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Temporal Arteritis a Cough, Toothache, and Tongue Infarction

Temporal Arteritis a Cough, Toothache, and Tongue Infarction

GRAND ROUNDS AT THE JOHNS CLINICIAN’S CORNER HOPKINS BAYVIEW MEDICAL CENTER

Temporal A Cough, Toothache, and

David B. Hellmann, MD Temporal arteritis, the most common form of systemic in adults, CASE PRESENTATION is a panarteritis that chiefly involves the extracranial branches of the carotid DR HELLMANN: My patient, Professor R, who is a 79-year-old woman, was . The condition is illustrated in this article by the case of a 79-year-old well until she developed a disorder that woman with a dry cough, toothache, tongue infarction, and vision loss. The eventually produced blindness in her mean age of onset is 72 years and the disease rarely occurs in persons younger right eye. Although most of the com- than 50 years. The most common presenting manifestations are , mon presenting features of temporal ar- jaw , , and visual symptoms. Eighty- teritis (TA) are familiar to general in- nine percent of patients have an erythrocyte sedimentation rate greater than ternists and family physicians, the many 50 mm/h. However, about 40% of patients present with atypical manifes- disguises of this condition may chal- lenge the diagnostic skills of any expe- tations, including of unknown origin, respiratory tract symptoms (es- rienced physician. Learning to see pecially dry cough), and large artery involvement. Familiarity with such un- through these disguises is crucial to usual manifestations of temporal arteritis facilitates early diagnosis and early diagnosis and the prevention of treatment, thereby reducing the risk of vision loss. visual loss. Professor R, please tell us JAMA. 2002;287:2996-3000 www.jama.com how your illness began. PROFESSOR R: All my troubles be- PROFESSOR R: No, it was dry. I saw a and tongue , I could not eat very well gan the month my husband and I were physician who gave me some pills but the and lost 20 pounds during the week we moving from our home into a retire- cough persisted. Two months later I con- were away. When we returned from Ha- ment community. Before that, I was tinued to cough and felt exhausted. Since waii, my first visit was to the dentist. He completely healthy, playing tennis, it was winter, we thought that if we got found nothing wrong with my teeth. It swimming, and hiking. I am now a pro- away for a week to Hawaii I would get was actually an ophthalmologist who fessor emeritus and still perform re- better. The afternoon before we left for first suspected the correct diagnosis be- search on iconography of libraries in the Hawaii I awoke from a nap and could not cause of my blindness and other symp- 17th and 18th centuries. It keeps me see out of my right eye. It was all black. toms; the eye doctor tested my sedimen- interested, traveling, and visiting li- I could not reach a physician so I told tation rate and then immediately braries in Europe. My husband and I my husband, “Let’s just go. By the time admitted me to the hospital. have been doing all sorts of exciting we get back I’m sure I’ll be seeing fine DR HELLMANN: That is right, the things and I have never been in a hos- again.” And we went on our trip. erythrocyte sedimentation rate (ESR) pital except for a broken arm. The DR HELLMANN: Did your vision get was 115 mm/h. Results of the other tests month we sold our I was under better? performed at that time, including a a great deal of stress. Moving from a PROFESSOR R: No, it never did. complete count, serum chem- house into a 2-bedroom apartment, I DR HELLMANN: Did you experi- istries, and a , were had to give up a lot, especially my own ence a headache or any other symp- normal. She was treated with intrave- library of 2000 books. So, I was so tired toms, aside from the , cough, and that I began to take naps for the first Author Affiliation: Johns Hopkins University School visual loss? of Medicine, Department of Medicine, Johns Hop- time. I thought the tiredness would go PROFESSOR R: I never had a head- kins Bayview Medical Center, Baltimore, Md. away but after 2 to 3 weeks I also de- ache. But after I started coughing and be- Corresponding Author and Reprints: David B. Hell- mann, MD, Johns Hopkins University School of Medi- veloped a cough. fore I had the eye problem, I did de- cine, Department of Medicine, Johns Hopkins Bay- DR HELLMANN: Did you bring up velop a toothache. I could not quite view Medical Center, 4940 Eastern Ave, Baltimore, MD anything when you coughed? 21224 (e-mail: [email protected]). determine which tooth or teeth hurt. My Grand Rounds at The Johns Hopkins Medical Insti- mouth just hurt all over. Then I devel- tutions Section Editors: David B. Hellmann, MD, D. oped a burning sensation on the left side William Schlott, MD, Stephen D. Sisson, MD, The Johns See also Patient Page. Hopkins Hospital, Baltimore, Md; David S. Cooper, MD, of my tongue. Between my toothache Contributing Editor, JAMA.

