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Temporal Arteritis

Giant Cell Arteritis

Giant-cell arteritis (temporal arteritis, cranial arteritis, Horton disease) is an inflammatory disorder of cranial blood vessels, principally the temporal artery. It is characterized by headache, jaw claudication and reduced visual acuity.


Presentation

In individuals with GCA, the most commonly presented conditions include headache, fever, bruits, tenderness and sensitivity of the scalp, claudication of the jaw, claudication of the jaw and necrosis, reduction in visual acuity, acute visual loss, diplopia, acute tinnitus and polymyalgia rehumatica in 50% of the cases [7].

With the inflammation, the supply of blood to the eye will be affected and this is the main cause of blurred vision and sudden blindness.

In most cases involving the eye, arteritic anterior ischemic optic neuropathy is caused due to the involvement of the ophthalmic artery. Since loss of vision in both eyes can happen abruptly, this case is regarded as a medical emergency.

Pain
  • An 81-year-old woman with limited mouth opening and pain upon chewing was referred to the Orofacial Pain Clinic at Nihon University Dental Hospital.[ncbi.nlm.nih.gov]
  • A 75-year-old woman presented with pain on both sides of the head. The pain occurred in the form of agonizing attacks 2-3 times a week. Accompanying neck pain was present, together with continuous lethargy and fatigue.[ncbi.nlm.nih.gov]
  • , and pain and weakness of the femoral muscles.[ncbi.nlm.nih.gov]
  • The patients have a burning or throbbing type of pain. Ultimately, there is localized inflammation or cellulitis over the swollen, tortuous artery.[ncbi.nlm.nih.gov]
  • Jaw pain Tongue pain Shoulder and hip pain Pain and stiffness in the neck Muscle aches Flu-like symptoms Excessive sweating General ill feeling Loss of appetite Sensitive, tender scalpc Weakness or excessive tiredness Weight loss Mouth sores Dry cough[migraine.com]
Fever
  • A 76-year-old woman with pulmonary asbestosis was admitted with fever and polymyalgia. She subsequently developed a visual disorder, hemoptysis, and hemoperitoneum. A biopsy of the temporal artery revealed the presence of giant-cell arteritis.[ncbi.nlm.nih.gov]
  • It presents with headache, fever, elevated erythrocyte sedimentation rate and anemia. If untreated, complications like ischemic optic neuritis may even lead to blindness.[ncbi.nlm.nih.gov]
  • We report the case of a 70-year-old patient who presented with fever of unknown origin. The initial diagnosis was infective endocarditis as a mitral valve vegetation was found in a transesophageal echocardiogram.[ncbi.nlm.nih.gov]
  • The patient was a 48-year-old male who presented with a 2-week history of fever, diffuse abdominal pain, and malaise.[ncbi.nlm.nih.gov]
  • […] tissue disease: Necrotizing vasculitides Giant-cell or temporal arteritis occurs chiefly in older people and is manifested by severe temporal or occipital headaches (in the temples or at the back of the head), mental disturbances, visual difficulties, fever[britannica.com]
Weight Loss
  • He also suffered body weight loss, general malaise and many typical manifestations of giant cell arteritis, such as jaw claudication, a tender, non-pulsating engorgement of his temporal arteries, and a highly elevated erythrocyte sedimentation rate and[ncbi.nlm.nih.gov]
  • Symptoms may be accompanied by vision disorder, subfebrile temperature, fatigue, lack of appetite, weight loss, sweating, and joint pains. While cortisone therapy reduces the symptoms, its effects in terms of improving quality of life are limited.[ncbi.nlm.nih.gov]
  • loss and fatigue Main risk is that inflammation of the arteries to the eye causes irreversible blindness, so again must be treated promptly Diagnosis: Think of it![ole.bris.ac.uk]
  • loss Mouth sores Dry cough, throat pain Bulging, thick, tender artery on one side Confusion or forgetfulness leading to dementia Depression Stroke[migraine.com]
Fatigue
  • Accompanying neck pain was present, together with continuous lethargy and fatigue. Physical examination revealed decreased cutaneous elasticity and muscle mass, and a depressive mood state was present.[ncbi.nlm.nih.gov]
  • […] arteritis : arterial inflammation that often involves the temporal arteries and may lead to blindness when the ophthalmic artery and its branches are affected, is characterized by the formation of giant cells, and may be accompanied by fever, malaise, fatigue[merriam-webster.com]
