Giant Cell Arteritis Presenting with Headache and Jaw Pain

Author: V. Dimov, M.D., Assistant Professor at University of Chicago, Allergist/Immunologist, Internist
Reviewer: S. Randhawa, M.D., Allergist/Immunologist, Internist, Fort Lauderdale, FL

A 75-year-old African American female (AAF) who is a nursing home (NH) resident started to complain of occasional jaw pain 4 months ago. She also began complaining of headache 3 weeks ago, which is throbbing, rated as 5/10 on 0-10 scale, worse in the afternoon, and radiating down the neck.

Past medical history (PMH)

CVA with right hemiparesis, depression, atrial fibrillation (AFib), osteoarthirits (OA), gout, diabetes mellitus type 2 (DM2).

Medications

ASA, clonidine, Colace (docusate), furosemide (Lasix), glyburide, lisinopril, metoprolol, multivitamin (MVT), nifedipine, Ambien (zolpidem), Elavil (amitriptyline).

Physical examination

WD/WN sitting comfortably in a wheelchair.
VSS.
HEENT: NC/AT, temporal arteries palpable, slightly painful on the right.
Neuro: R-sided hemiparesis.
The rest of the physical exam was WNL.


Outline of side of face, showing the main surface markings. The superficial temporal artery is visible at center, to the left of the ear. Image source: Gray's Anatomy, public domain.

What is the most likely diagnosis?

Tension headache.
Headache due to cervical spondylosis.
Headache due to TMJ dysfunction.
Temporal arteritis.

What is the one test that you should do first?

Erythrocyte sedimentation rate (ESR), followed by eye examination - visual acuity and fundoscopy.

The most notable complication of giant cell arteritis is blindness, which may occur in 10 to 15 percent of untreated patients.

What happened?

ESR was 83 mm/hr.

The patient was started on prednisone 60 mg PO QAM. She felt better and her pain decreased to 3/10 on a 0-10 scale. A rheumatology consultation was called and a bilateral temporal biopsy was done. The temporal artery biopsy showed temporal arteritis on the right.

What happened next?

You probably recall that the patient has a history of diabetes. Her glycosylated hemoglobin (HbA1c) was 10% one year ago. With better glucose control, HbA1c decreased to 7% measured six months ag.

After starting the steroids, 3 weeks ago, the patient's blood glucose has been frequently above 200 mg/dL despite increasing her glyburide dose to 5 mg PO BID.

What to do to control the steroid-induced hyperglycemia?

We have 3 options:

- decrease prednisone dose
- increase glyburide dose
- add metformin
- use methotrexate as a steroid-sparing agent

Treatment with dose steroids for temporal arteritis typically continues for at least 4-6 weeks and the response is monitored by symptoms and ESR. The steroid dose is slowly tapered over the course of 9-12 months.

What happened next?

ESR was ordered. Metformin as added to the treatment regimen. The patient will be seen by a rheumatologist to consider treatment with methotrexate.

Final diagnosis

Temporal arteritis. Hyperglycemia as a side effect to steroid treatment in a patient with diabetes.

What did we learn from this case?

Suspect temporal arteritis in elderly patients with headache and/or jaw pain.

Temporal arteritis (TA), also known as giant cell arteritis (GCA), is a common form of systemic vasculopathy affecting patients older than 50 years. This inflammatory process has been shown to involve the aorta, carotid, subclavian, vertebral, and iliac arteries, therefore, “giant cell arteritis” may be more appropriate than “temporal arteritis” to identify this type of vasculitis, though both terms are used interchangeably.

ESR is a sensitive test for temporal arteritis. Most patients with temporal arteritis have an ESR greater than 80 mm/h. However, up to 20% of patients with temporal arteritis may have a normal or low ESR. Normal ESR level can not exclude a diagnosis of temporal arteritis.

Treatment of giant cell arteritis should not be delayed while awaiting biopsy.

Based on the 1990 American College of Rheumatology criteria for classification of temporal arteritis, at least 3 of the following 5 items must be present (sensitivity 93.5%, specificity 91.2%):

- Age of onset older than 50 years
- New-onset headache or localized head pain
- Temporal artery tenderness to palpation or reduced pulsation
- Erythrocyte sedimentation rate (ESR) greater than 50 mm/h
- Abnormal arterial biopsy (necrotizing vasculitis with granulomatous proliferation and infiltration)

When treating patients with steroids, monitor for increased blood glucose, blood pressure, and mood changes.

