Giant cell arteritis
The term "temporal arteritis" is no longer used. It refers to giant cell arteritis that affects the temporal artery.
Background
- Systemic vasculitis most commonly involving medium-sized arteries in the carotid circulation, affecting 1% of the population[1]
- Giant cell arteritis, with possible involvement of large vessels like aorta leading to[2]:
- Temporal arteritis
- Aortic regurgitation
- Aortic arch syndrome
- Aortic dissection
- Elevated risk in Women and 50-70 yrs of age
- "Rule of 50s" can help remember useful points - "temporal arteritis affects patients at least 50 years of age, with a serum ESR > 50 mm/hr and is treated with 50mg of prednisone daily"
- Can cause painless, ischemic optic neuropathy with severe vision loss if left untreated
- Associated with polymyalgia rheumatica [3]
- 50% of patients with Giant Cell Arteritis have concomitant Polymylalgia Rheumatica. 15% of patients with Polymyalgia Rheumatica develop Giant Cell Arteritis
Clinical Features
- Fever
- Headache in 85%
- Gradually worsens over days
- Worse at night
- Usually unilateral near temple
- Jaw claudication
- Weight Loss
- Myalgia (polymyalgia rheumatica)
- Visual loss in one eye in 50%
- Posterior ciliary artery
- May present as amaurosis fugax or transient diplopia
- Second eye may be affected within weeks after first
- Tender, pulseless temporal artery
- Afferent pupillary defect
- Pale and edematous optic disc
Likelihood Ratio of Findings
Jaw claudication and a beaded temporal artery increase the likelihood of temporal arteritis the greatest[4]
| Finding | (+) Likelihood Ratio of Temporal Ateritis | Negative Likelihood Ratio |
| Jaw claudication | 4.2 (2.8-6.2) | 0.72 (0.65 - 0.81) |
| Diplopia | 3.4 (1.3-8.6) | 0.95 (0.91 - 0.99) |
| Temporal artery beading | 4.6 (1.1 - 18.4) | 0.93 (0.88-0.99) |
| Enlarged temporal artery | 4.3 (2.1-8.9) | 0.67 (0.5-0.89) |
| Painful temporal artery | 2.6 (1.9-3.7) | 0.82 (0.74-0.92) |
| Absent temporal artery pulse | 2.7 (0.55 - 13.4) | 0.71 (0.38 - 1.3) |
| Abnormal ESR | 1.1 (1.0-1.2) | 0.2 (0.08 - 0.51) |
Differential Diagnosis
Killers
- Meningitis/encephalitis
- Retropharyngeal abscess
- Intracranial Hemorrhage (ICH)
- SAH / sentinel bleed
- Acute obstructive hydrocephalus
- Space occupying lesions
- CVA
- Carbon monoxide poisoning
- Basilar artery dissection
- Preeclampsia
- Cerebral venous thrombosis
- Hypertensive emergency
- Depression
Maimers
- Giant cell arteritis of temporal artery (temporal arteritis)
- Idiopathic intracranial hypertension (Pseudotumor Cerebri)
- Acute Glaucoma
- Acute sinusitis
- Cavernous sinus thrombosis or cerebral sinus thrombosis
- Carotid artery dissection
Others
- Trigeminal neuralgia
- TMJ pain
- Post-lumbar puncture headache
- Dehydration
- Analgesia abuse
- Various ocular and dental problems
- Herpes zoster ophthalmicus
- Herpes zoster oticus
- Cryptococcosis
- Febrile headache (e.g. pyelonephritis, nonspecific viral infection)
- Ophthalmoplegic migraine
- Superior Vena Cava Syndrome
Aseptic Meningitis
- Viral
- Tuberculosis
- Lyme disease
- Syphilis
- Leptospirosis
- Fungal (AIDS, transplant, chemotherapy, chronic steroid use)
- Noninfectious
Acute Vision Loss (Noninflamed)
- Arteritic anterior ischemic optic neuropathy
- Amaurosis fugax
- Central retinal artery occlusion (CRAO)†
- Central retinal vein occlusion (CRVO)†
- High altitude retinopathy
- Open-angle glaucoma
- Optic neuritis
- Posterior Reversible Encephalopathy Syndrome (PRES)
- Retinal detachment†
- Temporal arteritis†
- Traumatic optic neuropathy
- Vitreous hemorrhage
- Stroke†
†Emergent Diagnosis
- Large vessel
- Medium vessel
- Polyarteritis nodosa
- Buerger disease
- Kawasaki disease
- Granulomatosis with polyangiitis
- [[Churg-Strauss syndrome
- Microscopic polyangiitis
- Small vessel
- Essential mixed cryoglobulinemia
- Henoch-Schonlein purpura
- Behcet disease
Evaluation
- ESR ~70-110
- 84% sensitivity, 30% specificity[5]
- >15% of patients can have a normal ESR
- CRP elevated[6]
- May be more sensitive than ESR
- Doesn't have normal age related increase like ESR does
- 4% of patients have normal CRP and ESR with biopsy confirmed diagnosis[7]
American College of Rheumatology Criteria[8]
- 3 or more criteria 93% sensitive and 91% specific
- Age ≥ 50 years old
- New onset of headache
- Temporal artery tenderness or DECREASED temporal pulse (not related to carotid disease)
- ESR ≥ 50 mm/hr
- Artery biopsy with necrotizing arteritis or a granulomatous process with multinucleated giant cells
Management
- Methylprednisolone 1000mg IV QD x3d
- Needs temporal artery biopsy, although treatment with high dose corticosteroids should be initiated prior to biopsy to avoid permanent vision loss as a result of ophthalmic artery involvement.
Disposition
- Admission
See Also
References
- Gonzalez-Gay, MA et al. Epidemiology of the vasculitides. Rheum Dis Clin North Am. 2001;27:729-749
- Morabito GC, Tartaglino B. Chapter 279. Emergencies in Systemic Rheumatic Diseases. In: JE, Stapczynski JS, Cline DM, Ma OJ, Cydulka RK, Meckler GD, eds. 's Emergency Medicine: A Comprehensive Study Guide. 7th ed. New York: McGraw-Hil
- Lehrmann JF, Sercombe CT: Systemic Lupus Erythmatosus and the Vasculitides, in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 7. St. Louis, Mosby, Inc., 2010, (Ch) 116: p 1497-1510.
- Smetana GW, et al. Does this patient have temporal arteritis? JAMA. 2002;287:92-101
- Kermani TA, et al. Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of giant cell arteritis. Semin Arthritis Rheum. 2012; 41:866–871.
- Kermani et al. Utility of Erythrocyte Sedimentation Rate and C-Reactive Protein for the Diagnosis of Giant Cell Arteritis. Semin Arthritis Rheum. 2012 Jun; 41(6): 866–871.
- Jhun P, et al. Giant Cell Arteritis: Read the Fine Print! Ann Em Med. 2015; 65(5):615–617.
- Hunder GG. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990; 33(8):1122-8
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