Temporal (Giant Cell) Arteritis


Article Author:
William Gossman
Ryan Peterfy


Article Editor:
Babak Khazaeni


Editors In Chief:
Chaddie Doerr


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
7/11/2019 9:46:25 PM

Introduction

Temporal arteritis (TA), also called giant cell arteritis (GCA) or cranial arteritis, is a systemic inflammatory vasculitis of medium and large-sized arteries occurring most frequently in adults. This results in an ischemic optic neuropathy with irreversible or significant visual loss on the affected side with possible contralateral involvement. Left untreated, it can result in many systemic, neurologic, and ophthalmologic complications. Although the temporal artery is most commonly involved, other arteries may certainly be affected. These include the aorta and the subclavian, iliac, ophthalmic, occipital, and vertebral arteries. Although not necessary, the disease is commonly associated with polymyalgia rheumatica.[1][2][3]

Etiology

Although the exact etiology of GCA is currently unknown, various genetic, environmental, and autoimmune possibilities have been hypothesized. An association with Toll-like receptor 4 gene polymorphism as well as HLA-DR4 have been identified.[4][5][6]

Epidemiology

While TA generally occurs in people over the age of 50, the average age of onset is 75. Women are more commonly affected, almost four times more than men. It is a relatively rare disease, occurring in about 10 to 20 people over the age of 50 per year, depending on the nationality of the patient. It is more commonly found in people of European descent but may present in a patient of any ethnicity.[7][8]

Pathophysiology

As GCA is a disease characterized by cell-mediated immunity, the pathophysiology is thought to involve the body's inappropriate response to vascular endothelial injury. The initial insult to the vascular wall causes a massive release of cytokines, in turn attracting macrophages and multinucleated giant cells. This inflammatory cascade causes activation of CD4 helper T cells, causing further inflammation and hyperplasia. [9][10]

Histopathology

The biopsy of the artery will usually reveal the following features:

  • Luminal narrowing with intimal proliferation
  • Disruption of the internal elastic lamina
  • Giant cell formation with granulomas
  • Necrosis and panarteritis
  • Predominant cell type are monocytes

History and Physical

The patient will usually present with complaints of a headache, painless vision loss, jaw claudication, fatigue, fever, anorexia, or temporal artery tenderness. Patients may state that their headache has been occurring for a duration of 2 to 3 months and worsens with exposure to cold and at night when the pressure of the pillow causes pain to the artery. On physical exam, the temporal artery may be thickened, painful, nodular, or erythema may occur on the overlying skin. It is important to realize that the physical exam of the temporal artery may be inconclusive. In addition, the temporal artery may not necessarily be the artery involved which further complicates the diagnosis. Many patients will present with signs or symptoms of TIA or stroke. The ophthalmologic exam will generally be benign, although if the circulation of the optic nerve is involved, the patient may display an afferent pupillary defect. Because of the inconsistencies regarding the history and physical, temporal arteritis is often a difficult disease to diagnose in the emergency setting.

Evaluation

The American College of Rheumatology has developed a set of criteria for the diagnosis of temporal arteritis. Three of the five criteria must be present to make the diagnosis.[11][12][13] These include:

  • Age greater than or equal to 50 at the onset of disease
  • New headache
  • Temporal artery abnormalities such as tenderness of the superficial artery or decreased pulsation
  • ESR greater than or equal to 50 mm/h
  • Abnormal artery biopsy including vasculitis, a predominance of mononuclear cell infiltration or granulomatous inflammation, or multinucleated giant cells.

Due to the patchy nature of the disease in the affected artery, a biopsy may yield a false negative if not taken of adequate length. The recommended length is at least 1 cm, with 1.5 cm to 3 cm showing a sensitivity of 85% to 90%, to achieve a successful biopsy. An elevated CRP, although not required for diagnosis, may also be helpful. Although imaging has generally not been indicated in the diagnosis of temporal arteritis in the past, recent studies show ultrasound as a useful adjunct. If a dark halo is seen around the vessel, sensitivity approaches 80% to 100%. High-resolution 3T MRI has also been shown to be effective in some studies. Although the clinical picture may be helpful in the diagnosis, it is important to note that only a biopsy of the affected artery can give a definitive diagnosis.