Improvement of systemic symptoms typically occurs within 72 hours of initiation of therapy. Corticosteroid therapy may last for 1-2 years, depending on the patient’s response.

References

Giant cell arteritis: Suspect it, treat it promptly. Cleveland Clinic Journal of Medicine April 2011 vol. 78 4 265-270.
Temporal Arteritis: Overview. eMedicine Emergency Medicine, 2008.
Management of Giant Cell Arteritis and Polymyalgia Rheumatica. AFP, 2000.
Polymyalgia Rheumatica and Giant Cell Arteritis. AFP, 2006.

Related reading

Waking up to darkness. What is the diagnosis? Giant cell arteritis. BMJ, 2011.

Published: 03/02/2005
Updated: 03/07/2011

13 comments:

  1. As a Rheumatologist I would suggest a consult.Most if not all Rheum's would get a bilateral biopsy at the same time as initiating therapy with prednisone.A negative biopsy (approx. 40% of active TA cases)does not help but a positive bx is very helpful.I would start low dose methotrexate (10 to 15 mg po or SQ weekly).This has been show to be steriod sparing, allowing better control of blood sugars.

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  2. maybe an extension of the bbp mnemonic

    4 or 5 B
    B P
    B GL
    B ones
    Brain
    Bruising

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  3. Hello
    I'm finding it a little confusing that international nonproprietary names are not used. I'm also surprised that she wasn't prescribed metforming for diabetes.

    Look forward to learning from more cases. Thank you.

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  4. Thank you for your questions and suggestions. Some of them were incorporated in the update of this clinical case from 03/07/2009.

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  5. What should we do if the patients has a painfull vesicular rash in her face( possible herpes Zoster) pluse temporal pain and vision problems (temporal arteritis).

    1.Should we give prednisone for temp arteritis while we wait for results from the tsanck smear. "We can not wait for temporal biopsy or ESR to guive treatment"

    2.Or should we guive (acyclovir and prednisone) "covering herpes and temporal arteritis . Using only a clinical diagnosis (observation of the rash) for herpes. Any one?????????

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  6. If you have a clinical diagnosis, you treat appropriately, and in the meantime you verify or rule out your diagnosis with laboratory tests.

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  7. Well for real life this is fine but for the CCS step 3 exam this is a problem. In the step 3 exam This case would end by the time we get the result from the tsanc smear without giving the correct treatment (acyclovir) for someone with possible herpes. So what should be the right treatment aproach for possible herpes and temporal arteritis in the the step 3 CCs exam???? Any one??? Corticoids and acyclovir riht at the beginig of the diagnosis??

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  8. Where did you see anything about rash? Why you should treat for temporal arteritis and herpes zoster at the same time? This will never be an option on Step 3, relax... :)

    You can only do Tzank smear if the patient has vescicles (rash) which this patient does not have. The most important skill you need for USMLE is to be able to read the questions carefully.

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  9. That is one of the cases for CCs patient with a facial rash like herpes and temporal artheritis symtoms..

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  10. Would this automimmune disorder affect the results of the HbA1c test?

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  11. "Would this automimmune disorder affect the results of the HbA1c test?"

    No. However, steroids are often given to treat automimmune disorders and they increase the blood glucose. Blood glucose increase leads to elevation in HbA1c. As the automimmune disorder is better controlled, the steroid dose can be decreased or even eliminated, this leads to a decrease in HbA1c and this is what happened with this patient.

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  12. I have had temporal arteritis for the last two years. After the biopsy I have been on Prednisone 5mg. I am now down to 1mg per day but find the problem is still strong in arms, legs and pelvic region
    and neck. Is there another way I can relieve this pain. I walk frequently which temporarily helps, but it is quite debilitating for me. Any advice would be appreciated

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  13. I would see a rheumatologist if I were you (if you are not already seeing one). Staying on prednisone for 2 years is stretching it a bit. Unless it is absolutely necessary, of course.

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