II. Epidemiology

  1. Closely associated with Polymyalgia Rheumatica
    1. Polymyalgia is present in 27-53% of those with Temporal Arteritis
  2. Prevalence: 500 per 100,000 over age 50 years
  3. Rarely occurs under age 50 years
  4. Average age of presentation: 72 years (peak age 70-80 years old)

III. Pathophysiology

  1. Inflammation of medium and large arteries originating from aortic arch
    1. Most commonly affects external Carotid Artery branches (esp. temporal artery, occipital artery)
    2. Infiltration of arterial wall with inflammatory cells
    3. Localized to elastic laminae
      1. May extend to panarteritis
  2. Intima thickens results in lumen narrowing, Occlusion
    1. Causes Arteritic Ischemic Optic Neuropathy

IV. Symptoms

  1. See Polymyalgia Rheumatica for rheumatologic symptoms
  2. Onset of symptoms
    1. Insidious over months or
    2. Sudden Onset
  3. New Onset Headache (LR+ 3.6, LR- 0.43)
    1. Test Sensitivity >50 to 70%
    2. Test Specificity 82%
    3. Over temporal or occipital arteries (but may occur elsewhere)
    4. Boring ache of moderate intensity with minimal relief from Analgesics
    5. Scalp tenderness, and touch may provoke Headache
  4. Jaw Claudication (LR+ 20, LR- 0.62)
    1. Test Sensitivity: 39%
    2. Test Specificity: 98%
    3. Facial Muscle pain or weakness with chewing and relieved with rest
    4. May be atypical with tooth, sinus, Tongue or Ear Pain
  5. Visual symptoms (LR+ 2.5, LR- 0.81)
    1. Test Sensitivity: 28%
    2. Test Specificity: 89%
    3. May precede permanent Vision Loss by hours or days
    4. Vision may be partially obscured
    5. Visual Field cuts
    6. Diplopia
    7. Acute Vision Loss or Amaurosis Fugax
  6. Systemic symptoms (at least one systemic symptom occurs in 75% of patients)
    1. Fever of Unknown Origin
    2. Malaise
    3. Fatigue
    4. Anorexia
    5. Weight loss
  7. Respiratory and other symptoms (10% of cases)
    1. Sore Throat
    2. Dry Cough
    3. Tongue Pain (or tongue Claudication)
    4. Pharyngitis
    5. Dysphagia
    6. Choking Sensation
    7. Lower extremity Claudication

V. Signs

  1. See Polymyalgia Rheumatica for rheumatologic findings
  2. Tenderness or Hypersensitivity over temporal or occipital arteries or scalp (LR+ 21, LR- 0.6)
    1. Test Sensitivity: 41%
    2. Test Specificity: 98%
  3. Temporal artery abnormalities (LR+ 19, LR- 0.44)
    1. Test Sensitivity: 57%
    2. Test Specificity: 97%
    3. Palpate superior to ear tragus and compare to contralateral side
      1. Pulse reduction
      2. Nodular, beaded or thickened
  4. Eye Exam to exclude other causes of Vision change
    1. Visual Acuity
    2. Extraocular Movements
    3. Pupillary Light Reflex
      1. Observe for Afferent Pupillary Defect
    4. Funduscopic Exam
  5. Cardiovascular Exam (large vessels including the aorta may be affected)
    1. Peripheral pulses (evaluate for symmetry, esp. between radial pulses)
  6. Complete Neurologic Exam
    1. Evaluate for neurologic deficits suggestive of Cerebrovascular Accident

VI. Complications

  1. Sudden Vision Loss (Anterior Ischemic Optic Neuropathy)
    1. Occurs in 8-15% of Temporal Arteritis patients
    2. See Amaurosis Fugax
    3. Secondary to narrowing of arterial lumens
      1. Ophthalmic terminal branches
      2. Posterior ciliary arteries
    4. Visual deficit is permanent
      1. May be preceded by visual changes or Headaches by hours or days
      2. Contralateral eye is typically affected within 1-2 weeks (requires prompt management)

