic the fever of an infection, such as pneumonia.
Fatigue: Many temporal arteritis patients suffer from general fatigue.
Weight loss: Temporal arteritis patients may experience a loss of weight.
Upper respiratory complaints: A dry cough and moderate to severe sore throat occur in some cases.
Horner's syndrome: This involves a drooping upper eyelid, pupil contraction and receding eyeball. It may occur with temporal arteritis.
Chest pain: Rarely, temporal arteritis is accompanied by chest pain, which suggests cardiovascular involvement.
Symptoms can also be due to other conditions, such as another form of vasculitis, Lyme disease
, systemic lupus erythematosus, rheumatoid arthritis, amyloidosis (abnormal deposit of proteins in tissues) or TMJ disorder. A biopsy is used to identify temporal arteritis.
In many cases, the disease patients also suffer from pain and stiffness in the neck, arms and hips, particularly in the morning. This is a symptom of polymyalgia rheumatica, a condition that commonly occurs with temporal arteritis.
Causes and Risk factors:
The exact etiology of this disease remains unknown. This disease is a chronic, systemic vasculitis primarily affecting the elastic lamina of medium- and large-sized arteries. Histopathology of affected arteries is marked by transmural inflammation of the intima, media, and adventitia, as well as patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells. Mural hyperplasia can result in arterial luminal narrowing, resulting in subsequent distal ischemia.
The temporal artery is commonly affected, often resulting in temporal-lobe headaches. Other commonly affected vessels include the ophthalmic, posterior ciliary, and, to a lesser extent, the central retinal artery. Inflammation in these locations can cause irreversible visual impairment and ischemic optic neuritis.
Despite increased understanding of the inflammatory cascade responsible for the disease process, the initial event that triggers the cascade remains uncertain. Although many infectious pathogens, such as Parvovirus B19 and Chlamydia species have been suggested as possible inciting agents, the actual role of microbial pathogens is still unclear.
Current theory regarding the etiology of this disease holds that a maladaptive response to endothelial injury leads to an inappropriate activation of cell-mediated immunity via immature antigen-presenting cells. The subsequent release of cytokines within the arterial vessel wall can attract macrophages and multinucleated giant cells, which gives diseased vessels their characteristic histology. This also leads to an oligoclonal expansion of T-cells directed against antigens in or near the elastic lamina. Ultimately, this cascade results in vessel wall damage, intimal hyperplasia, and eventual stenotic occlusion.
These inflammatory changes are also seen in polymyalgia rheumatica (PMR). Polymyalgia rheumatica and temporal arteritis are closely related inflammatory conditions, and it is suggested that they may be slightly different manifestations of the same underlying disease process. The symptoms of polymyalgia rheumatica are more systemic, including pain and stiffness in the shoulder and pelvic musculature, as well as fever, malaise, and weight loss. The relationship between polymyalgia rheumatica and temporal arteritis warrants consideration as it has been estimated that approximately half of patients initially presenting with temporal arteritis have been found to also have polymyalgia rheumatica. Conversely, about 10% of patients initially presenting with polymyalgia rheumatica were found to have temporal arteritis upon further investigation.
The etiology of this disease is multifactorial, as both genetic and environmental associations have been identified. Some major histocompatibility complex molecules, particularly human leukocyte antigen HLA-DR4 and HLA-DRB104 alleles may have a role in a patient's susceptibility to temporal arteritis.
Although the cause of temporal arteritis is not known, many factors are known to increase an individual's risk of developing the disease. These risk factors include:
Age: This may be the most important risk factor for temporal arteritis. This condition almost exclusively affects people over the age of 50. The likelihood of developing temporal arteritis increases as patients age and peaks in their 80s. Temporal arteritis can develop in children, according to the Arthritis Foundation, but this is exceedingly rare.
Sex: Women are twice as likely as men to develop temporal arteritis.
Genetics: There seems to be a genetic predisposition to temporal arteritis, which tends to run in families. In addition, it primarily affects people of Northern European descent.
Climate: This disease is more prevalent in colder latitudes. However, it is not known if this is because of the cold weather or the genetic makeup of the people living in those areas.
Impairment of the immune system: An abnormal immune response appears to be a factor in temporal arteritis. Severe infections or heavy use of antibiotics may increase risk of the disorder.