2996 JAMA, June 12, 2002—Vol 287, No. 22 (Reprinted) ©2002 American Medical Association. All rights reserved. TEMPORAL ARTERITIS nous in high doses the eighth decade.1,3 The average age of Table. Classic Symptoms and Findings and underwent a right temporal artery onset of TA is 72 years. Perhaps as a re- in Temporal Arteritis* . The biopsy showed granulo- sult of the aging population in this coun- Frequency, % matous with multinucle- try, greater recognition of the disease on Symptoms ated giant cells, rupture of the internal the part of physicians, or both, the inci- Headache 77 51 elastic lamina, and luminal narrow- dence of TA has been rising in some Constitutional symptoms 48 ing. These findings were diagnostic of populations.1 Temporal arteritis has been Polymyalgia rheumatica 34 temporal arteritis. One day after the bi- reported in all groups, but appears es- Visual symptoms 29 Findings opsy, Professor R was discharged tak- pecially common in people of Scandi- Fever 26 ing , 60 mg/d. navian or Northern European heri- Abnormal temporal artery 53 1,2 Erythrocyte 94 I saw Professor R for the first time 1 tage. Certain genes (ie, HLA-DR4 sedimentation week later. Her vital signs were normal. haplotypes 0401 and 0404/8, which are rate Ͼ40 mm/h She had minimal light perception in the contained in the HLA-DRB1 locus) have *Data derived from Machado et al.5 right eye with a relative afferent pupil- been associated with an increased risk of lary defect. That is to say, her pupils con- developing TA.7 Although the cause of sense of discomfort in or around the jaw stricted less when I shone a light in the TA is unknown, the disease appears to that may be unrelated to chewing. One right eye than when I shone the light in be T-cell dependent and driven.8 of my other patients had a diffuse man- the healthy left eye. The right optic disc dibular discomfort that she attributed to was pale. The right temporal artery was Classic Manifestations her face-lift, even though that surgery had surgically absent while the left—barely The classic manifestations of TA are taken place months earlier and had pulsatile—was hard and stiff. An ische- headache, jaw claudication, polymyal- healed without difficulty. A sense of den- mic ulcer the size of a jellybean was pre- gia rheumatica (PMR), and visual symp- tal discomfort, as described by Profes- sent along the left lateral surface of the toms (TABLE).4-6,9 Headache is the most sor R, is another common variant of jaw tongue. The peripheral pulses were pal- common feature, occurring eventually in claudication. I have also seen patients pable and symmetrical, and there were more than 70% of patients. Although the with TA present with pain in the sinus no in the carotid, subclavian, ax- headache often causes a deep aching pain region or in the . Some were treated illary, abdominal aortic, or femoral ar- over the temporal area, the headache can with antibiotics for sinusitis or de- tery regions. be extremely variable in location, inten- spite the absence of any physical find- sity, and quality. Quite often, the only ings to support those diagnoses. Given DISCUSSION distinctive feature of the headache is that the variability in presentation of the head- Professor R’s presentation is instruc- it is new. Even if the patient has experi- aches and jaw claudication associated tive because it emphasizes some of the enced or tension for with TA, the diagnosis should be con- unusual ways in which TA can pre- years, he/she will note that this head- sidered whenever anyone older than 50 sent. When it presents atypically, as it ache is different. Alternatively, the pa- years complains of persistent unex- did in Professor R, it may not be diag- tient may say, “I am 72 and have never plained pain above the neck. nosed before the development of blind- had headaches until now.” Some pa- Polymyalgia rheumatica is defined as ness, the most feared of tients develop inflammation of the oc- pain and stiffness in the , neck, TA. Knowing the disguises that TA can cipital artery, causing pain at the base of and girdle areas and is worse in the wear affords physicians the best chance the . These symptoms are easily mis- morning and improves as the day goes of diagnosing and treating TA before the taken for cervical spine disease. on. Because of the pain, a patient may development of blindness. Jaw claudication is the occurrence of have trouble combing hair, putting on a pain in muscles of the face caused by pro- coat, or standing up from the toilet. It has Definition, Epidemiology, tracted chewing.9 This symptom re- been said that getting out of bed is to PMR and Pathogenesis sults from and is essentially an- what making a fist is to rheumatoid Temporal arteritis is the most common gina of the muscles of mastication. In . While most patients with PMR form of in adults. The contrast to temporal mandibular believe the pain is associated with weak- disease is defined as a panarteritis that disease, jaw claudication does not pro- ness, they always say that pain is the pre- preferentially involves the extracranial duce pain with the initiation of chew- dominant feature. (This contrasts with branches of the carotid artery.1-6 Aging ing or with the chewing of soft foods. and other forms of inflam- is the single greatest risk factor for the Rather, jaw claudication is induced by matory , in which weakness is disease: TA virtually never occurs be- the chewing of tougher foods such as the major complaint). Magnetic reso- fore the age of 50 years, and the annual meat. I emphasize, however, that only nance imaging studies have demon- incidence rises steadily thereafter, from about half of the patients with jaw pain strated clearly that the pain of PMR results 1.54 of 100000 people in the sixth de- from TA provide a classic description of from inflammation of bursae in the shoul- cade of life to 20.7 of 100000 people in claudication. Many report merely a vague der and hip regions, and to a lesser extent

