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Show Journal ofNeuro- Ophthalmology 20( 3): 213- 215, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Low Diagnostic Yield With Second Biopsies in Suspected Giant Cell Arteritis Helen V. Danesh- Meyer, MD, Peter J. Savino, MD, Ralph C. Eagle, Jr., MD, Kenneth C. Kubis, MD, and Robert C. Sergott, MD Objectives: The clinical diagnosis of giant cell arteritis may be confirmed with a biopsy of the superficial temporal artery. Because of " skip lesions," a histologic diagnosis of giant cell arteritis may be missed with a unilateral biopsy. The authors report a study that investigates whether a biopsy of the contralateral superficial temporal artery provides any additional information for confirmation of a diagnosis of giant cell arteritis. Methods: Available medical records of 91 consecutive patients who underwent bilateral superficial temporal artery biopsy procedures were reviewed. Information that was abstracted included sequence of biopsy procedures, length specimens, and histologic diagnosis. Microslides from all biopsy specimens were retrieved and reexamined in a masked fashion by the ocular pathologist ( RCE) who had made the original diagnoses. Results: Seventy- two bilateral simultaneous superficial temporal artery biopsies and 19 bilateral sequential biopsies were performed. The mean length of biopsy specimens was 23 mm, and the mean length of the total artery removed from each patient was 33 mm. The pathologist's original diagnosis and the diagnosis at reexamination were in 100% agreement. In 90 ( 99%) of the 91 patients, the histologic diagnoses in the left and right superficial temporal arteries were the same. This is a concordance rate of 98.9% ( 38 of 39 positive biopsy results) among the positive biopsy results. Conclusion: There is a low yield of information from a second temporal artery biopsy in patients with suspected giant cell arteritis. This suggests that patients who present to the ophthalmologist with possible giant cell arteritis will, in most cases, have a similar diagnosis on both temporal artery biopsies if the specimens are adequate. Key Words: Giant cell arteritis- Histology- Temporal artery biopsy. Giant cell arteritis ( GCA) is a systemic vasculitis of medium and large arteries that may result in permanent visual loss in 15% to 20% of patients ( 1). The clinical diagnosis may be confirmed with a biopsy of an arterial Manuscript received March 15, 2000; accepted May 1, 2000. From the Neuro- Ophthalmology Service ( HVD- M, PJS, KCK, RCS) and the Pathology Department ( RCE), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania. Address correspondence and reprint requests to Helen Danesh- Meyer, MD, Neuro- Ophthalmology Service, Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA 19107. segment, such as the superficial temporal artery. Because of " skip lesions," however, sampling longer arterial segments may help to improve the diagnostic accuracy. For similar reasons, it has become common practice in many centers to investigate suspected GCA with bilateral, rather than unilateral, superficial temporal artery biopsies. We report a study that investigates whether a second biopsy provides any additional information in this setting. MATERIALS AND METHODS Available medical records were reviewed of all 91 patients who had bilateral superficial temporal artery biopsy procedures at Wills Eye Hospital between January 1988 and December 1998. The following information was abstracted: sequence of biopsy procedures, length of biopsy specimens, and final histologic diagnosis. Microslides from all biopsy specimens were retrieved and reexamined in a masked fashion by the ocular pathologist ( RCE) who made the original diagnoses. All specimens were presented as individual biopsies and not as pairs belonging to one patient; this was done to eliminate the possibility that the result of the first biopsy would bias the interpretation of the other side. The histopathologic criterion for the diagnosis of GCA was the same for both assessments, i. e., a chronic granulomatous vasculitis characterized by a mononuclear infiltrate in the media or adventitia that contained epithelioid histiocytes, but not necessarily giant cells. RESULTS Seventy- two patients underwent left- and right- sided arterial biopsies during the same operation, and 19 patients underwent biopsy as separate procedures several days apart. The mean