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Show *~, Journal of Neuro- Ophthalmology 20( 3): 2/ 9- 22/, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Editorials Giant Cell Arteritis Untreated giant cell arteritis ( GCA) has potentially devastating visual, neurologic, and systemic consequences. In particular, patients with untreated GCA have a significant risk of experiencing permanent unilateral or bilateral loss of vision from ischemic optic neuropathy, central retinal artery occlusion, or damage to the post-chiasmal visual pathways. There is increasing evidence that early diagnosis and treatment of GCA with high-dose systemic corticosteroids may not only prevent visual loss, but it also may partially restore vision in patients with arteritic ischemic optic neuropathy. The diagnosis of GCA usually is suspected on the basis of the clinical setting, the history, and the examination. It is supported by the finding of an elevated erythrocyte sedimentation rate, C- reactive protein, or both. The gold standard for diagnosis, however, is the temporal artery ( TA) biopsy. Only when there is a positive TA biopsy result can one make a definitive diagnosis of GCA. This is particularly important given the potential side effects of long- term steroids in elderly patients. In addition, there are disorders other than GCA, such as malignant tumors, that can produce clinical manifestations and laboratory abnormalities that mimic those of GCA. Therefore, it behooves the clinician caring for a patient with possible GCA to be as certain as possible that the patient actually has that disease before committing the patient to long- term therapy. The reports in this issue of the Journal of Neuro- Ophthalmology by Danesh- Meyer et al. ( 1) and Pless et al. ( 2) join a growing body of literature that indicates that performance of a bilateral TA biopsy, whether simultaneously or sequentially, increases the yield of a positive result ( 3- 9). Pless et al. ( 2) reviewed 60 bilateral TA biopsy results and reported a 5% chance of obtaining a positive biopsy result on one side and a negative biopsy result on the other side, whereas Danish- Meyer et al. ( 1) found a 1% discordance among 91 bilateral TA biopsies. Other authors have reported percentages of discordance ranging from 14.5% ( 4) to 3.3% ( 9). Danish- Meyer et al. ( I) performed a meta- analysis of existing literature and concluded that the overall chance of discordance is about 4%, which is a figure that agrees almost completely with the results of Pless et al. ( 2). Because performance of a bilateral TA biopsy increases the chance of obtaining a positive TA biopsy result, and therefore increases the probability that the clinician will be able to make a definitive diagnosis of GCA, to me, the only issue is whether or not the added yield of about 4% is sufficient to warrant the additional surgery. The risks of a TA biopsy are few ( 10). Fisher ( II) reported the case of a patient who experienced a stroke after biopsy of a temporal artery that was providing circulation to the brain in the setting of an internal carotid artery occlusion. Vollrath- Junger and Gloor ( 12) described three patients in whom a TA biopsy might have produced major complications. In one case, there was severe stenosis of the ipsilateral internal carotid artery, and the cerebral perfusion to the ipsilateral hemisphere was primarily dependent on the TA on that side. If that artery had been biopsied, the patient might have experienced a major stroke. In the other two cases, there was proximal stenosis of the ophthalmic artery, and biopsy of the ipsilateral TA might have resulted in blindness in the ipsilateral eye. Foged ( 13) reported chronic skin ulceration in the temporal region several months after a TA biopsy, and Slavin ( 14) reported a transient brow droop after a TA biopsy. Infection and hemorrhage are potential complications of any surgical procedure. I have never seen a patient whose biopsy site became infected, but I am aware of a patient in whom a large subcutaneous hematoma developed after a TA biopsy when the ligature slipped off the proximal end of the artery. The patient required emergency surgery to control the hemorrhage. All things considered, it is my opinion that the benefits of diagnosing GCA definitively far outweigh the risks of a bilateral TA biopsy. Therefore, regardless of whether the concordance rate for bilateral TA biopsies is 1%, as suggested by Danesh- Meyer et al. ( 1), or 5%, as suggested by Pless et al. ( 2), I would reiterate the recommendations made in the paper by Boyev et al. ( 9), of which I was a co- author: " Bilateral temporal artery biopsies should be considered in all patients in whom the diagnosis of GCA is suspected." The biopsies can be simultaneous or sequential; however, one must remember that if a unilateral TA biopsy is performed in a patient who has been placed on systemic steroids, and the specimen shows no evidence of GCA, there may a temptation to discontinue administration of the patient's steroids pending biopsy of the other side at a later date. This, in turn, may predispose the patient to the consequences of the disease. Neil R. Miller, MD Johns Hopkins Hospital Baltimore, Maryland REFERENCES 1. Danesh- Meyer HV, Savino PJ, Eagle RC Jr., et al. Low diagnostic yield with second biopsies in suspected giant cell arteritis. J Neu-roophthalmol 2000; 20: 213- 5. 