Treatment / Management

The timing of treatment is critical in these patients to prevent vision loss. Steroids should be started immediately upon suspicion of temporal arteritis. Although there is no consensus among physicians regarding the amount of steroid therapy to begin, 60 milligrams of prednisone PO or 1 mg/kg daily can be used as a general guideline. This may be required for 1 to 2 years. The patient should be followed by their primary care physician with frequent ESR draws. If acute vision loss is present, the patient may be started on 250 mg to 1000 mg of intravenous (IV) steroids for 3 days. Low-dose aspirin should also be started daily. A rheumatologist should be consulted immediately upon starting steroid treatment and before performing a biopsy. An ophthalmologist should also see the patient to perform a full eye exam to rule out other serious causes of vision loss. If strong clinical suspicion is present, the biopsy may be performed by either a neurosurgeon, ophthalmologist, plastic surgeon, or general surgeon. Treatment with steroids must be started when the disease is suspected, rather than while awaiting biopsy results. Biopsy results may still be positive a week after beginning steroids. Patients may be safely discharged on oral steroids with close follow-up without hospital admission. Symptoms warranting admission include the inability of the patient to care for themselves or severe symptoms.[14][15]

Differential Diagnosis

  • Carotid artery disease
  • Sinusitis
  • Malignancy
  • Herpes zoster
  • Other Collagen vascular disorders
  • Post herpetic neuralgia
  • Rheumatoid arthritis

Pearls and Other Issues

Remember, the temporal artery may be normal on physical exam. This may be due to early disease or involvement of a vessel other than the temporal artery. During the physical exam, checking the intraocular pressure should be performed to rule out acute glaucoma. In a patient with a new headache and an elevated ESR, GCA should be considered. Hints to temporal artery tenderness include pain from wearing glasses or a hat. Transient episodes of recurring blurriness in one eye is usually an indicator of reversible disease, but once total monocular vision loss occurs, this is usually permanent. Temporal arteritis is a diagnosis that is seldom made in the emergency department. Patients who present to the ED with headaches and improve after analgesia are often discharged home and are misdiagnosed. Palpation of the temporal artery should become a part of every provider's physical exam on patients who present with a headache.

Enhancing Healthcare Team Outcomes

The management of temporal arteritis is with a multidisciplinary team that consists of an internist, neurologist, rheumatologist, an ophthalmologist, a nurse, pharmacist and surgeon. Education is the most important step as patients need to know the complications of this disorder and the need for close monitoring. All patients should be urged to follow up with an ophthalmologist to ensure that vision loss is not occurring. Anyone with weakness, loss of vision, difficulty with gait, dysphagia or speech problems should immediately seek medical assistance. The pharmacist should educate the patient on corticosteroid compliance and the potential side effects. All patients should be told that despite treatment they may develop problems with other blood vessels in the future. [16][17](Level V)

Outcomes

For the majority of patients, who get prompt treatment, there is a complete recovery. Symptomatic improvement occurs in 2-4 days after treatment. To avoid the adverse effects of the corticosteroids, tapering is recommended after 4-6 weeks. Blindness from temporal arteritis is very rare today. However, the course of the disease does vary from patient to patient and may last 3 months to 5 years. The biggest problem with the treatment of temporal arteritis today is the morbidity associated with the corticosteroids. Individuals likely to require prolonged treatment with steroids include females, older age, and those with a higher baseline ESR. For those who are untreated, the prognosis is poor; these individuals may suffer from blindness, develop a stroke or an MI. Overall, about 1-3% of patients with temporal arteritis die from a stroke or an MI. [18](Level V)


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Temporal (Giant Cell) Arteritis - Questions

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A 71-year-old female presents with pain and a throbbing, right-sided headache. Examination reveals a tender temporal artery, and a biopsy is immediately done. What are the classic histopathology findings?



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A 68-year-old male presents to the clinic with general malaise and a low-grade fever. He says he was told in the emergency room a few days ago that he had vasculitis and that the physician started him on corticosteroids. A physical exam is unremarkable. What is the most common vasculitis in this age group?



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How sensitive is an elevated erythrocyte sedimentation rate for polymyalgia rheumatica/temporal arteritis?



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A 65-year-old female was treated with prednisone for polymyalgia rheumatica (PMR) for one year, followed by successful withdrawal of the medication. Four months later the patient develops weight loss, fatigue, left sided headaches, and subjective fevers. The patient is concerned about a visual change that started yesterday. The exam shows tenderness over the left temple and the left retina has a pale disc with blurred margins. The rest of the exam is normal. What is the most likely diagnosis?



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A patient with a history of polymyalgia rheumatica one year ago develops presumed temporal arteritis with loss of vision over the past 24 hours. What is the appropriate treatment for this patient?



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A 68-year-old patient diagnosed with temporal arteritis three years ago has been on steroids intermittently since the diagnosis. He is currently on 5 mg of prednisone daily. The patient presents to the emergency department with substernal "tearing" chest pain with radiation to the back. The left brachial pulse is reduced when compared to the right. Heart rate is 130 bpm and blood pressure is 90/60 mm Hg. Which of the following tests will most likely establish a diagnosis?



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Which of the following is not a cause of sudden, bilateral visual loss?



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Which is not a symptom associated with giant cell arteritis?