  • Association with polymyalgia rheumatica Characteristic symptoms: Headache, often wakes at night Tenderness over the temporal arteries (check you know how to feel for this pulse) Jaw pain when chewing (claudication) Systemic symptoms such as weight loss and fatigue[ole.bris.ac.uk]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: November 26, 2018[patientslikeme.com]
Anemia
  • It presents with headache, fever, elevated erythrocyte sedimentation rate and anemia. If untreated, complications like ischemic optic neuritis may even lead to blindness.[ncbi.nlm.nih.gov]
  • Neurological examination disclosed chin hypoesthesia while investigations showed a normocytic anemia, ESR of 100, and CRP of 72. A CT brain scan, chest X-ray, and bone scan showed no evidence of malignancy.[ncbi.nlm.nih.gov]
  • […] disease: Necrotizing vasculitides Giant-cell or temporal arteritis occurs chiefly in older people and is manifested by severe temporal or occipital headaches (in the temples or at the back of the head), mental disturbances, visual difficulties, fever, anemia[britannica.com]
  • Tests of the red blood cells may show mild anemia. Sometimes blood tests for liver function will also be abnormal. The definitive diagnostic test is a temporal artery biopsy.[encyclopedia.com]
  • Also, since TA is associated with inflammation, it may cause symptoms such as fatigue, anemia, weight loss, and general malaise.[news-journalonline.com]
Loss of Appetite
  • Definition / general Sudden loss of vision in one eye, low grade fever, loss of appetite and general weakness May be due to obstruction of posterior ciliary artery Microscopic (histologic) description Granulomatous inflammation of arteries with discontinuous[pathologyoutlines.com]
  • […] of appetite Sensitive, tender scalpc Weakness or excessive tiredness Weight loss Mouth sores Dry cough, throat pain Bulging, thick, tender artery on one side Confusion or forgetfulness leading to dementia Depression Stroke[migraine.com]
  • […] of appetite or weight loss Some of these symptoms may be related to other medical conditions, such as polymyalgia rheumatica (which causes severe aches and pain in large muscle groups, especially in the hips and shoulders).[my.clevelandclinic.org]
  • Giant Cell Arteritis Symptoms Early symptoms of giant cell arteritis may resemble flu symptoms such as fatigue, loss of appetite and fever.[arthritis.org]
  • Generalized symptoms associated with giant cell arteritis include fatigue, loss of appetite, weight loss, flu-like sensations, and fevers that are prolonged or recurrent. Jaw pain or facial, tongue, or throat pain is possible but less common.[verywell.com]
Jaw Claudication
  • Clinical features are headache, visual loss, ophthalmoplegia, jaw claudication, temporal headache, with tenderness and thickening on the affected temporal artery. We present 3 cases of tongue necrosis due to this granulomatous arteritis.[ncbi.nlm.nih.gov]
  • Classic symptoms include polymyalgia rheumatica, new-onset headache, jaw claudication, and visual symptoms such as diplopia and amaurosis fugax.[ncbi.nlm.nih.gov]
  • Clinical manifestations may vary from the classic constellation of temporal headache in the elderly accompanied by constitutional signs, jaw claudication, and visual symptoms; therefore, a high index of clinical suspicion may be necessary to identify[ncbi.nlm.nih.gov]
  • He also suffered body weight loss, general malaise and many typical manifestations of giant cell arteritis, such as jaw claudication, a tender, non-pulsating engorgement of his temporal arteries, and a highly elevated erythrocyte sedimentation rate and[ncbi.nlm.nih.gov]
  • Jaw claudication, eye pain, photophobia, diplopia, and even blindness may accompany the temporal symptoms. As many as 20% to 60% of inadequately treated or untreated patients will lose their vision.[ncbi.nlm.nih.gov]
Vascular Disease
  • The possibility of infectious triggers stimulating the development of inflammatory vascular diseases has generated much recent interest.[ncbi.nlm.nih.gov]
  • Key points • Temporal arteritis is a systemic vascular disease in which inflammation, usually with giant cells, affects the internal elastic lamina, adventitia, and adjacent media of medium and large arteries with a predilection for those in the head[medlink.com]
  • disease of unknown origin, occurring in the elderly, characterized by severe headache, fever, and accumulation of giant cells in the walls of medium-sized arteries, especially the temporal arteries.[medical-dictionary.thefreedictionary.com]