2. Pless M, Rizzo JF III, Lamkin JC, et al. Concordance of bilateral temporal artery bipsy in giant cell arteritis. J Neuroophthalmol 2000; 20; 216- 8. 219 220 EDITORIALS 3. Klein RG, Campbell RJ, Hunder GG, et al. Skip lesions in temporal arteritis. Mayo Clin Proc 1976; 51: 504- 10. 4. Hall S, Lie JT, Kurland LT, Persellin S, et al. The therapeutic impact of temporal artery biopsy. Lancet 1983; 2: 1217- 20. 5. Hedges TR III, Geiger GL, Albert DM. The clinical value of negative temporal artery biopsy specimens. Arch Ophthalmol 1983; 101: 1251- 4. 6. Hall S, Hunder GG. Is temporal artery biopsy prudent? Mayo Clin Proc 1984; 59: 793- 5. 7. Hayreh SS, Podhajasky PA, Zimmerman B. Giant cell arteritis: validity and reliability of various diagnostic criteria. Am J Ophthalmol 1997; 123: 285- 96. 8. Ponge T, Barrier JH, Grolleau JY, et al. The efficacy of selective unilateral temporal artery biopsy versus bilateral biopsies for diagnosis of giant cell arteritis. J Rheumatol 1988; 15: 997- 1000. Biopsy of the superficial temporal artery is so safe and simple that it can be, and usually is, performed by an ophthalmologist. The most common complication, in my experience, has been failure to identify the artery. There is general agreement that one should obtain a long specimen and that the pathologist should sample it sub-serially, lest missing a segmentally involved artery lead to a falsely negative diagnosis. However, there is no consensus among the physicians who evaluate patients for giant cell arteritis concerning the value of biopsy. Some authorities consider biopsy superfluous in those cases in which the symptoms, signs, and laboratory results are classical. Admittedly, it is usually prudent in those cases to institute corticosteroid treatment, even if results of the biopsy prove to be negative. However, when patients develop serious untoward side- effects of long- term corticosteroid treatment and physicians counsel discontinuation of treatment, histopathologic proof of the diagnosis helps to buttress the case for continued therapy. Premature cessation of treatment can lead to blindness or stroke. Three publications, two of them in this issue of the Journal of Neuro- Ophthalmology, take the biopsy controversy a step further. These papers concern the value of bilateral temporal artery biopsy, and although there are methodologic differences among them, similar results are reported. The investigators in all three publications retrospectively analyzed the results of bilateral temporal artery biopsies in case series from their parent hospitals. The numbers of cases are impressive, and the authors agree that when both temporal arteries are biopsied in patients suspected to have giant cell arteritis, there is a very high concordance of diagnosis between the two sides. There are few exceptions. Boyev et al. ( 1), reporting the Wilmer Institute's experience with 150 patients who had simultaneous bilateral biopsies and 36 patients in whom the second side was biopsied after an interval, found that almost 3% were discordant. Pless et al. ( 2) reviewed bilateral biopsies from 60 patients from the Massachusetts Eye and Ear Infirmary and determined that there was a 5% chance of having a positive biopsy test result 9. Boyev LR, Harris LL, Miller NR, et al. Efficacy of unilateral versus bilateral temporal artery biopsies in detecting temporal arteritis. Am J Ophthalmol 1997; 128: 211- 5. 10. Stuart R. Temporal artery biopsy in suspected temporal arteritis: a five- year survey. NZ Med J 1989; 102: 431- 3. 11. Fisher CM. Discussion of Schlezinger NS, Schatz NJ. Giant cell arteritis ( temporal arteritis). Trans Am Neurol Assoc 1971 ; 96: 12. 12. Vollrath- Junger C, Gloor B. Warum eine Dopplersonographie vor jeder Biopsie der A. temporalis? Klin Monatsbl Augenheilkd 1989; 195: 169- 71. 13. Foged EK. Chronic ulceration following biopsy of the temporal artery. Hautarzt 1981; 32: 647. 14. Slavin ML. Brow droop after superficial temporal artery biopsy. Arch Ophthalmol 1986; 104: 1127. on the second side after the first result was negative. Danesh- Meyer et al. ( 3) found that the diagnosis on the two sides was discordant in only 1 of 91 patients who underwent bilateral biopsies at the Wills Eye Hospital. Based on these studies and on several others referenced in these articles, it is clear that only a very small percentage of patients with giant cell arteritis will have a negative temporal artery biopsy result on one side and a positive biopsy result on the other side. However, despite the low risk of missing the diagnosis, because the stakes are so high, it makes sense to routinely perform bilateral biopsies or to biopsy the other side if the first side has a negative result in patients whose symptoms, signs, and laboratory results point to the diagnosis of giant cell arteritis. I prefer examination of specimens after routine paraffin embedding, rather than frozen sectioning, for the same reasons as enumerated by Danesh- Meyer et al. ( 3). In those patients whose