Viral factors: Some studies have suggested a link to pneumonia and parvovirus, but this remains highly controversial.
There seems to be a link between temporal arteritis and polymyalgia rheumatica. According to the National Institutes of Health, about 15 percent of Americans who have polymyalgia rheumatica also develop temporal arteritis. Further, about half of all temporal arteritis patients also have polymyalgia rheumatica. Patients may develop these conditions at the same time or may develop temporal arteritis after symptoms of polymyalgia rheumatica disappear.
People with rheumatoid arthritis may also be at an increased risk of temporal arteritis. Researchers have found that women who smoke or have cardiovascular problems such as murmurs might also be at greater risk of developing this disease.
When temporal arteritis is suspected, the doctor will order blood tests including a erythrocyte (red blood cell) sedimentation rate (ESR) and C-reactive protein test.
Erythrocyte sedimentation rate (ESR) is a nonspecific marker of inflammation. It is the most commonly used laboratory test in diagnosing temporal arteritis. Most patients with temporal arteritis have an ESR greater than 80 mm/h. However, up to 20% of patients with temporal arteritis may have a normal or low ESR, and thus a normal ESR level can not exclude a diagnosis of temporal arteritis.
C-reactive protein (CRP) is an acute-phase protein released by hepatocytes in inflammatory states. CRP has been found to be elevated (>2.45 mg/dL) in patients with temporal arteritis, even in patients with a normal ESR. An advantage to CRP is that the normal value range does not vary with age or sex. A normal CRP is less than 0.5 mg/dL. An elevated CRP may help to make the diagnosis when taken under consideration with a normal ESR
Complete blood cell count (CBC) may reveal leukocytosis, anemia, or thrombocytosis. Several studies have documented an association between an elevated platelet count >400 X 103/L and temporal arteritis. However, this test is not sufficiently sensitive or specific to be useful in the diagnosis of temporal arteritis.
Elevated liver function test (LFT) results, particularly alkaline phosphatase, are obtained in about one half of patients with temporal arteritis.
Several imaging tests may be used in the diagnosis of temporal arteritis. These include:
Ultrasound. Imaging using high-frequency sound waves. May detect swelling of temporal arteritis. Doppler ultrasound may be used to detect narrowing and decreased blood flow of the arteries in the head.
Cerebral angiography. A form of minimally invasive x-ray where a flexible tube (catheter) is inserted into an artery and threaded into the cerebral arteries in the brain. A contrast medium is injected and x-rays are taken as the medium fills the arteries. This reveals arterial blood flow and may be used to detect reductions in that flow.
CAT scan (computed axial tomography) or MRI (magnetic resonance imaging). May detect involvement of large arteries. These tests also have the capacity to follow changes in the thickness of arterial walls that cannot otherwise be detected.
Prehospital Care: Patients generally do not present via emergency medical services, and no particular prehospital interventions are warranted.
Emergency Department Care: Optimal care of patients with temporal arteritis in the emergency department involves maintaining a high index of suspicion and a low threshold to treat.
Treatment consists of corticosteroids. Although corticosteroids are the only proven treatment of temporal arteritis, few studies exist regarding dosing protocols. It is generally agreed that patients with suspected temporal arteritis should be started on oral prednisone 60 mg/day in the emergency department, with a temporal artery biopsy performed as an outpatient procedure scheduled within one week.
Improvement of systemic symptoms typically occurs within 72 hours of initiation of therapy. Patients should be counseled that corticosteroid therapy may be lengthy (1-2 y) and can lead to the typical complications associated with long-term steroid use. Recent data suggest that initial high-dose intravenous corticosteroid administration is beneficial in reducing temporal arteritis remission rates. However, further study is warranted before this is routinely practiced.
Consultations: An ophthalmologist should be consulted for a complete, dilated ocular examination to rule out other causes of vision loss, particularly when the diagnosis is uncertain.
A rheumatologist or internist should direct follow-up care for these patients, monitor remissions and recurrence, and manage complications associated with long-term corticosteroid therapy
Medicine and medications:
- Methotrexate (Trexall).
- Ibuprofen (Motrin).
- Naproxen (Naprelan '500').
- Cyclophosphamide (Cytoxan).
Over the Counter Medications for Temporal Arteritis.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
- Aspirin (Bayer Aspirin).
- Ibuprofen (Midol IB).
- Naproxen (Aleve).