  • Thoracic aortic aneurysms have also been related to systemic vascular diseases such as temporal arteritis. Laboratory Studies The ESR is the most useful serum laboratory test for diagnosing polymyalgia rheumatica and temporal arteritis.[aafp.org]
Temporal Artery Pain
  • artery pain, mononeuropathy Polymyalgia rheumatica – may occur in up to 50% of patients Manifests with severe stiffness and pain in shoulders, thighs, and buttocks Dermatologic, renal, pulmonary – rarely involved ARUP Lab Tests Tests generally appear[arupconsult.com]
  • Meta-analyses of several studies have determined that both jaw claudication and diplopia are the most diagnostically powerful indicators of GCA. [55] [56] Furthermore, temporal artery abnormalities such as temporal artery pain and an abnormal temporal[eyewiki.aao.org]
Palpitations
  • Palpitations in the head denote temporal arteries regardless of presence of pulsation. In many cases, the temporal area remains tender. All around the body, decreased pulses may be found.[symptoma.com]
Shoulder Pain
  • Under the new criteria developed by the American College of Rheumatology and The European League Against Rheumatism, patients ages 50 years and older can be classified as having PMR if they meet the conditions below: Shoulder pain on both sides Morning[webmd.com]
  • pain and/or stiffness Less than two weeks from onset of symptoms to maximal symptoms ESR greater than 40 mm per hour Morning stiffness lasting longer than one hour Patient older than 65 years Depression and/or weight loss Bilateral upper arm tenderness[aafp.org]
  • Symptoms of PMR include bilateral aching neck, shoulder pain or stiffness and pelvic pain or stiffness. Additionally, many patients with PMR note constitutional symptoms such as fever, weight loss, and anorexia.[eyewiki.aao.org]
Myalgia
  • The following clinical findings were essential for the diagnosis: advanced or old age of the patient, massive headache in the temporal and/or occipital regions, myalgia primarily in the shoulders and the neck that responded relatively poorly to treatment[ncbi.nlm.nih.gov]
  • Arteritis Symptom or complaint Number of patients Presenting symptom Finding at diagnosis Headache 32 68 Polymyalgia rheumatica 25 39 Fever 15 42 Visual symptoms without loss of vision 7 30 Weakness, malaise, fatigue 5 40 Tenderness over arteries 5 27 Myalgias[aafp.org]
  • Distinct tongue numbness and vertigo have also been reported. [37] Myalgias, especially of the proximal muscles, are also associated with GCA.[eyewiki.aao.org]
Hip Pain
  • pain Pain and stiffness in the neck Muscle aches Flu-like symptoms Excessive sweating General ill feeling Loss of appetite Sensitive, tender scalpc Weakness or excessive tiredness Weight loss Mouth sores Dry cough, throat pain Bulging, thick, tender[migraine.com]
  • pain Absence of swelling in the small joints of the hands and feet, and absence of positive blood tests for rheumatoid arthritis Continued The new classification criteria may also help to evaluate existing treatments for polymyalgia rheumatica.[webmd.com]
Arthralgia
  • […] often involves the temporal arteries and may lead to blindness when the ophthalmic artery and its branches are affected, is characterized by the formation of giant cells, and may be accompanied by fever, malaise, fatigue, anorexia, weight loss, and arthralgia[merriam-webster.com]
  • In NAION, patients will usually not note any associated systemic symptoms such as headache, jaw claudication, scalp tenderness, weight loss, anorexia, fever, or myalgias/arthralgias.[eyewiki.aao.org]
Diplopia
  • Classic symptoms include polymyalgia rheumatica, new-onset headache, jaw claudication, and visual symptoms such as diplopia and amaurosis fugax.[ncbi.nlm.nih.gov]
  • Jaw claudication, eye pain, photophobia, diplopia, and even blindness may accompany the temporal symptoms. As many as 20% to 60% of inadequately treated or untreated patients will lose their vision.[ncbi.nlm.nih.gov]
  • Fifteen days before, she complained of diplopia. She had doubtful symptoms of giant cell arteritis and showed a normal physical exam.[ncbi.nlm.nih.gov]
  • Patients with jaw claudication, diplopia, and an abnormal temporal artery on examination are more likely to have a temporal artery biopsy that is positive for GCA. GCA is unlikely if levels of inflammatory markers are normal.[bestpractice.bmj.com]
  • […] occlusive vasculopathy Granulomas form in arterial media Often associated with polymyalgia rheumatica Clinical Presentation Constitutional – fever, fatigue, malaise, weight loss Otorhinologic – jaw claudication Ophthalmologic – transient visual changes, diplopia[arupconsult.com]