©2002 American Medical Association. All rights reserved. (Reprinted) JAMA, June 12, 2002—Vol 287, No. 22 2997 TEMPORAL ARTERITIS

inflammatory responses may explain Box. Atypical Manifestations of Temporal Arteritis why some patients have a normal ESR Respiratory tract symptoms despite having active TA.13,14 Dry cough Sore throat Atypical Manifestations Tongue pain About 40% of patients with TA do not Choking sensation present with classic symptoms but rather with atypical manifestations Large artery involvement (BOX).3,15-19 The prominence of Profes- Upper and lower extremity claudication Thoracic or abdominal aortic sor R’s dry cough is noteworthy be- Peripheral nervous system features cause respiratory symptoms can be an Mononeuritis multiplex (especially of the brachial plexus) important manifestation of TA. The res- features piratory manifestations of TA were highlighted by a Mayo Clinic study Transient ischemic attacks showing that about 1 patient in 10 has Dementia respiratory symptoms.20 In 4%, these are Hallucinations the chief complaints.20 Dry cough, Syndrome of inappropriate antidiuretic hormone which our patient had, is the most com- Tumorlike lesions mon respiratory manifestation of TA. Breast mass The cause of the cough is not known. Ovarian mass Microangiopathic hemolytic Chest imaging results are normal in these patients. Because TA is a sys- temic disease, it is possible that cough centers, which are distributed through- by of the nearby .10 These gia. Some patients also present with ar- out the midbrain, the airways, the dia- findings help explain why patients most thritis that can be difficult to distin- phragm, and ,21 become ir- commonly localize the discomfort to the guish from .12 An ritated by inflammation in nearby blood tissues rather than to joints. abnormal temporal artery is found on vessels. The cough resolves quickly af- Visual symptoms, including loss of vi- physical examination in only half of the ter prednisone is started. sion and , develop in about one patients.4-6,9 Therefore, a normal tem- When should dry cough, a very com- third of patients (Table).4-6,9 Blindness is poral artery by physical examination mon symptom with many causes, be the most worrisome complication be- does not exclude this diagnosis. Abnor- considered a possible manifestation of cause it is usually irreversible. Blind- malities of the temporal artery include TA? First, because TA is so rare before ness usually results from an anterior is- thickening, enlargement, or the loss of 50 years, a younger person with cough chemic , which in turn pulsation. Professor R had a thickened, should not be suspected of having the is caused by occlusion of the posterior ropy temporal artery. Enlarged tempo- disease. Second, dry cough is never the ciliary artery, a branch of the ophthal- ral are not specific for TA; for sole symptom of patients with TA. mic artery. The posterior ciliary artery is example, extensive Paget disease of the Cough occurring in the absence of other the main source of blood flow to the op- skull can be accompanied by increased symptoms is not suggestive of TA. In- tic nerve head.11 Blindness is almost never blood flow to the skull and enlarged tem- deed, some have had classic manifesta- the first symptom of TA. Therefore, there poral arteries. tions, including headache and PMR, is almost always a window of opportu- The most common laboratory abnor- which happened to be less trouble- nity to make the diagnosis and prevent malities in TA are a markedly in- some to the patient than the cough. visual loss. When blindness occurs, it de- creased ESR (averaging nearly 100 mm/ Some patients may not have had clas- velops an average of 5 months after the h), a normochromic normocytic sic manifestations of TA but did have onset of TA. Unfortunately, our patient anemia, and (in approximately 30% of other symptoms. Professor R experi- developed this complication only 2 cases) a mildly elevated alkaline phos- enced , , fatigue, and months after the beginning of her symp- phatase.4-6,9 Although an elevated ESR tooth and tongue pain before she devel- toms. In patients with anterior ische- is usually a reliable companion of TA, oped blindness. Thus, the patient’s age mic optic neuropathy, no disc abnor- approximately 11% of patients pre- and the results of the review of symp- malities are detectable for the first few sent with an ESR of 50 mm/h or less and toms help identify the patients with hours. Subsequently, the disc swells and 4% have an ESR of 30 mm/h or less.13,14 cough who warrant further evaluation. becomes pale.11 Recent use of for an- Other respiratory tract symptoms of Other common manifestations of TA other condition (eg, ), local- TA include a sore throat (pharyngitis- include weight loss, fever, and arthral- ized arteritis, or inability to express fully like symptoms), tenderness of the ante-