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A 65-year-old female presents complaining of a left-sided headache and has a history of brief vision loss in her left eye. Her neurologic and ophthalmologic exams are normal. She has no carotid bruits. She has tenderness over her left temple. Blood work reveals an elevated erythrocyte sedimentation rate. What is the most appropriate management?



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A 75-year-old presents with headache, jaw claudication, visual disturbances, and scalp tenderness. The ESR is elevated. What is the next step in this patient's management?



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In giant cell arteritis, which vessel is usually involved?



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Which ethnic group is most prone to giant cell arteritis?



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Which group is most commonly affected by giant cell arteritis?



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Which of the following about temporal arteritis is FALSE?



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Which of the following is frequently seen in patients with giant cell arteritis?



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A 72-year-old patient presents with complaints of a new, moderately diffuse headache, fever, and muscle pain. Blood work reveals elevated erythrocyte sedimentation rate over 120 millimeters/min. Which of the following is the most likely diagnosis?



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Which clinical feature is associated with temporal arteritis?



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Which is not a correct pairing of symptoms and pathology?



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An 80 year old patient presents with fever, decreased visual acuity, scalp tenderness, and very prominent temporal artery. What is the most likely diagnosis?



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A 68-year-old male has an ischemic stroke secondary to basilar artery occlusion and has subsequent ataxia and dysarthria. After acute hospitalization, while in rehabilitation, he complains of left temporal headaches and starts losing weight. He complains of pain in his left jaw with chewing. The patient is not doing well with physical therapy and the therapist reports proximal muscle weakness. Which of the following physical findings is most likely?



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A 68 year old male has an ischemic stroke secondary to basilar artery occlusion and has subsequent ataxia and dysarthria. After acute hospitalization, while in rehab, he complains of left temporal headaches and starts losing weight. He complains of pain at his left jaw with chewing. The patient is not doing well with physical therapy and the therapist reports proximal muscle weakness. What is the initial test for diagnosis?



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A 68-year-old male has an ischemic stroke secondary to basilar artery occlusion and has subsequent ataxia and dysarthria. After acute hospitalization, while in rehabilitation, he complains of left temporal headaches and starts losing weight. He complains of pain in his left jaw with chewing. The patient is not doing well with physical therapy, and the therapist reports proximal muscle weakness. Elevation of erythrocyte sedimentation rate is found. Which of the following tests should be done?



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Which of the following is not a possible cause of vision loss in temporal arteritis?



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Which of the following is an incorrect statement about temporal arteritis?



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Which of the following arteries is most likely affected by giant cell arteritis?



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What percentage of patients will experience vision loss in giant cell arteritis?



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A 60-year-old female presents with a headache and blurriness of vision in the right eye for the last 2 days. On exam, the patient having the tenderness in the right temporal area. What is the next best step in management?



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What is the average age onset of temporal arteritis?



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Select the correct statement about temporal arteritis (TA).



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Which of the following is not true about giant cell arteritis?



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An older male experiences temporary loss of vision in his left eye, headaches, pain when chewing food on the left side, and stiffness and pain in his hips. He has a history of coronary artery disease, hyperlipidemia, and chronic obstructive pulmonary disease. Funduscopic exam shows anterior ischemic optic neuropathy and visual acuity in the left eye is decreased. The temporal artery is painful. There are no carotid bruits, and the cardiac exam is normal. Erythrocyte sedimentation rate (ESR) is 95 mm/h. The hemoglobin is 7.9 g/dL. Plain head CT shows mild atrophy but no acute ischemic event. Select the best management for this patient.



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Which of the following is not associated with giant cell arteritis?



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An elderly lady complains of pain in her face when eating and in her scalp when combing her hair. She reports an intermittent bitemporal headache and neck pain. Which noninvasive test would be most accurate for her diagnosis?



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A 70 year old male has a 3 week history of visual changes, headaches, shoulder and hip pain and stiffness. Vasculitis is suspected and arterial biopsy is done. What is the most likely finding on histopathologic examination?



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What visual defect is associated with temporal arteritis?



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What is the gold standard to diagnose temporal arteritis?



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A 35-year-old woman has a past medical history of right-sided headache and tenderness in the right temporal region that started two months ago. She denies any visual changes, nausea, vomiting or loss of consciousness. Her ESR is 82. Which of the following conditions is commonly associated with the most likely diagnosis?



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To effectively diagnose temporal arteritis, the American College of Rheumatology recommends obtaining a biopsy of the affected artery of what length?



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A 72-year-old man develops severe headaches, fever, and decreased visual acuity. What specific physical finding should be sought?



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A 76-year-old male patient is seen for new-onset right-sided headaches for several weeks. He describes their severity as seven out of 10 and the quality as throbbing and continuous. He reports these headaches were preceded by malaise and neck and shoulder pain. He also notes pain of his right face during meals. His past medical history is significant for one transient ischemic attack this year. On physical exam, there is a small non-healing erosion over the left temple and tenderness to palpation of the right temple where a strongly bounding pulse is noted. His vision is 20/20. Initial lab workup reveals an elevated erythrocyte sedimentation rate. What is the best next step in management?