Eye Pain
  • Jaw claudication, eye pain, photophobia, diplopia, and even blindness may accompany the temporal symptoms. As many as 20% to 60% of inadequately treated or untreated patients will lose their vision.[ncbi.nlm.nih.gov]
  • Pain and stiffness in the neck, shoulders or hips are common symptoms of a related disorder, polymyalgia rheumatica.[mayoclinic.org]
  • Other than vision loss, patients may complain of diplopia, eye pain, or symptoms from cranial neuropathies (i.e. ptosis , anisocoria, diplopia).[eyewiki.aao.org]
Scotoma
  • The affected eye will often show an altitudinal visual field defect, but arcuate and cecocentral scotoma’s have also been reported. Case History Perform a thorough and careful case history when confronted with an acute ischemic optic neuropathy.[reviewofoptometry.com]
  • […] exertion Audible pulsations or bruits over the axillary region Cramping or aching legs while walking which improves with resting Dry cough Double vision Other complications Limb claudication Transient ischemic attacks (TIAs) or stroke Scintillating scotoma[lecturio.com]
Unilateral Blindness
  • Visual symptoms of diplopia, blurred vision, or amaurosis fugax may antedate development of sudden unilateral blindness. Many patients describe a fullness or pressure sensation behind their eyes.[rheumaknowledgy.com]
Tenderness of the Scalp
  • Other symptoms may include: Fatigue Fever Jaw pain that may become worse after chewing Tenderness at the scalp or temples Vision problems, such as double vision, blurry vision , or transient (brief) vision loss; if this is not treated, it could be followed[my.clevelandclinic.org]
  • Symptoms specifically related to the inflamed arteries of the head include: Headaches, often severe Tenderness of the scalp or temples Double vision Temporary or sustained vision loss (like having a curtain pulled partly over the eye) Dizziness or problems[arthritis.org]
  • Temporal arteritis involves a variety of symptoms including tenderness in the scalp, headaches, pain in one or both temples, fatigue, double vision, neck pain, weight loss and pain in the jaw, especially while chewing.[kadrmaseyecare.com]
  • Because giant cell arteritis is commonly due to inflammation of the arterial blood vessels affecting the head, it frequently leads to headaches , pain in the jaw when repetitively chewing, and tenderness of the scalp (usually over the inflamed arteries[medicinenet.com]
  • Common symptoms New headache, usually in the temples Tenderness of the scalp Swelling of the arteries near the temples Vision changes like a curtain in the field of vision, sudden vision loss (temporary or permanent) or double vision.[vasculitisfoundation.org]
Facial Pain
  • Trigeminal neuralgia (TN) complicated with temporal arteritis (TA) is not a common disease, but it is a very important syndrome to consider for diagnosing facial pain in individuals older than 50 years.[ncbi.nlm.nih.gov]
  • Symptoms of temporal arteritis Headaches, throbbing pain on one side or in back of head Fever Vision changes , blurred vision or double vision Loss of vision in one or both eyes – which can be permanent Hearing loss Facial pain Joint pain or stiffness[migraine.com]
  • Baker for a consultation today: Disturbance in vision (blurred vision, double vision) Acute vision loss (sudden loss of vision) Fever Excessive sweating Throbbing headache General ill feeling Loss of appetite Facial pains Mouth sores Depression Unintentional[susanbakermd.com]
  • Associated Co-morbidities Polymyalgia Rheumatica (PMR) Visual Disturbances Facial pain Osteoporosis Hypokalemia Various infections such as oral/esophageal thrush Herpes Zoster [8] Medications High dose corticosteroids are the most widely accepted medication[physio-pedia.com]
Headache
  • Clinical features are headache, visual loss, ophthalmoplegia, jaw claudication, temporal headache, with tenderness and thickening on the affected temporal artery. We present 3 cases of tongue necrosis due to this granulomatous arteritis.[ncbi.nlm.nih.gov]
  • Three weeks later, a localized headache over her right superficial temporal artery was reported, and progressive bilateral blindness appeared. A biopsy showed a late stage of temporal arteritis.[ncbi.nlm.nih.gov]
  • We describe a 70-year-old woman with a 2-month history of a numb chin and gradually increasing bilateral headache and malaise.[ncbi.nlm.nih.gov]
  • Because of persistent headache after surgical excision of the lesion, the patient was treated with prednisolone.[ncbi.nlm.nih.gov]