2998 JAMA, June 12, 2002—Vol 287, No. 22 (Reprinted) ©2002 American Medical Association. All rights reserved. TEMPORAL ARTERITIS rior neck, tongue pain, hoarseness, and times more likely than age-matched cal features that substantially increased the sensation of choking.20 Tongue pain controls to develop thoracic aortic an- the likelihood of TA among these pa- results from ischemic damage that may eurysms.23 Although the can tients were jaw claudication (positive manifest as glossitis, ulceration of the complicate TA at any time, the mean likelihood ratio [LR], 4.2; 95% confi- tongue, lingual claudication (tongue pain time to recognition of the aneurysms dence interval [CI], 2.8-6.2), diplopia with talking), or lingual infarction. is 5 to 6 years after the diagnosis of TA. (positive LR, 3.4; 95% CI, 1.3-8.6), and Fever of unknown origin is another More recent studies using positron temporal artery beading (positive LR, important manifestation of TA.18 Tem- emission tomographic scans suggest 4.6; 95% CI, 1.1-18.4). A normal ESR poral arteritis accounts for only 2% of that as many as one half of all patients reduced the likelihood of TA (negative all of unknown origin, but 16% with TA develop inflammation of the LR, 0.2; 95% CI, 0.08-0.51).30 of those occurred in patients older than or its major branches.25 These attempts to make the diagno- 65 years.18 The fever in TA averages Although some patients with aortic in- sis of TA evidence-based powerfully un- 39.1oC and can reach nearly 40°C; about volvement by TA are asymptomatic, oth- derscore the importance of clinical two thirds of patients have rigors and ers develop aortic resulting in judgment. While TA affects less than drenching sweats, features that often aortic regurgitation or sudden death. One 1% of the population, 39% of the pa- conjure diagnoses of or lym- of my other patients illustrates this prob- tients referred for temporal biopsy had phoma.18 Of great help in distinguish- lem. Her disease had been quiescent for a positive result.30 It is striking to re- ing the fever of unknown origin of TA 5 years when she suddenly developed se- port how well physicians, analyzing all from those caused by infection or ma- vere . The initial evaluation was the data, identify cases of TA, espe- lignancy is that almost all patients with unrevealing and she was given a tenta- cially when considering the low pre- TA and fever of unknown origin have a tive diagnosis of a nephrocalcinosis. The dictive value of individual clinical vari- normal count, at least next day, the identity of her problem be- ables. A broad consideration of the before starting prednisone.18 came clear when she developed typical and atypical features of TA may Large artery involvement is more com- aortic regurgitation. Pathologic studies further improve clinical judgment. mon among patients with TA than gen- suggest that aneurysms can result from erally appreciated.22,23 One study of 238 smoldering inflammation or from weak- Establishing the Diagnosis patients with TA noted involvement of ening of the vessel wall from previous in- To be classified as having TA, a patient the carotid, vertebral, and subclavian ar- flammation.23 must meet 3 of the following 5 criteria teries in 14% of patients.22 Such involve- Other atypical manifestations of TA are established by the American College of ment may cause upper extremity clau- mass lesions of the breast or ovaries that : (1) 50 years or older, (2) dication, unequal blood pressures in the mimic tumors,26 the syndrome of inap- new-onset localized headache, (3) tem- arms, transient ischemic attacks, or ce- propriate antidiuretic hormone secre- poral artery tenderness as decreased