VII. Associated Conditions

  1. Polymyalgia Rheumatica (50%)
  2. Jaw Claudication (see above)
  3. Aorta involvement (15% of cases)
    1. Aortic Arch Syndrome
    2. Vertebrobasilar Artery thrombosis
    3. Upper extremity Claudication
    4. Raynaud Phenomenon
    5. Thoracic Dissection
    6. Myocardial Infarction
    7. Mesenteric Infarction

IX. Diagnosis

  1. Precautions
    1. Strongly consider GCA in over age 60 years old with sudden onset Vision Loss or Diplopia (including transient)
    2. Delayed treatment may result in blindness
    3. Start Corticosteroids immediately when diagnosis suspected
    4. Do not wait for biopsy results prior to starting Corticosteroids
    5. Obtain biopsy within 2 weeks of starting Corticosteroids (first 2-3 days preferred)
  2. Temporal Artery Biopsy
    1. Biopsy shows chronic inflammation, necrotizing arteritis
      1. Mononuclear cell predominance OR
      2. Granulomatous process with multinucleated giant cells
    2. Obtain at least 1 cm sample from most symptomatic side
      1. Long biopsy specimens (>2 cm) are preferred
    3. Test Sensitivity: 80-92%
      1. False Negative Rate up to 20%
      2. Consider biopsy of contralateral side if negative biopsy despite high clinical suspicion
      3. Consider empiric treatment despite negative biopsy if high clinical suspicion
    4. Biopsy positive for 2 weeks after starting Prednisone
      1. Biopsy is best within 48 to 72 hours
  3. Findings most suggestive of Temporal Arteritis
    1. Beaded (nodular thrombus) temporal artery (+LR 4.6)
    2. Prominent temporal artery (+LR 4.3)
    3. Jaw Claudication (+LR 4.2)
    4. Diplopia (+LR 3.4)
    5. Temporal artery pulse absent (+LR 2.7)
    6. Temporal artery tender (+LR 2.6)
    7. Smetana (2002) JAMA 287:92-101 [PubMed]
  4. American College of Rheumatology criteria (3 of 5 criteria required)
    1. Age 50 years or older
    2. Localized Headache of new onset
    3. Tenderness or decreased pulse over the temporal artery
    4. ESR 50 mm/h or higher
    5. Temporal artery biopsy with necrotizing arteritis (see findings above)

X. Labs

  1. See Polymyalgia Rheumatica for complete lab evaluation of differential diagnosis
  2. Acute phase reactants
    1. Obtain both ESR and C-RP
      1. Test Sensitivity approaches 99% in Temporal Arteritis when both are obtained
    2. Erythrocyte Sedimentation Rate (Westergren) Increased
      1. See Polymyalgia Rheumatica for interpretation
      2. ESR typically >50 mm/h (averages 83 mm/h in Temporal Arteritis, often >100 mm/h)
      3. Age based cut-offs
        1. Men: Age/2
        2. Women: (Age + 10)/2
    3. C-Reactive Protein increased
      1. Higher Test Sensitivity than ESR for initial diagnosis
      2. No well defined cut-off criteria (unlike ESR with cutoff of 50 mm/h)
    4. Interleukin-6
      1. May be elevated in new or recurrent GCA and returning to normal with remission
      2. Consider when ESR and C-RP are normal despite high clinical suspicion
  3. Nonspecific Lab findings
    1. Moderate normocytic, normochromic Anemia
    2. Thrombocytosis
    3. Decreased Serum Albumin
    4. Increased Alkaline Phosphatase (50% of cases)

XI. Imaging

  1. Indications
    1. Strong clinical suspicion for GCA, but negative biopsy or biopsy unavailable
  2. Doppler Ultrasound of temporal artery
    1. Positive (abnormal) if noncompressible, hypoechoic temporal artery with wall thickening
    2. Sufficient for Temporal Arteritis diagnosis if positive
    3. Luqmani (2016) Health Technology Assessment
      1. https://www.ncbi.nlm.nih.gov/books/NBK401227/
    4. Karassa (2005) Ann Intern Med 142(5): 359-69 [PubMed]
    5. Aranda-Valera (2017) Clin Exp Rheumatol 35 Suppl 103(1):123-127 +PMID:28244857 [PubMed]
  3. Other advanced imaging studies
    1. Fluorodeoxyglucose PET Scan
    2. MRI
    3. CT Aortic Survey (or MRI equivalent)
      1. Consider aortic imaging for symptoms or signs of large vessel involvement
      2. Consider in all new GCA cases (esp. if comorbid Hypertension)