  • Temporal arteritis is a form of vasculitis that involves the large- and medium-diameter arteries and leads to progressive headache.[ncbi.nlm.nih.gov]
Stroke
  • Ischemic stroke is a serious though relatively rare complication of GCA. Similar cases have been reported, in which ischemic stroke developed despite or possibly due to steroid therapy.[ncbi.nlm.nih.gov]
  • Blocked carotid artery can lead to stroke or TIA – transient ischemic attack any time in life.[lecturio.com]
  • With a mean follow-up of 5 years (range, 1-11 years), 6/39 (15%) of patients developed stroke or cardiovascular events or died compared with 7/39 (18%) of age-matched controls.[ncbi.nlm.nih.gov]
  • Some studies show that taking aspirin may reduce the risk of vision loss, stroke, and mini-strokes. In some people, symptoms improve with treatment but then come back. This is called a relapse .[northshore.org]
  • Studies have shown that low-dose aspirin can reduce the risk of stroke and visual loss in patients with giant cell arteritis.[medicinenet.com]
Dizziness
  • A variety of systemic symptoms are also often present, including nausea, vomiting, chills, dizziness, and loss of weight. Temporal arteritis is not a common diagnosis in maxillofacial practice.[ncbi.nlm.nih.gov]
  • Symptoms specifically related to the inflamed arteries of the head include: Headaches, often severe Tenderness of the scalp or temples Double vision Temporary or sustained vision loss (like having a curtain pulled partly over the eye) Dizziness or problems[arthritis.org]
  • It's also possible to experience dizziness or problems with balance. Giant cell arteritis can affect the blood supply to the eye causing blurred vision, double vision, or blindness.[verywell.com]
  • […] to seek medical advice Call your healthcare provider right away if any of these occur: Worsening symptoms Numbness or weakness of the face, one arm, or one leg Slurred speech, confusion, or trouble speaking or walking Severe headache Fainting spell, dizziness[fairview.org]
  • , stomach, or arm Shortness of breath Nausea or vomiting Lightheadedness or a sudden cold sweat You have any of the following signs of a stroke: Numbness or drooping on one side of your face Weakness in an arm or leg Confusion or difficulty speaking Dizziness[drugs.com]
Confusion
  • Acute symptoms may be confused with stroke Initiate appropriate monitoring and oxygen Patients may be hypotensive from 1 of the rare sequelae (aortic dissection, abdominal aortic a...[5minuteconsult.com]
  • […] stiffness in the neck Muscle aches Flu-like symptoms Excessive sweating General ill feeling Loss of appetite Sensitive, tender scalpc Weakness or excessive tiredness Weight loss Mouth sores Dry cough, throat pain Bulging, thick, tender artery on one side Confusion[migraine.com]
  • Diseases, www.niams.nih.gov The Arthritis Foundation, www.arthritis.org When to seek medical advice Call your healthcare provider right away if any of these occur: Worsening symptoms Numbness or weakness of the face, one arm, or one leg Slurred speech, confusion[fairview.org]
  • Discomfort or pain in your back, neck, jaw, stomach, or arm Shortness of breath Nausea or vomiting Lightheadedness or a sudden cold sweat You have any of the following signs of a stroke: Numbness or drooping on one side of your face Weakness in an arm or leg Confusion[drugs.com]
  • Diagnosis of temporal arteritis and polymyalgia rheumatica Temporal arteritis and arthritis are not the same disorder and should not be confused. A person can have temporal arteritis or giant cell arteritis and arthritis at the same time.[belmarrahealth.com]
Vertigo
  • Two days after prednisolone therapy was initiated, the patient began to experience transient vertigo attacks. Two days later, dysarthria, left-sided hemiparesis, right abducens palsy, and horizontal nystagmus developed.[ncbi.nlm.nih.gov]
  • Sleep Apnea Splenomegaly Streptococcal Pharyngitis Stroke Stroke, Hemorrhagic Temporal Arteritis Thoracic Aortic Dissection Upper Gastrointestinal Bleed Urinary Incontinence, Women Urinary Tract Infection, Child Urinary Tract Infection, Women Vaginitis Vertigo[jamaevidence.mhmedical.com]
  • Compromise of the arteries supplying the otic region can lead to tinnitus, hearing loss and vertigo. Thoracic aortic aneurysms have also been related to systemic vascular diseases such as temporal arteritis.[aafp.org]
  • Distinct tongue numbness and vertigo have also been reported. [37] Myalgias, especially of the proximal muscles, are also associated with GCA.[eyewiki.aao.org]

