Arteritis, Temporal – Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

Also known as giant cell arteritis. A large vessel vasculitis affecting the elderly. Primarily involves the cranial branches of the carotid arteries and can cause visual loss.

Epidemiology

  • Disease of the elderly, almost never seen in persons <50 years. Incidence increases with age
  • Female > Male (2:1)

Incidence

More common in Northern Europe, esp. Scandinavia (20 per 100,000 per year) vs Southern Europe (10 per 100,000 per year). In the US, incidence in a largely white Minnesota cohort was 19 per 100,000 per year. It is likely less for the US as a whole. Temporal arteritis is very rare in populations of non-European ancestry.

Risk Factors

  • Increasing age
  • Polymyalgia rheumatica
  • Atherosclerosis and smoking in women but not men

Genetics

  • Some family clusters have been documented.
  • HLA-DR4 and HLA-DRB1 are associated with temporal arteritis.

Etiology

Unclear, involves cell-mediated immune response and Il-6 production

Commonly Associated Conditions

Polymyalgia rheumatica

Polymyalgia rheumatica, Giant-cell arteritis, Superficial temporal artery, Cardiovascular Disorders, Nutrition and Metabolism Disorders,

Diagnosis

History

Usually gradual in onset but may be abrupt

  • New or changed headache (usually unilateral temporal but may be generalized)
  • Scalp and facial tenderness
  • Jaw and tongue claudication with chewing (most specific symptom for temporal arteritis)
  • Visual changes
    • Amaurosis fugax
    • Diplopia
    • Scotoma
    • Blindness
  • Nonproductive cough
  • Arm claudication
  • Constitutional symptoms
    • Fever
    • Weight loss
    • Fatigue
    • Polymyalgia rheumatica (stiffness and aching in shoulder and hip girdles). Present in 50%.

Physical Exam

  • Prominent, tender, or pulseless temporal artery
  • Scalp tenderness
  • Cranial nerve palsies
  • Visual field defect
  • Abnormal retinal exam (cotton wool spots, pale edematous retina)
  • Proximal muscle tenderness but no weakness

Diagnostic Tests & Interpretation

Lab

  • ESR/CRP; usually very elevated (ESR >50). <10% have a normal ESR. If both ESR and CRP are normal, consider alternative diagnoses.
  • Normocytic, normochromic anemia; usually mild
  • Elevated acute phase reactants (platelets, LFTs, albumin)

Imaging

  • Ultrasonography of the temporal artery can be up to 88% sensitive and 97% specific for diagnosing temporal arteritis but is quite operator dependent. It does not replace biopsy for definitive diagnosis. (1)
  • MRI/MRA in patients with arm claudication or other evidence of aortic branch involvement. It also should be considered, esp. in patients with an aortic insufficiency murmur, to evaluate for presence of aortic aneurysm.

Diagnostic Procedures/Surgery

Temporal artery biopsy: The gold standard for the diagnosis of temporal arteritis

  • Treatment prior to biopsy is unlikely to affect the biopsy results, but biopsy should be done within 2 weeks of commencing treatment.
  • A segment >2 cm long is needed as there are often skip lesions.
  • Serial sections should be done.
  • Routine biopsy of both temporal arteries is not necessary; however, if the first biopsy is negative and clinical suspicion remains high, a contralateral biopsy could be considered.

Pathological Findings

Inflammatory infiltrate (either mononuclear cells or granulomas with giant multinucleated cells) seen in the intima and media of large vessels with resultant disruption of the internal elastic lamina. Lesions may be isolated (e.g., skip lesions).