length of the biopsy specimens was 23 mm ( range, 4- 41), and the mean length of the total artery removed from each patient was 33 mm ( range, 10- 65). For all 182 specimens, there was perfect concordance between the pathologist's original diagnosis and the diagnosis at reexamination. In 90 ( 99%) of the 91 patients, the histologic diagnoses in the left and right 213 214 H. V. DANESH- MEYER ET AL. superficial temporal arteries were the same. This is a concordance rate of 98.9% ( 38 of 39 positive biopsy results) among positive biopsy results. The one discordant pair involved arterial samples of an especially short length ( 4 mm and 6 mm). ( Table 1) There was no history of subsequent visual loss from GCA after a follow- up of 3 months in any patient with negative biopsy results. DISCUSSION The current study of 182 arterial specimens in 91 individuals finds high concordance between the histologic diagnosis of GCA in the two biopsy specimens taken from different arteries in the same individuals; there was only one discordant pair, which was probably a result of inadequate sampling by the surgeon. Moreover, the reproducibility of the histologic findings in our tertiary referral center was 100%. Only a few previous studies are directly comparable to the current report because some involved frozen tissue specimens ( 2), rather than the more commonly used fixed tissue samples described here, or they involved patients in different settings ( e. g., rheumatology practices) ( 3,4). When taken together, the current study and the previous studies performed in similar populations using similar histologic methods ( 5- 8) suggest that there is a low yield of information from a second temporal artery biopsy in patients with suspected GCA, as indicated by the pooled concordance rate of 96% ( 95% confidence interval, 93%- 99%) among these different studies ( x2 = 2.3; 2P < 0.01) ( Table 2). This suggests that patients who present to the ophthalmologist with possible GCA will, in most cases, have a similar diagnosis for both temporal artery biopsies if the specimens are adequate. This raises the issue of whether a unilateral biopsy is adequate to exclude a diagnosis of suspected GCA. Certainly, the probability of missing a diagnosis of GCA is low. Combining all the available studies, it is suggested that there is a 4% chance that the unilateral biopsy alone will fail to establish the correct diagnosis. However, this is not a negligible amount. Giant cell arteritis is a potentially life- threatening and sight-threatening condition. Furthermore, most patients will present to the ophthalmologist with uniocular involvement. A delay in the diagnosis may result in involvement of the other eye in approximately one third of patients. To realize that 4% of patients may go blind from a preventable cause is not acceptable, given the readily available treatment. For this reason, many referral centers will routinely perform frozen section diagnosis on the temporal artery biopsy. If the frozen section results are negative, a biopsy TABLE 1. Concordance between right and left superficial temporal arteries Right artery Positive Negative Left artery Positive Negative 38 Nil 1 52 TABLE 2. Studies evaluating the efficacy of bilateral temporal artery biopsies Biopsy pairs Author Discordant Total Percentage (%) Ophthalmology setting Klein et al. ( 1976) Boyev et al. ( 1999) Hedges et al. ( 1983) Hayreh et al. ( 1988) leumatology setting Halletal. ( 1983) Ponge et al. ( 1988) Hall et al. ( 1984) 7 6 3 7 15 18 33 187 182 63 76 103 189 234 of the contralateral side is performed. We have several concerns regarding this approach. First, in one study, frozen section diagnosis was shown to have a false-negative rate of approximately 18% ( 3). This means that a patient who is suspected to have GCA may be denied treatment for several days on the basis of a negative frozen section result, until the results of the fixed specimen become available. This is a window of vulnerability for profound bilateral visual loss. Second, frozen sections are not available to all clinicians that perform temporal artery biopsy. Finally, compulsory frozen sections increase the cost of medical care. Another approach is to perform a unilateral temporal artery biopsy as standard protocol and to perform a second biopsy only for patients with an initial negative biopsy and a high clinical index of suspicion for the disease. This is common policy