Workup

Palpitations in the head denote temporal arteries regardless of presence of pulsation. In many cases, the temporal area remains tender. All around the body, decreased pulses may be found. During fundal examinations, evidence of ischemia may be noted [8].

Some of the laboratory tests that must be carried out include:

  • Platelets count (may be elevated)
  • C-reactive protein tests ( this may also be elevated)
  • ESR ( Erythrocyte sedimentation rate will show readings of >6omm/hour
  • Liver function tests (this will often show raised alkaline phosphatase)
Thrombocytosis
  • […] changes or new onset localized headache Elevated inflammatory marker(s) Criteria for Diagnosis Histologic diagnosis – temporal artery biopsy showing segmental inflammation Laboratory Testing Nonspecific testing – helpful in excluding other diagnoses CBC – thrombocytosis[arupconsult.com]
  • In the setting of clinical suspicion and a raised ESR, thrombocytosis has a relatively high specificity for distinguishing GCA from other diseases [7] .[physio-pedia.com]
  • Anemia of chronic disease, decreased serum albumin, polyclonal gammopathy, and reactive thrombocytosis are also seen. Elevation in liver tests, particularly alkaline phosphatase, is common.[rheumaknowledgy.com]
  • Laboratory testing may reveal elevated erythrocyte sedimentation rate (ESR), elevated C reactive protein (CRP), elevated alkaline phosphatase in one third of patients and, in 50% of patients, normocytic, normochromic anemia and thrombocytosis.[hss.edu]
  • Complete blood counts (CBC) may show a mild thrombocytosis. Anemia, usually caused by anemia of inflammation, is common in affected patients. Less common, elevations in liver transaminases or a low serum albumin may occur.[rheumatologyadvisor.com]
Elevated Sedimentation Rate
  • On lab testing, he had an elevated sedimentation rate (sed rate), which reflects inflammation in the body. His sed rate was 70 while a normal level is between 0 and 20.[headaches.org]

Treatment

Oral corticosteroids are highly and rapidly effective in temporal arteritis but are often associated with adverse effects.

