Article, Rheumatology

Giant cell temporal arteritis with a normal erythrocyte sedimentation rate: report of a case

Case Report

Giant cell temporal arteritis with a normal erythrocyte sedimentation rate: report of a case

Abstract

Giant cell temporal arteritis (GCA) is a systemic vasculitis that affects individuals older than 50. erythrocyte sedimentation rate has typically been used in the ED as a screening test to rule out GCA. There have been documented cases in medicine and rheumatology journals of biopsy-proven giant cell arteritis with a normal or low ESR before Steroid therapy. In the ED, the practitioner must use multiple resources to make the diagnosis of GCA, as there is no specific test that establishes the diagnosis. Delaying this diagnosis based on a laboratory value can create substantial morbidity and mortality for the patient. We report a case of an Elderly woman who developed visual changes and had a delayed diagnosis because of a normal ESR value.

A healthy, active 83-year-old woman presented to the ED complaining of intermittent, left eye visual changes for 2 weeks. She noticed episodes of painless, blurry vision of her left eye with diplopia, associated with a left frontal headache. These symptoms had gotten more frequent resulting in 10 episodes, each lasting 15 minutes, in the previous 7 days. She reported no prior episodes of eye complaints, no history of cerebrovascular disease, and no vertigo or other focal deficits. She did not wear corrective lenses. The patient’s only other medical problem was hypothyroidism.

On examination, the patient was well appearing. Her vital signs were entirely normal. On eye examination, the patient’s pupils were equal, round, and reactive to light bilaterally; her lids and lashes were normal; and she has no conjunctival injection. visual acuity examination revealed 20/25 bilater- ally with pinhole occluder. On fundoscopic examination, there were no structural abnormalities of the eye. The patient’s retinal vessels appeared normal and she had no optic disc edema. On head examination, there was no tenderness to palpation over the temporal arteries. Heart, lung, and Abdominal examination were within normal limits. On musculoskeletal examination, the patient had full, painless range of motion. Neurologic examination result was normal.

The patient underwent computed tomography angiogra- phy of her head and neck, which was unremarkable. She had a normal ESR of 16 mm/h, C-reactive protein (CRP) of 6.9 mg/L, hemoglobin of 13.7 g/dL, white blood cell count of 6700/mm3, and a platelet count of 260000 per cubic millimeter. She was discharged with ophthalmology follow-up.

The patient returned to the ED 1 week later with acute, painless Visual loss to her left eye that occurred 9 hours before presentation. She also had a sharp left-sided frontal headache. Her physical examination was unchanged except for the left eye visual deficit and significant left optic disc edema on fundoscopic examination. The patient’s laboratory results were not remarkably different, with a mild increase in the patient’s ESR to 27 mm/h and CRP to 19.3 mg/L.

Despite the patient’s low ESR, her presentation was worrisome for GCA, and she was started on steroids in the ED. In several days, the patient had a temporal artery biopsy performed, confirming the diagnosis of GCA. To date, the patient’s vision has not significantly improved despite therapy.

Giant cell temporal arteritis is a vasculitis affecting medium- to large-sized arteries. Prevalence increases with age, and women are more commonly affected than men. The histologic marker of GCA is mononuclear cell infiltration involving all layers of the arterial wall. This results in nonthrombotic occlusion of the lumen second- ary to intimal hyperplasia [1]. In 80% to 90% of patients with GCA, the vasculitis affects the extracranial carotid arterial tree including the superficial temporal, vertebral, and ophthalmic arteries. It less frequently affects the internal and external Carotid arteries and the central retinal arteries. Ophthalmic artery inflammation produces a sudden, painless, usually permanent visual loss. Visual blurring and diplopia usually precede partial or complete blindness [2].

