E-Case 5-C

Vyn Reese, M.D.

February 18-March 1, 2002

HPI: Mrs. G is a 73 year old woman complaining of pain in the right side of her face for the last three or four days. She is concerned she may have an infected tooth since she had root canal done one week previously.  She reports the pain is made worse by chewing but is present all the time. She thinks she may have had a low-grade fever at home but hasn't checked it. She denies any difficulty swallowing, visual disturbance, myalgias or any other systemic complaint.

• Medication: 1. Estrogen 0.625 mg.po q d. 2. Maxzide 25/37.5 po q d.

• PMH: s/p hysterectomy and bilateral SPO in distant past, s/p right total hip replacement 3 years ago, longstanding hypertension, and a history of polymyalgia rheumatica, finished a two year course of prednisone one year ago.

• Social history: Mrs. G is a widow, she lives alone and is a nondrinker/nonsmoker.

• Family history and extended ROS is negative.

• Px: Mrs. G is a pleasant woman with a red streak just inferior to her right TMJ

  • B/P 136/82  P88 T. 99.0 F
  • HEENT:  1x3 cm erythematous streak just inferior to right TMJ.  New crown with some surrounding erythema and swelling in the right second upper molar. Temporal arteries are normal.Visual acuity is at her baseline bilaterally corrected.Neck: normal carotids, no thyromegaly, or masses, no palpable lymphadenopathy.
  • Chest: clear to A&P Cor: no murmurs, rubs or gallops.
  • Abdomen: benign
  • Extremities: normal ROM at the shoulders, slightly diminished ROM right hip in all directions but nontender.  Left hip ROM normal.
  • Lab: Normal CBC, ESR 38 mm per hour.

Question:

What would you do now? Would you do any further diagnostic tests or treatments?

The decision was made to treat Mrs. G with a high dose oral cephalosporin and to refer her back to her dentist.

One week later Mrs. G is seen in follow-up c/o a severe right sided headache that has gotten much worse despite taking the antibiotics. She denies other symptoms except she thinks her fever is higher. Her dentist can't find anything wrong with her tooth. Her physical exam is remarkable for tenderness, erythema, and prominence of her right temporal artery and its pulse is diminished. Her temperature is 100.0 F. The red streak on the right side of her face has resolved. Lab was remarkable for an ESR of 88 mm. per hour. CBC and Comprehensive Chemistry panel were normal.

Questions

1. What treatment would you order?
2. What diagnostic test would you order?

1. It was felt the most likely diagnosis at this point was temporal arteritis and high dose prednisone was ordered. Treatment with corticosteroids can't be delayed if this diagnosis is seriously entertained because progressive loss of vision from this disorder is common. Treat before the biopsy results are available. The biopsy should remain positive for at least two weeks on high dose prednisone therapy.

2. A temporal artery biopsy. Although at this point the diagnosis is fairly certain it should be confirmed given the morbidity of long term steroids. The biopsy of the right temporal artery was positive for Giant Cell Arteritis.

What features of this case made temporal arteritis the most likely diagnosis and led to the biopsy?

Mrs. G. likely had jaw claudication although this symptom was obscured by her recent dental work. True jaw claudication is very specific for temporal arteritis and markedly increases the likelihood of a positive biopsy. Palpably tender, prominent temporal arteries or beaded temporal arteries on physical exam also raise the likelihood of a positive biopsy. It is also unusual to have temporal arteritis without an elevated sedimentation rate. The most unusual aspect of this case was the occurrence of temporal arteritis after treatment for polymyalgia rheumatica.  These two disorders frequently overlap but PMR responds classically to low dose steroids while much higher doses are needed to treat temporal arteritis.  PMR is much more common than temporal arteritis.  The diagnosis of temporal arteritis is often more difficult than this classic presentation. In any patient over age fifty with a severe, new onset headache and an elevated sedimentation rate temporal arteritis needs to be high in the differential diagnosis even if the history and physical exam are negative.  Untreated temporal arteritis leads to unilateral or bilateral blindness in approximately twenty percent of cases.

References

1. Does This Patient Have Temporal Arteritis? Smetana et al, JAMA, Jan. 2, 2002-Vol 287, No. 1pp. 92-101.

2. Giant Cell Arteritis and Polymyalgia Rheumatica, Hunder, Medical Clinics of North America, Vol. 81, No. 1, Jan. 1997, pp. 195-219.

3. Risk Factors for Visual Loss in Giant Cell [Temporal] Arteritis:Prospective Study of 174 Patients. Liozon et al, The American Journal of Medicine, Aug. 15, 2001, Vol. 111, pp.211-217.