EM@3AM: Giant Cell/Temporal Arteritis

Author: Brit Long, MD (@long_brit, EM Attending Physician, San Antonio, TX) // Edited by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital)

Welcome to EM@3AM, an emDocs series designed to foster your working knowledge by providing an expedited review of clinical basics. We’ll keep it short, while you keep that EM brain sharp.

A 57-year-old female presents with right sided headache and mild fever (100.8 F). She has noted increased difficulty chewing, which worsens with foods such as steak.  You note an enlarged temporal artery with beading on exam. Her neurologic exam is normal, and she notes no vision changes.

What is the diagnosis, and what are your next steps?

Answer: Giant Cell or Temporal Arteritis

Background: This disease is a vasculitis affecting predominantly medium-sized vessels in the carotid circulation.^1-5

– It may affect large vessels like the aorta, resulting in aortic regurgitation, aortic dissection, and aortic arch syndrome.

– Greatest danger is ischemic optic neuropathy and vision loss if untreated.

– Women and ages 50-70 years are at greatest risk.^1-5

Presentation: Headache (85%), fever, jaw claudication, weight loss, tender and pulseless temporal artery, vision changes (50% affected).^1-5

– Vision loss is due to vasculitis of the posterior ciliary artery, which may present as amaurosis fugax. The patient may lose vision in the second eye within two weeks.

– This is associated with polymyalgia rheumatic in 50% of patients with confirmed arteritis. Up to 15% of patients with polymyalgia rheumatic will develop arteritis.

Findings (with likelihood ratio): Jaw claudication (4.2, 95% CI 2.8-6.2), diplopia (3.4, 95% CI 1.3-8.6), beading of the temporal artery (4.6, 95% CI 1.1 – 18), enlarged temporal artery (4.3, 95% CI 2.1-8.9), painful temporal artery (2.6, 95% CI 1.9-3.7), absent pulse in temporal artery (2.7, 95% CI 0.55-13.4), abnormal ESR (1.1, 95% CI 1.0-1.2).¹

American College of Rheumatology Criteria: 1) Age > 50 years, new headache, temporal artery tenderness or decreased temporal pulse, ESR > 50 mm/hr, biopsy findigs with granuloma or multinucleated giant cells.²

– > 3 criteria possesses sensitivity 93% and specificity 91%.²

Of note, this is a clinical diagnosis. These criteria were developed for research purposes, rather than acute diagnosis.

Evaluation: Obtain inflammatory markers including ESR and CRP. ESR is often 70-110, with 85% sensitivity. However, over 15% of patients have normal ESR. Close to 4% will have normal ESR and CRP.^4,5

– Normocytic anemia, thrombocytosis, and leukocytosis may be present (markers of systemic inflammation).^4,5

Management: Methylprednisolone 1 g IV daily for 3 days if the patient has vision symptoms or involvement of large vessels (aortitis).^3-5

– Other treatment options include prednisone 40-60 mg orally per day.

– Temporal artery biopsy is needed (within 1-2 weeks).

– Admission and rheumatology consultation is recommended.

References:

1. Smetana GW, et al. Does this patient have temporal arteritis? JAMA. 2002;287:92-101.
2. Hunder GG. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990; 33(8):1122-8.
3. 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-Hill
4. 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.
5. Jhun P, et al. Giant Cell Arteritis: Read the Fine Print! Ann Em Med. 2015; 65(5):615–617.