Prognosis

Full recovery is the common result when there is adequate and prompt therapy. In recent years the reduced rate of neuro-ophthalmologic complications show a marked improvement in the recognition and treatment of the condition [9]. This explains why blindness can now be regarded as a rare complication.

If left untreated, the prognosis for individuals with this condition is very poor. Blindness and death is very common resulting from stroke, myocardial infarction and dissecting aortic aneurysm.

Etiology

The specific cause of these autoimmune disorders is yet to be documented. Parvovirus B19 and mycoplasma infection have been denoted as possible causes but this is yet to be proven [4].

Epidemiology

Between 1950 and 1975 in the USA, the distribution of GCA in Minnesota increased from 5.1 to 17.4 cases per 100,000 individuals each year in adults aged 50 years and above. The increase was attributed to improved rate of accurate diagnosis and clinical awareness [2]. By January 1, 1975 the prevalence was placed at 133 cases per 100,000 adults aged 50 and above.

Internationally, there have been few publications of epidemiological studies but the incidence rates do not vary greatly within the defined susceptible age bracket. The highest incidence can be traced to Scandinavia where 23-33 people per 100,000 are affected amongst people aged 50 and above.

The prevalence is heavily dependent on the number of individuals aged 50 years and above. The average age of onset is 75 years. Therefore, countries where there is lower life expectancy show a lower prevalence.

Although GCA can lead to MI, stroke or death it is yet to be associated with a statistically significant increase in any of these. GCA leads to death in generally 2% of cases. As is to be expected, the severity of the vasculitis and topographic extent is greater in fatal cases than what is obtainable in nonfatal cases.

It is however, difficult to quantify the number of patients who died indirectly or directly due to extensive use of corticosteroids [3].

There has not been any proven racial predisposition to GCA. However, the epidemiologic studies are limited due to the fact that they have mostly been performed on predominantly Caucasian population. This can signify that the condition is more common amongst this race.

The ration of occurrence in sexes is 3.7:1 against females. The average age for the onset of the condition is 75 years. The condition is the most common systemic vasculitis affecting elderly patients. It is rarely described in individuals younger than 40 years of age.

Sex distribution
Age distribution

Pathophysiology

The condition is generally a disease affecting cellular immunity. The CD4+ T helper cells reacting to an antigen presented by macrophages mediates vasculitic damage. The internal elastic lamina is affected by the primary inflammatory response. The hallmark of GCA is the multinucleated giant cells and these may contain elastic fiber fragments. The antigen that triggers the condition is still not known but elastin is suspected by experts [5].

In many patients, the superficial temporal artery is involved. This offers a convenient biopsy site. However the topographic distribution of the condition extends towards the aortic arch and its branches. This reflects its predilection for the internal elastic lamina.

Since intracranial arteries do not have an internal elastic lamina, GCA does not cause widespread intracranial cerebral vasculitis. As well as the vertebral and carotid arteries, GCA involves cervicocephalic arteries. Below is a look at how the condition affects arteries [6].

  • Internal, external and common internal carotid artery involvement occurs extracranially. On rare occasions, proximal intracranial segments get affected.
  • The involvement of the pulmonary artery has been described in many cases.
  • Ischemic mononeuropathies, crural infarction, renal infarction and intestinal infarction are common complications documented when the femoral, iliac, renal, mesenteric and the descending aorta arteries are affected. This is less often described.
  • Vertebral arteries are frequently involved and the superficial temporal arteries are affected in fatal cases. Basal artery involvement is rare however.
  • The posterior ciliary and ophthalmic arteries are intraorbital branches that are commonly affected.
  • Although vertebral arteritis is extracranial, it may extend intracranially beyond the dural penetration.
  • A characteristic angiographic pattern of vasculitis is produced when there is involvement of the subclavian, axillary and proximal brachial arteries.