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Temporal (Giant Cell) Arteritis - References

References

Shahi F,Samson A, Positron emission tomography and reframing vasculitis as a spectrum of disease when investigating a patient with a fever of unknown origin. BMJ case reports. 2018 Sep 19     [PubMed]
Zhang Y,Wang D,Chu X,Zhang W,Zeng X, Differences in clinical manifestations and prognosis of Chinese giant cell arteritis patients with or without polymyalgia rheumatica. Irish journal of medical science. 2018 Sep 24     [PubMed]
Keser G,Aksu K, Diagnosis and differential diagnosis of large-vessel vasculitides. Rheumatology international. 2018 Sep 17     [PubMed]
Yavne Y,Tiosano S,Ben-Ami D,Watad A,Guy A,Comaneshter D,Cohen AD,Amital H, Giant cell arteritis and inflammatory bowel disease - Is there a connection? Results from a population-based study. Autoimmunity reviews. 2018 Sep 11     [PubMed]
Merrill KS,Lee MS,McClelland CM, Red Flags in the Assessment of Adult Ophthalmoplegia. Journal of binocular vision and ocular motility. 2018 Jan-Mar     [PubMed]
Misra DP,Naidu GSRSNK,Agarwal V,Sharma A, Vasculitis research: Current trends and future perspectives. International journal of rheumatic diseases. 2018 Aug 30     [PubMed]
Pucelj NP,Hočevar A,Ješe R,Rotar Ž,Hawlina M,Fakin A,Pižem J,Tomšič M, The incidence of giant cell arteritis in Slovenia. Clinical rheumatology. 2018 Jul 30     [PubMed]
Kobayashi D,Suyama Y,Osugi Y,Arioka H,Takahashi O,Kuriyama N, Incidence of cardiovascular events in polymyalgia rheumatica and giant cell arteritis amongst an Asian population: Propensity score matched cohort study. International journal of rheumatic diseases. 2018 Jun     [PubMed]
Sammel AM,Fraser CL, Update on giant cell arteritis. Current opinion in ophthalmology. 2018 Nov     [PubMed]
Guevara M,Kollipara CS, Recent Advances in Giant Cell Arteritis. Current rheumatology reports. 2018 Apr 2     [PubMed]
Ratschiller T,Müller H,Pirklbauer M,Silye R,Sulzbacher G,Zierer A, Giant cell arteritis as unusual cause of critical arm ischemia. Journal of vascular surgery cases and innovative techniques. 2018 Sep     [PubMed]
Starling AJ, Diagnosis and Management of Headache in Older Adults. Mayo Clinic proceedings. 2018 Feb     [PubMed]
Kermani TA,Sreih AG,Cuthbertson D,Carette S,Hoffman GS,Khalidi NA,Koening CL,Langford CA,McAlear CA,Monach PA,Moreland L,Pagnoux C,Seo P,Warrington KJ,Ytterberg SR,Merkel PA, Evaluation of damage in giant cell arteritis. Rheumatology (Oxford, England). 2018 Feb 1     [PubMed]
Muratore F,Pipitone N,Salvarani C, Standard and biological treatment in large vessel vasculitis: guidelines and current approaches. Expert review of clinical immunology. 2017 Apr     [PubMed]
Bienvenu B,Ly KH,Lambert M,Agard C,André M,Benhamou Y,Bonnotte B,de Boysson H,Espitia O,Fau G,Fauchais AL,Galateau-Sallé F,Haroche J,Héron E,Lapébie FX,Liozon E,Luong Nguyen LB,Magnant J,Manrique A,Matt M,de Menthon M,Mouthon L,Puéchal X,Pugnet G,Quemeneur T,Régent A,Saadoun D,Samson M,Sène D,Smets P,Yelnik C,Sailler L,Mahr A, Management of giant cell arteritis: Recommendations of the French Study Group for Large Vessel Vasculitis (GEFA). La Revue de medecine interne. 2016 Mar     [PubMed]
Tsetsou S,Michel P,Ribi C,Hirt L,Kawasaki A,Hugli O,De Leval L,Bart PA,Waeber G,Meuli R,Raffoul W,So A,Du Pasquier R, [Giant cell arteritis: guidelines of the University Hospital of Lausanne]. Revue medicale suisse. 2015 Feb 11     [PubMed]
Alberts M, Temporal arteritis: improving patient evaluation with a new protocol. The Permanente journal. 2013 Winter     [PubMed]
Koster MJ,Matteson EL,Warrington KJ, Large-vessel giant cell arteritis: diagnosis, monitoring and management. Rheumatology (Oxford, England). 2018 Feb 1     [PubMed]

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