The clinical diagnosis of GCA can be made when at least 3 of 5 criteria are met. They are:

  1. Age at onset 50 years or older
  2. New headache (new onset or new type of headache)
  3. Temporal artery abnormality (tenderness to palpation or decreased pulsation)
  4. Elevated ESR (>=50 mm/h)

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255.e2 Case Report

  1. Abnormal artery biopsy (vasculitis with mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells)

Our patient met 3 of the 5 diagnostic criteria, including age 50 years or older, new headache, and an abnormal artery biopsy.

Although the ESR has traditionally been used as a screen for GCA, it is only one of 5 criteria for diagnosis. In a study of 240 patients with GCA, only one had an ESR less than 40 mm/h [3]. However, another study showed that an elevated ESR has a sensitivity of only 76% to 86% [4]. There have been well-documented cases and studies in medicine and rheumatology literature of biopsy-proven GCA with a normal or low ESR before steroid therapy [5-11], yet the ED practice of using the ESR as a screen persists. Some authors have advocated using a lower cut-off value for the ESR of 30 mm/h, as most patients will not have a truly normal ESR. Our patient would still have been missed by these criteria, as she had 2 ESR values of less than 30 mm/h. A low ESR may be seen in polycythemia, congestive heart failure, and in patients taking anti-inflammatory drugs [5,6]. In our patient, there were no significant comorbidities and no acute or chronic use of anti-inflammatory medications to affect the ESR.

C-Reactive protein may have a higher sensitivity (97.5%), but there have been cases reported of patients with a normal CRP and GCA revealed on biopsy. The advantage of using CRP in comparison to ESR, when attempting to diagnose GCA, is that it is not influenced by age or other hema- tological factors [4]. The cut-off value typically used for a positive CRP is greater than 5 mg/L. One study showed that patients with a CRP value less than 50 mg/L more commonly had visual manifestations [5]. Our patient had a mildly elevated CRP on her second visit, 19.3 mg/L. Thrombocy- tosis has also been associated with GCA, but is not diagnostic [3]. As no laboratory tests have perfect sensitivity for GCA, reliance on them can result in a delay to diagnosis, resulting in substantial morbidity and mortality, as occurred with this patient.

Computed tomography and magnetic resonance imaging

[12] may add diagnostic information by detecting arterial wall abnormalities. Doppler ultrasound [13] may detect a stenosed lumen. Temporal artery biopsy, however, remains the diagnostic gold standard.

Corticosteroids are the primary therapy to suppress clinical manifestations of GCA. Steroids do not reverse intimal hyperplasia, but lower ischemic insult by reducing tissue edema, thereby improving vision. Daily prednisone is given until improvement occurs [14,15]. The incidence of GCA-related blindness has been lowered to 7% to 25% with the introduction of corticosteroids when started early in the progression of visual changes [16]. Steroid treatment reduces the likelihood of a positive biopsy, but biopsy sensitivity is not significantly affected if it is performed within 2 weeks of therapy initiation [17]. If clinical

suspicion is high for GCA in the ED, steroids should not be withheld.

ED physicians must use multiple resources to make the diagnosis of GCA. The most important piece of information, however, is the clinical presentation. Giant cell temporal arteritis presents in many ways. Some patients have systemic manifestations (headache, neck pain, jaw claudication, abnormal temporal arteries, or general malaise), some have ocular manifestations (eye pain, diplopia, or visual loss), others have a mixture of manifestations, and some have occult presentations [18]. One must use the entire clinical picture as well as ancillary tests for guidance when attempting to establish this diagnosis. Ancillary tests cannot rule out GCA, so it is important that steroids not be withheld based on an ESR value if the diagnosis of GCA is suspected. It has been proven that the early initiation of steroids substantially reduces the risk of GCA-related blindness [14,15]. If steroids were started during our patient’s initial visit when she was experiencing blurry vision, this may have spared her eventual Vision loss.

Michael Ciccarelli DO Donald Jeanmonod MD Rebecca Jeanmonod MD

Department of Emergency Medicine Albany Medical College, Mail Code 139

Albany, NY 12208, USA

E-mail address: [email protected] doi:10.1016/j.ajem.2008.06.032

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