XII. Management

  1. Urgent referral to rheumatology in all cases
  2. Do not delay starting if high level of suspicion
    1. Biopsy within 2 weeks of starting Corticosteroids (best within first 48-72 hours)
    2. Alternatively, temporal artery Ultrasound may be performed
  3. Corticosteroids
    1. Starting Dose
      1. No visual symptoms or Jaw Claudication
        1. Prednisone 40-60 mg (at least 0.75 mg/kg) orally daily
        2. Consider 40 mg instead of 60 mg orally daily for those with Diabetes Mellitus
      2. Visual symptoms (e.g. Amaurosis Fugax), Jaw Claudication or other critical cranial ischemia
        1. Solumedrol 250 mg every 6 hours for 3 days
        2. Following solumedrol course, switch to oral Prednisone 60 mg orally daily (as above)
      3. Established Vision Loss
        1. Prednisone 60 mg (at least 0.75 mg/kg) orally daily
        2. Protects the contralateral, unaffected eye
    2. Taper protocol (typically after 4-8 weeks on initial dose)
      1. Start tapering after 4 weeks on Prednisone AND symptom resolution and ESR/CRP normalization
      2. Taper dose by 10 mg every 2 weeks until 20 mg dose is reached, then
      3. Taper dose by 2.5 mg every 2-4 weeks until 10 mg dose is reached, then
      4. Taper dose by 1 mg every 1-2 months
      5. Anticipate low dose (10 mg) reached by 6 months
    3. Efficacy
      1. Symptoms and Signs remit in approximately 1 month
      2. Targets: Vision change, fever, Headache, myalgias
      3. Do not use ESR normalization as a target
    4. Course
      1. Continue Prednisone 1-2 years if ocular complications
      2. Prednisone may require continuation up to 5 years in some cases
  4. Adjuvant medications
    1. Tocilizumab (Actemra)
      1. Monoclonal Antibody associated with significant sparing of Corticosteroids
      2. Dosed weekly or to every other week
      3. Unizony (2012) Arthritis Care Res 64(11):1720-9 +PMID:22674883 [PubMed]
    2. Aspirin 81 mg orally daily
    3. Concurrent Methotrexate is not typically recommended (unlike Polymyalgia Rheumatica)
      1. However its use, along with biologic agent are left to local expert opinion (e.g. rheumatology)
  5. Prevention of Bone Mineral Density Loss due to Corticosteroids (e.g. Prednisone)
    1. See Corticosteroid Associated Osteoporosis
    2. Vitamin D Supplementation
    3. Calcium Supplementation
    4. Consider DEXA Scan
    5. Consider Bisphosphonates
  6. Other Prevention of complications from Corticosteroids (e.g. Prednisone)
    1. See Polymyalgia Rheumatica
    2. Consider Gastrointestinal Prophylaxis (e.g. Omeprazole or other Proton Pump Inhibitor)

XIII. Management: Follow-up

  1. Rheumatology consult
    1. One week after starting steroids, then
    2. Three weeks after starting steroids, and then
    3. Six weeks
  2. Rheumatology or primary care
    1. Visit at 3 months, 6 months, 9 months and 12 months
  3. Labs (each visit)
    1. Complete Blood Count
    2. Erythrocyte Sedimentation Rate (ESR)
    3. C-Reactive Protein (C-RP)
    4. Basic chemistry panel (Electrolytes, Renal Function tests, and Serum Glucose)
  4. Imaging
    1. Chest XRay to assess aortic root every 2 years
  5. Evaluation
    1. Relapse symptoms
      1. Fever without other cause
      2. New or Recurrent Headache, sclp tenderness, Jaw Claudication or Vision change
      3. Extremity Claudication, CVA symptoms
      4. Proximal Muscle pain (Shoulder or pelvic girdle) or Morning stiffness
      5. Fatigue
    2. Adverse effects to treatment (Corticosteroid adverse effects)
      1. Gastritis or peptic ulcer
      2. Bone density
      3. Hyperglycemia

