Giant cell arteritis
Background
- Systemic vasculitis most commonly involving medium-sized arteries in the carotid circulation affection 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 w/ severe vision loss if left untreated
- Associated with polymyalgia rheumatica (30-40%)[3]
Clinical Features
- Fever
- Headache in 85%
- Gradually worsens over days
- Worse at night
- Usually unilateral near temple
- Jaw claudication
- Weight Loss
- Myalgias
- Visual loss in one eye in 50%
- Posterior ciliary artery
- May present as amaurosis fugax
- Second eye may be affected within weeks after first
American College of Rheumatology Criteria[4]
- 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
Differential Diagnosis
Headache
Common
Killers
- Meningitis/encephalitis
- Myocardial ischemia
- 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)
- Painful
- Arteritic anterior ischemic optic neuropathy
- Optic neuritis
- Temporal arteritis†
- Painless
- Amaurosis fugax
- Central retinal artery occlusion (CRAO)†
- Central retinal vein occlusion (CRVO)†
- High altitude retinopathy
- Open-angle glaucoma
- Posterior reversible encephalopathy syndrome (PRES)
- Retinal detachment†
- Stroke†
- Vitreous hemorrhage
- Traumatic optic neuropathy (although may have pain from the trauma)
†Emergent Diagnosis
Vasculitis Syndrome Types
- Large vessel
- Takayasu arteritis
- Giant cell arteritis (temporal arteritis)
- Medium-vessel
- Kawasaki disease
- Polyarteritis nodosa
- Thromboangiitis obliterans (Buerger's disease)
- Primary angiitis of the central nervous system
- Small-vessel
- Henoch-Schönlein purpura
- ANCA-associated vasculitides
- Granulomatosis with polyangiitis (Wegner's)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- Cutaneous leukocytoclastic angiitis (“hypersensitivity vasculitis”)
- Essential cryoglobulinemia, cryoglobulinemic vasculitis due to hepatitis C
- Behçet's disease]
- Secondary vasculitides and other/miscellaneous
- Drug-induced vasculitis
- Serum sickness
- Vasculitis associated with other rheumatic diseases (e.g. SLE)
Diagnosis
- Temporal artery tenderness
- Afferent pupillary defect
- Pale and edematous optic disc
- ESR ~70-110
- 84% sensitivity, 30% specificity[5]
- >15% of patients can have a normal ESR
- CRP elevated
- 4% of patients have normal CRP and ESR with biopsy confirmed dx[6]
Management
- Methylprednisolone 1000mg IV QD x3d
- Needs temporal artery biopsy
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: Tintinalli JE, Stapczynski JS, Cline DM, Ma OJ, Cydulka RK, Meckler GD, eds. Tintinalli'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.
- ↑ Hunder GG. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990; 33(8):1122-8
- ↑ 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.
- ↑ Jhun P, et al. Giant Cell Arteritis: Read the Fine Print! Ann Em Med. 2015; 65(5):615–617.