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Contents | Director | One | Two | Three | Four | Topic | EKG | Rad 1 | Rad 2 Albany Medical Review - January 2002 |
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Case Report-
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Monica Kaul, MD
Mr. JC is a 73-year-old caucasian man who presented for a work-up of fever of unknown origin. For the past 3 weeks, the patient had been having undulating fever of 101. Appropriate outpatient work-up had failed to reveal an etiology. Repeated lab work had consistently shown elevated ESR, and normocytic normochromic anemia. He suffered from a recent sinus infection for which he was being treated. He had complaints of increased fatigue and an unintentional 10-lb weight loss. Relevant past medical history included a 40 pack year smoking history and emphysema. Initial in-patient lab work failed to uncover any etiology. On further examination, the patient complained of mild fronto-temporal tenderness and jaw weakness after prolonged chewing. Physical exam was unremarkable. Laboratory findings were significant for elevated ESR (123) and normocytic normochromic anemia.
DISCUSSION Giant Cell Arteritis (GCA) is an inflammatory vasculopathy of the medium and large sized arteries, characteristically in the carotid artery and its branches. Although a systemic vasculitis that has widespread effect, it is known to spare the skin, kidneys and lungs. GCA is an ophthalmologic and systemic emergency with devastating consequences of blindness or death. It is a disease most commonly seen in caucasians, particularly among those of Scandinavian descent. It has an annual incidence rate varying from 0.49 to 27.3 per 100,000 persons greater than 50, making it the most common vasculitis in this age group. The high incidence seen among populations of Scandinavian descent, and some familial associations with HLA-DR4, is indicative of some genetic predisposition for the disease. Postmenopausal women are more likely to be affected suggesting a possible hormonal etiology, but yet to be investigated. Histologically, the lesions of giant cell arteritis consist of focal granulomatous inflammatory processes along the internal elastic lamina of the vessel wall. These lesions may lead to occlusion of blood vessels by proliferation of intima and fragmentation of internal elastic lamina. Weakening of the vessel wall has also been noted and can eventually lead to rupture. The inflammatory lesions of temporal arteritis have been noted to present in three forms. The classic one is the granulomatous lesion with giant cells within the internal elastic lamina previously described. The second consists of nonspecific inflammatory cells, such as lymphocytes and eosinophils, within the arterial wall. The third histological variation that is noted is intimal fibrosis within the internal elastic lamina with no morphologic disruption. In the management of giant cell arteritis it is important to consider your clinical suspicion for the disease, ESR, and the results of the temporal artery biopsy. The clinical suspicion for temporal arteritis is heightened by the presence of a few definable features. Most significant of these features are age greater than 50 and the presence of decreased pulsation and/or tenderness in the scalp region. Other definable features were elevated ESR, claudication of jaw or tongue, and pain on deglutition. Although, the presenting symptoms may be a combination of over 30 variables, the above-mentioned criteria correlate the highest with a diagnosis of GCA. The clinical suspicion for giant cell arteritis can be supported by the temporal artery biopsy. The sensitivity of the test has been reported to vary anywhere from 56-93%. Because of the segmental nature of the lesions, it is recommended that a biopsy between 2-5 cm in size be obtained for optimal analysis. If clinical suspicion is high, contra lateral temporal artery biopsy is recommended. Newer diagnostic techniques, such as color Doppler sonography that detects decreased blood flow velocity, have controversial diagnostic value. The treatment of the GCA is with corticosteroids. The recommended dose is between 40-60 mg qd. A higher dose is recommended depending on clinical severity (i.e.: the presence of ocular involvement). Intravenous methylprednisone has been noted to be effective initially in patients with vision loss, in some cases leading to restoration of vision. The duration of treatment and the tapering dosage of steroids are for the most part individualized. Clinical improvement and decreasing ESR are acceptable markers for monitoring resolution of disease. The average duration of giant cell arteritis is 1-2 years. Other immunosuppressive agents have yet to be proven clinically efficacious, but have been used in cases of steroid failure.
The overall prognosis with treatment is very good. Common problems noted after treatment are secondary to the side effects of steroid therapy. Recurrences of GCA have been reported.
REFERENCES
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30.01.2002 |
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