Prevention

There are no guidelines for prevention of temporal arteritis.

Summary

Temporal Arteritis, often known as Giant-cell arteritis, GCA or cranial arteritis is a disorder that affects the blood vessels [1]. It is an inflammatory disease which mostly involves the large and medium arteries located in the head.

The branch of the external carotid artery is the most affected. The disorder is a form of vasculitis. Blindness is the most serious complication of GCA but this complication can be easily corrected using corticosteroids.

The condition derives its name from the inflammatory cell involved as displayed in a biopsy. Temporal arteritis and giant-cell arteritis are often used interchangeably due to the temporal artery’s frequent involvement.

However, the condition can affect larger vessels like the aorta, leading to giant cell aortitis. The condition is equally known as cranial arteritis and Horton’s disease.

Patient Information

Patients need to be fully aware of the seriousness of reoccurring symptoms. This will help them seek for quick medical attention when they come back. Secondly, the importance of adhering strictly to the steroid dose schedule must be emphasized [10].

With the right administration of steroids, the disease can be treated in 3 years. When necessary, dietary restrictions and other interventions will help reduce the incidence of adverse effects arising from the intake of steroids a great deal.

Make sure you don’t stop taking the steroids abruptly. Missing out on a dose may not trigger off anything but as soon as the body gets used to taking the steroids, stopping suddenly will bring about serious bouts of withdrawal effects in just a couple of days.
Unless your doctor says so, taking anti-inflammatory painkillers while taking the painkillers must be avoided. Taking these two at once may lead to the development of stomach ulcers. Therefore, apart from low-dose aspirin, you have to avoid other anti-inflammatory painkillers.

While on steroids, don’t forget to carry your steroid card regularly. This card details your condition, dosage and other valuable information that will come in handy in the event of an emergency.

Your dosage of steroids will be increased if you become ill with other conditions or undergo surgery. This is to provide your body with the right dosage of steroid needed to properly combat physical stress.

References

Article

  1. Cantini F, Niccoli L, Storri L, Nannini C, Olivieri I, Padula A. Are polymyalgia rheumatica and giant cell arteritis the same disease?. Semin Arthritis Rheum. Apr 2004;33(5):294-301.
  2. Borchers AT, Gershwin ME. Giant cell arteritis: A review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev. Jan 21 2012
  3. Eberhardt RT, Dhadly M. Giant cell arteritis: diagnosis, management, and cardiovascular implications. Cardiol Rev. Mar-Apr 2007;15(2):55-61.
  4. Nordborg C, Larsson K, Aman P, Nordborg E. Expression of the class I interferon-related MxA protein in temporal arteries in polymyalgia rheumatica and temporal arteritis. Scand J Rheumatol. Mar-Apr 2009;38(2):144-8.
  5. Rodríguez-Pla A, Bosch-Gil JA, Rosselló-Urgell J, Huguet-Redecilla P, Stone JH, Vilardell-Tarres M. Metalloproteinase-2 and -9 in giant cell arteritis: involvement in vascular remodeling. Circulation. Jul 12 2005;112(2):264-9.
  6. Sainuddin S, Saeed NR. Acute bilateral tongue necrosis – a case report. Br J Oral Maxillofac Surg 2008 46 (8): 671–2.
  7. Zadik Y, Findler M, Maly A, et al. A 78-year-old woman with bilateral tongue necrosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011 111 (1): 15–9.
  8. Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum 1998; 41:778.
  9. Myklebust G, Gran JT. A prospective study of 287 patients with polymyalgia rheumatica and temporal arteritis: clinical and laboratory manifestations at onset of disease and at the time of diagnosis. Br J Rheumatol 1996; 35:1161.
  10. Hall S, Persellin S, Lie JT, et al. The therapeutic impact of temporal artery biopsy. Lancet 1983; 2:1217.

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Last updated: 2018-06-22 05:45