XIV. Course

  1. Self limited course over months to years with Corticosteroids
  2. Increased mortality related to other regions of Vasculitis
    1. Brekke (2019) Arthritis Res Ther 21(1): 154 [PubMed]
  3. Risk of permanent blindness if untreated
    1. Contralateral eye is typically affected within 1-2 weeks (requires prompt management)

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Related Studies

Ontology: Giant Cell Arteritis (C0039483)

Definition (MEDLINEPLUS)

Giant cell arteritis is a disorder that causes inflammation of arteries of the scalp, neck, and arms. The inflammation narrows the arteries, which keeps blood from flowing well. Giant cell arteritis often occurs with another disorder called polymyalgia rheumatica. Both disorders are more common in women than in men. They almost always affect people over the age of 50.

Early symptoms of giant cell arteritis resemble the flu: fatigue, loss of appetite, and fever. Other symptoms include headaches, pain and tenderness over the temples, double vision or visual loss, dizziness, and problems with coordination and balance. You may also have pain in your jaw and tongue.

Your doctor will make the diagnosis based on your medical history, symptoms, and physical examination. There is no single test to diagnose giant cell arteritis, but you may have tests that measure inflammation.

Treatment is usually with corticosteroids. Early treatment is important; otherwise there is a risk of permanent vision loss or stroke. However, when properly treated, giant cell arteritis rarely comes back.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Definition (MSH) A systemic autoimmune disorder that typically affects medium and large ARTERIES, usually leading to occlusive granulomatous vasculitis with transmural infiltrate containing multinucleated GIANT CELLS. The TEMPORAL ARTERY is commonly involved. This disorder appears primarily in people over the age of 50. Symptoms include FEVER; FATIGUE; HEADACHE; visual impairment; pain in the jaw and tongue; and aggravation of pain by cold temperatures. (From Adams et al., Principles of Neurology, 6th ed)
Concepts Disease or Syndrome (T047)
MSH D013700
ICD9 446.5
SnomedCT 155442004, 195354005, 195357003, 195355006, 195356007, 87511001, 400130008, 414341000
English Arteritides, Giant Cell, Giant Cell Arteritides, Arteritis, Giant Cell, GCA - Giant cell arteritis, Giant cell arteritis NOS, GIANT CELL ARTERITIS, GCA, POLYMYALGIA RHEUMATICA, HORTON DIS, HORTONS DIS, Arteritis, Giant Cell, Horton's, Giant Cell Arteritis, Horton's arteritis, Arteritis, Giant Cell, Horton, Giant Cell Arteritis, Horton, Horton Giant Cell Arteritis, Horton's Giant Cell Arteritis, Horton Disease, Horton's Disease, Hortons Disease, Giant Cell Arteritis [Disease/Finding], horton's disease, gca, horton disease, horton's arteritis, arteritis cell giant, giant cell arteritis, giant cell arteritis (diagnosis), Giant cell arteritis NOS (disorder), Horton's disease, giant cell; arteritis, arteritis; giant cell, Giant cell arteritis, Giant cell arteritis (disorder), Arteritis;giant cell
Dutch giant cell arteritis, ziekte van Horton, Horton arteritis, arteriitis; reuscel, reuscel; arteriitis
French Artérite temporale, Artérite à cellules géantes, Maladie d'Horton, Artérite giganto-cellulaire de Horton, Artérite gigantocellulaire de Horton, Maladie de Horton
German Horton-Arteritiis, Horton-Krankheit, Riesenzellen-Arteriitis, Senile Riesenzellarteriitis, Horton-Magath-Brown-Syndrom, Riesenzellarteriitis, Temporalarteriitis, Horton-Neuralgie, Horton-Riesenzellarteriitis
Italian Arterite di Horton, Arterite a cellule giganti, Malattia di Horton, Arterite a cellule giganti di Horton
Portuguese Arterite de células gigantes, Arterite de Horton, Arterite de Células Gigantes, Arterite de Células Gigantes de Horton, Arterite de Horton de Células Gigantes, Doença de Horton
Spanish Arteritis de células gigantes, Arteritis de Células Gigantes, enfermedad de Horton, arteritis temporal de células gigantes, arteritis de células gigantes, SAI (trastorno), arteritis de células gigantes, SAI, Arteritis de Células Gigantes de Horton, arteritis de células gigantes (trastorno), arteritis de células gigantes, Arteritis de Horton, Enfermedad de Horton
Japanese ホートン病, ホートンドウミャクエン, ホートンビョウ, キョサイボウセイドウミャクエン, 動脈炎-側頭, ホートン動脈炎, 動脈炎-巨細胞性, 巨細胞性動脈炎, 側頭動脈炎
Finnish Ohimovaltimotulehdus
Swedish Jättecellsarterit
Czech Hortonova arteriitida, Obrovskobuněčná arteriitida, obrovskobuněčná arteritida, Hortonova obrovskobuněčná arteriitida, Hortonova choroba, Hortonova nemoc, gigantocelulární arteritida
Polish Olbrzymiokomórkowe zapalenie tętnic, Zapalenie tętnic olbrzymiokomórkowe
Hungarian Óriássejtes arteritis, Horton-arteritis, Horton-betegség
Norwegian Hortons arteritt, Temporalarteritt, Kjempecellearteritt, Arteritis temporalis