Differential Diagnosis

  • CNS vasculitis
  • Embolic disease
  • Temporomandibular joint syndrome
  • Space-occupying lesion or other cause for new headache in the elderly
  • Retinal disease
  • CVA

Treatment

Medication

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First Line

Prednisone:

  • Due to the risk of irreversible vision loss, treatment with high-dose steroids should be started on strong clinical suspicion of temporal arteritis, prior to the temporal biopsy being done. The initial dose of prednisone is 60 mg/day [B]. Steroids should not be in the form of alternate day therapy, as this is more likely to lead to a relapse of vasculitis.
  • Pulsed dose intravenous methylprednisolone (1 g/day for 3 days) may be of benefit to patients who present with recent onset of visual symptoms (2)[C]. This is followed by prednisone 60 mg/day as above.
  • At 6–8 weeks, prednisone can be tapered by 5 mg every 2 weeks to a dose of 25 mg/day. Then the taper is slowed to 2.5 mg every 2 weeks to a dose 10 mg/day. After that, the prednisone is very gradually weaned by 1 mg every 3–6 months. Dose reduction should be considered only in the absence of clinical symptoms, signs and laboratory abnormalities suggestive of active disease. Many patients may require a slower taper.
  • Consider low-dose aspirin for all patients with giant cell arteritis (3)[C]
  • Patients on corticosteroids should be on bone protection therapy unless they have contraindications

Second Line

Methotrexate (10–15 mg/week) has a modest effect in temporal arteritis and could be considered in patients who have not responded to glucocorticoids or who have significant side effects from steroids (4)[B].

Ongoing Care

Follow-Up Recommendations

  • Follow up in clinic at least monthly initially. When stable and steroids are being tapered, every 3 months.
  • Disease relapse should be suspected in patients with new headaches, visual changes, fever, myalgias. A rise in ESR/CRP is usually seen with relapse. An increase in steroids by 10 mg/day is usually significant to control a relapse.

Patient Monitoring

  • Check ESR/CRP with each visit to monitor disease activity.
  • Consider yearly CXR (to evaluate for aortic aneurysm) and biannual DEXA scans.

Diet

Calcium and vitamin D supplementation

Patient Education

  • Consequences of discontinuing steroids abruptly (adrenal suppression, disease relapse)
  • Risks of long-term steroid use
  • Importance of reporting new headaches and vision changes to provider immediately

Prognosis

  • Prior vision loss is unlikely to be recovered, but treatment resolves the other symptoms and prevents future vision loss and stroke.
  • Average disease duration 3–4 years but may be up to 10 years.

Complications

  • Sequelae of long-term steroid use (osteoporosis, diabetes, thinned skin, weight gain)
  • Blindness
  • Stroke
  • Aortic aneurysm/dissection

References

1. Karassa FB, Matsagas MI, Schmidt WA, Ioannidis JP, et al. Meta-analysis: test performance of ultrasonography for giant-cell arteritis. Ann Intern Med. 2005;142:359–69.

2. Hayreh SS, Zimmerman B, Kardon RH, et al. Visual improvement with corticosteroid therapy in giant cell arteritis. Report of a large study and review of literature. Acta Ophthalmol Scand. 2002;80:355–67.

3. Lee MS, Smith SD, Galor A et al. Antiplatelet and anticoagulant therapy in patients with giant cell arteritis. Arthritis Rheum. 2006;54:3306–9.

4. Mahr AD, Jover JA, Spiera RF, Hernández-García C, Fernández-Gutiérrez B, Lavalley MP, Merkel PA, et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum. 2007;56:2789–97.

Additional Reading

Hunder GG, Bloch DA, Michel BA et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33:1122–8.

Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R, European Vasculitis Study Group et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68:318–23.

See Also (Topic, Algorithm, Electronic Media Element)

Depression; Fibromyalgia; Headache, Cluster; Headache, Tension; Polymyalgia Rheumatica; Polymyositis/Dermatomyositis

Codes

ICD9

446.5 Giant cell arteritis

Snomed

  • 400130008 temporal arteritis (disorder)
  • 414341000 giant cell arteritis (disorder)

Clinical Pearls

  • Due to the risk of irreversible vision loss, treatment with high-dose steroids (prednisone 60 mg/day) should be started immediately in patients suspected of temporal artery.
  • Temporal artery biopsy is the gold standard for diagnosis. Temporal artery biopsy is not likely to be affected by a few weeks of treatment.
  • Treatment consists of a very slow steroid taper. Bone protection therapy and low dose aspirin should be considered.

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