in many places throughout the world. Given the very low rate of discordance between temporal artery biopsy pairs, performance of a unilateral temporal artery biopsy with the option of a second biopsy in highly suspicious cases is an acceptable approach. However, it is dependent on several variables. The quality of the pathology service and the presence of a surgeon who can consistently obtain an adequate biopsy are crucial. The decision to do the second biopsy rests on the clinical experience of the treating physician. There are no signs or symptoms that guarantee a positive biopsy result, although when certain features are present, e. g., jaw claudication, new- onset headache, pallid optic disc swelling, or high erythrocyte sedimentation rate, a diagnosis of GCA is much more likely. However, occult GCA is well documented, occurring in 21% of patients with GCA ( 9). Furthermore, it appears that patients who present with symptoms based more in the rheumatologic spectrum of GCA ( e. g., arthralgias, constitutional symptoms) may have a higher rate of false- negative unilateral temporal artery biopsy results ( 3,4). Therefore, the clinician who adopts this latter approach should be comfortable dealing with a wide spectrum of GCA, should be able to provide an adequate specimen of artery, and should have available access to a highly skilled pathology service for correct interpretation of the specimen. However, if unilateral biopsy alone is performed, the clinician must be prepared to accept the small risk of potentially missing a case of GCA. If the first biopsy J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 LOW DIAGNOSTIC YIELD WITH SECOND BIOPSIES IN GCA 215 result is negative and the index of suspicion is high, we would recommend that the steroids not be discontinued until the results of the second biopsy are known. Bilateral blindness because treatment was discontinued after one negative temporal artery biopsy result has been documented ( 10). The final option is to perform simultaneous bilateral biopsies. This has advantages over frozen section diagnosis because it is less expensive, more available, and less time consuming. Bilateral simultaneous biopsies have the advantage over bilateral sequential biopsies of not needing to return the patient to the operating room a second time. Performing bilateral simultaneous biopsies eliminates the small but finite possibility of failure to diagnose GCA with a unilateral biopsy alone. The disadvantages are those associated with having a temporal artery biopsy, except that the patient has two biopsies instead of one. Complications of temporal artery biopsy have been documented and include infection, hemorrhage, damage to the facial nerve, and stroke ( 11). Bilateral simultaneous biopsies also increase the surgical time. There is no documented case of visual loss from GCA within the fixed- section reports of bilateral negative temporal artery biopsies. Therefore, consideration of simultaneous bilateral temporal artery biopsies appears to be a safe and prudent approach for diagnosis of GCA. REFERENCES 1. Font C, Cid MC, Coil- Vincent B, et al. Clinical features in patients with permanent visual loss due to biopsy- proven giant cell arteritis. Br J Rheumatol 1997; 36: 251- 4. 2. Hall S, Lie JT, Kurland LT, et al. The therapeutic impact of temporal artery biopsy. Lancet 1983; 2: 1217- 20. " 3. Hall S, Hunder GG. Is temporal artery biopsy prudent? Mayo Clin Proc 1984; 59: 793- 5. 4. 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. 5. Hayrch SS, Podhajsky PA, Zimmerman G. Giant cell arteritis: validity and reliability of various diagnostic criteria. Am J Ophthalmol 1997; 123: 285- 96. 6. 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. 7. Klein RG, Campbell RJ, Hunder GG, et al. Skip lesions in temporal arteritis. Mayo Clin Proc 1976; 51: 504- 10. 8. Hedges TR III, Gieger GL, Albert DM. The clinical value of negative temporal artery biopsy specimens. Arch Ophthalmol 1983; 101: 1251- 4. 9. Hayreh SS, Podhajasky PA, Zimmerman B. Occult giant cell arteritis: ocular manifestations. Am J Ophthalmol 1988; 125: 521- 6. 10. Brownstein S, Nicolle DA, Codere F. Bilateral blindness in temporal arteritis with skip areas. Arch Ophthalmol 1983; 101: 388- 91. 11. Vollrath JC, Gloor B. Warum cine Dopplersonographic vor jeder Biopsie der A. temporaisl. Klin Monatsbl Augenheilkd 1989; 195: 169- 71. J Neuro- Ophthalmol, Vol. 20, No. 3, 2000 |