Ontology: Optic Neuropathy, Ischemic (C0155305)

Definition (MSHCZE) Onemocnění s ischemií přední části papily zrakového nervu způsobené okluzí ciliárních tepen. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) Ischemic injury to the OPTIC NERVE which usually affects the OPTIC DISK (optic neuropathy, anterior ischemic) and less frequently the retrobulbar portion of the nerve (optic neuropathy, posterior ischemic). The injury results from occlusion of arterial blood supply which may result from TEMPORAL ARTERITIS; ATHEROSCLEROSIS; COLLAGEN DISEASES; EMBOLISM; DIABETES MELLITUS; and other conditions. The disease primarily occurs in the sixth decade or later and presents with the sudden onset of painless and usually severe monocular visual loss. Anterior ischemic optic neuropathy also features optic disk edema with microhemorrhages. The optic disk appears normal in posterior ischemic optic neuropathy. (Glaser, Neuro-Ophthalmology, 2nd ed, p135)
Concepts Disease or Syndrome (T047)
MSH D018917
ICD9 377.41
ICD10 H47.01
SnomedCT 14357004
English Ischemic Optic Neuropathies, Ischemic Optic Neuropathy, Neuropathies, Ischemic Optic, Neuropathy, Ischemic Optic, Optic Neuropathies, Ischemic, Optic Neuropathy, Ischemic, AION - Ac ischaem optic neurop, ION - Ischaemic optic neurop, ION - Ischemic optic neurop, ischemic optic neuropathy (diagnosis), ischemic optic neuropathy, Optic ischaemic neuropathy, Optic ischemic neuropathy, Ischemia, Optic Nerve, Nerve Ischemia, Optic, Optic Nerve Ischemia, Ischemias, Optic Nerve, Nerve Ischemias, Optic, Optic Nerve Ischemias, Ischemic optic neuropthy, Optic Neuropathy, Ischemic [Disease/Finding], ischemic neuropathy optic, optic nerve ischemia, ischaemic optic neuropathy, Ischemic optic neuropathy, AION - Acute ischaemic optic neuropathy, AION - Acute ischemic optic neuropathy, ION - Ischaemic optic neuropathy, ION - Ischemic optic neuropathy, Ischaemic optic neuropathy, Ischemic optic neuropathy (disorder), ischemic; neuropathic, optic nerve
Dutch ischaemische opticus neuropathie, ischemische opticus neuropathie, ischemie; neuropathie, nervus opticus, opticus ischaemie neuropathie, Ischemische opticusneuropathie, Nervus-opticusneuropathie, Neuropathie, ischemische opticus-, Opticusneuropathie, ischemische
French Neuropathie ischémique optique, Neuropathie optique ischémique, Ischémie du nerf optique, NOI (Neuropathie Optique Ischémique), Névrite optique ischémique
Portuguese Neuropatia óptica isquémica, Nevrite óptica isquémica, Isquemia do Nervo Óptico, Neuropatia Óptica Isquêmica
Spanish Neuropatía isquémica óptica, neuropatía óptica isquémica (trastorno), neuropatía óptica isquémica, Neuropatía óptica isquémica, Isquemia del Nervio Óptico, Neuropatía Isquémica Óptica, Neuropatía Óptica Isquémica
Swedish Opticusneuropatier, ischemiska
Japanese キョケツセイシシンケイショウ, 虚血性視神経症, 視神経症-虚血性
Czech neuropatie optická ischemická, Ischemická optická neuropatie, Optická ischemická neuropatie, Oční ischemická neuropatie, ischemická optická neuropatie
Finnish Iskeeminen optikusneuropatia
Italian Ischemia del nervo ottico, Neuropatia ottica ischemica
Polish Neuropatia nerwu wzrokowego niedokrwienna
Hungarian Ischaemiás opticus neuropathia, Opticus ischaemiás neuropathia
Norwegian Synsnerveiskemi, Iskemisk opticusnevropati, Opticusnerveiskemi, Iskemisk optikusnevropati, Optikusnerveiskemi
German ischaemische Neuropathie des Nervus opticus, Ischämie des Optikusnervs, Ischämische Optikusneuropathie, Optikusneuropathie, ischämische