clinical or laboratory findings are atypical, a unilateral negative biopsy result should suffice. This would include elderly patients without visual or ocular motor symptoms who are referred for biopsy by non- ophthalmologists because of new headache or an unexplained elevation of the erythrocyte sedimentation rate. It would be dangerous to sanctify these or any set of such algorithmic recommendations as so- called " practice guidelines." To do so could make clinicians potentially vulnerable to unjustified medical negligence suits. Practicing medicine algorithmically can make a good clinician out of a bad clinician, but it won't make a good clinician into a great clinician. The great clinician is the one who can recognize when the rules do not apply. Simmons Lessell, MD Massachusetts Eye and Ear Infirmary Harvard Medical School Boston, Massachusetts REFERENCES 1. Boyev LR, Harris LL, Miller NR, et al. Efficacy of unilateral versus bilateral temporal artery biopsies in detecting temporal arteritis. Am J Ophthalmol 1997; 128: 211- 5. Bilateral Temporal Artery Biopsies in Giant Cell Arteritis J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 EDITORIALS 221 2. Pless M, Rizzo JF III, Lamkin JC, et al. Concordance of bilateral temporal artery biopsy in giant cell arteritis. J Neuwophthalmol 2000; 20: 216- 8. Giant Cell Giant cell arteritis ( GCA) is a disease that may cause blindness, stroke, or death if the diagnosis is not made or if the treatment is delayed or inadequate. The classical symptomatology of GCA or polymyalgia rheumatica, when present, is helpful for establishment of the diagnosis, but it may be absent, such as in the so- called " occult" GCA. Results of several laboratory studies also may be abnormal in GCA and may provide valuable clues to the diagnosis. However, neither the erythrocyte sedimentation rate nor the C- reactive protein determination is 100% specific or sensitive for the presence of the disease. The single best test to determine the presence of GCA is the temporal artery biopsy. A positive biopsy result, with or without giant cells in the specimen, unequivocally establishes the diagnosis of GCA. It has been recognized that a patient may have GCA but have a negative temporal artery biopsy result when only one temporal artery is sampled. Three recently published papers from three ophthalmic institutions ( 1- 3) have shown remarkably similar results regarding the concordance of bilateral temporal biopsies. The possibility of one temporal artery result being negative for the changes of GCA and the contralateral artery result being positive, as long as the biopsy is of adequate length, appears to range from 1% to 5%. Given these similar data, what recommendations regarding temporal artery biopsy may be advanced when faced with a patient with possible GCA? First of all, a strong case can be made for performance of a temporal artery biopsy in every patient suspected to have GCA. Positive pathology results are the most specific " test" for GCA. A positive biopsy result will also discourage the internist or other treating physicians from lowering or discontinuing the corticosteroid treatment when side effects develop. Beyond this, should one or both temporal arteries undergo biopsy? The guidelines in the three papers differ. The options are: 3. Danesh- Meyer HV, Savino PJ, Eagle RC Jr., et al. Low diagnostic yield with second biopsies in suspected giant cell arteritis. J Neuwophthalmol 2000; 20: 213- 5. Arteritis 1. Perform a frozen section on the initial temporal artery segment. If results of the frozen section fail to demonstrate the diagnostic changes of GCA, the contralateral side should undergo biopsy. 2. Perform a unilateral biopsy. If results are negative, the contralateral side should undergo biopsy at a separate surgery only if the clinical suspicion for GCA is high. 3. Perform bilateral biopsies routinely at the same setting. Each of these options may be acceptable in certain clinical situations. Unilateral biopsy may be chosen as the appropriate procedure when the clinical and laboratory data are overwhelmingly suggestive of the presence or absence of GCA. The clinician should be aware, however, that there is a definable risk of missing the diagnosis if only one temporal artery is sampled. Therefore, to provide the best opportunity for establishment of a diagnosis of GCA so the patients may be treated promptly and appropriately, performance of bilateral temporal artery biopsy, with or without the aid of frozen sections, appears to be the safest strategy. Peter J. Savino, MD Wills Eye Hospital Thomas Jefferson Medical College Philadelphia, Pennsylvania REFERENCES 1. Boyev LR, Miller NR, Green WR. Efficacy of unilateral versus bilateral temporal artery biopsies for the diagnosis of giant cell arteritis. Am J Ophthalmol 1999; 128: 211- 3. 2. Pless M, Rizzo JF III, Lamkin JC, et al. Concordance of bilateral temporal biopsy in giant cell arteritis. J Neuroophthalmol 2000: 20; 216- 8. 3. Danesh- Meyer HV, Savino PJ, Eagle RC Jr., et al. Low diagnostic yield with second biopsies in suspected giant cell arteritis. J Neuroophthalmol 2000; 20: 213- 5. J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 |