Ontology: Cranial Arteritis (C1956390)

Concepts Disease or Syndrome (T047)
MSH D013700
English Cranial Arteritides, Arteritides, Cranial, Arteritis, Cranial, Cranial Arteritis, cranial arteritis, arteritis cranial
Portuguese Arterite Cranial
Spanish Arteritis Craneal
French Artérite crânienne
German Arteriitis cranialis, Kranial-Arteriitis
Czech kraniální arteritida
Norwegian Kraniearteritt
Italian Arterite cranica

Ontology: Temporal Arteritis (C1956391)

Definition (NCI) An autoimmune, systemic, giant cell granulomatous arteritis predominantly involving the arteries that supply blood to the central nervous system, head and eyes. Superficial arteries of the scalp that are involved tend to be enlarged and tender. Signs and symptoms include headaches, myalgias, visual disturbances, and skin necrosis.
Definition (CSP) subacute, granulomatous arteritis involving the external carotid arteries, especially the temporal artery; occurs in elderly persons and may be manifested by constitutional symptoms, particularly severe headache, and sometimes sudden unilateral blindness; shares many of the symptoms of polymyalgia rheumatica.
Concepts Disease or Syndrome (T047)
MSH D013700
SnomedCT 87511001, 400130008
English Temporal Arteritis, TEMPORAL ARTERITIS, CRANIAL ARTERITIS, Temporal Arteritides, Arteritis, Temporal, Arteritides, Temporal, cranial arteritis (diagnosis), temporal arteritis, cranial arteritis, temporal arteritis (diagnosis), Arteritis;temporal, arteritis temporal, Temporal arteritis, Cranial arteritis, Temporal giant cell arteritis, TA - Temporal arteritis, Temporal arteritis (disorder), temporal; arteritis, arteritis; temporal
Dutch craniale arteritis, arteriitis; temporalis, temporalis; arteriitis, temporale arteritis
French Maladie de Horton, Artérite temporale, MALADIE DE HORTON
Italian Arterite cranica, Arterite temporale
Portuguese Arterite craniana, Arterite Temporal, ARTRITE CRANIANA, Artrite temporária
Spanish Arteritis craneal, Arteritis Temporal, ARTERITIS CRANEAL, arteritis temporal (trastorno), arteritis temporal, Arteritis de la temporal
Japanese 頭部動脈炎, 側頭動脈炎, トウブドウミャクエン, ソクトウドウミャクエン
German Temporale Arteriitis, Arteriitis, temporale, Arteriitis temporalis, ARTERIITIS CRANIALIS
Czech Temporální arteriitida, Kraniální arteriitida, temporální arteritida
Hungarian Arteritis temporalis, Cranialis arteritis
Norwegian Temporal arteritt