tem- rebrovascular accidents. Lower extrem- tion, microangiopathic hemolytic ane- poral artery pulse, (4) ESR of 50 mm/h ity involvement that is sufficient to cause mia, , and central or higher, and (5) abnormal temporal ar- claudication is rare but has been re- nervous system symptoms (Box).15,16,27,28 tery biopsy findings demonstrating ported.24 To maximize the chance of de- Why patients with TA develop differ- mononuclear infiltration or - tecting large artery disease, the physi- ent manifestations is not entirely clear. tous inflammation.31 However, these clas- cal examination of patients suspected of However, evidence suggests that some sification criteria were never meant to having TA should include the measure- clinical subsets may involve unique serve as diagnostic criteria. Others have ment of in both arms, pathologic pathways that are caused by demonstrated the limitations of these cri- careful palpatation of the bracheal and differential expression of inflammatory teria, including having a positive predic- radial pulses, and auscultation for bruits .29 For example, interferon ␥, tive value of only 29%.32 not only above the carotid but also above elaborated by T cells, is increased in pa- In clinical practice, establishing the di- and below the clavicle for subclavian dis- tients with biopsy proven TA but not in agnosis of TA usually requires a biopsy ease and above the flexor surface of the patients who have PMR in the absence of the temporal artery. Because skip le- upper arm to detect axillary artery in- of vasculitis.8,29 sions are believed to occur in TA, diag- volvement. nosis is facilitated by obtaining large bi- Even the aorta is involved in a sub- Predicting the Presence of TA opsy specimens (Ͼ2 cm long) and by stantial number of patients with TA. A A recent literature review has tried to de- examining multiple pathologic sec- population-based study from Olmsted termine the accuracy of the history, the tions.2 The administration of cortico- County, Minnesota, revealed that 18% physical examination, and the ESR in the steroids given for less than 2 weeks does of patients with TA have aortic involve- diagnosis of TA.30 The analysis focused not reduce the yield of temporal artery ment, with the most common compli- on patients referred for TA biopsy and biopsy.33 Bilateral temporal artery biop- cation being thoracic aortic aneu- determined which features best pre- sies are not usually needed because they rysm.23 Indeed, patients with TA are 17 dicted a positive result. The only clini- are concordant in 95% to 99% of

©2002 American Medical Association. All rights reserved. (Reprinted) JAMA, June 12, 2002—Vol 287, No. 22 2999 TEMPORAL ARTERITIS cases.34-36 Patients with large artery in- trexate is a steroid-sparing agent in TA.42 20. Larson TS, Hall S, Hepper NG, Hunder GG. Res- piratory tract symptoms as a clue to arteri- volvement are diagnosed by arteriogra- In conclusion, TA is the most com- tis. Ann Intern Med. 1984;101:594-597. phy or magnetic resonance angiogra- mon systemic vasculitis in adults. Its 21. Irwin RS, Rosen MJ, Braman SS. Cough: a com- prehensive review. Arch Intern Med. 1977;137:1186- phy, either of which may demonstrate classic manifestations are headache, jaw 1191. long segments of smooth stenoses. Tem- claudication, PMR, and visual symp- 22. Klein RG, Hunder GG, Stanson AW, Sheps SG. poral artery biopsy is positive in only toms. However, TA can wear many dis- Larger artery involvement in giant cell (temporal) ar- teritis. Ann Intern Med. 1975;83:806-812. about half of patients who manifest large guises. The case presented empha- 23. Evans JM, O’Fallon M, Hunder GG. Increased inci- artery disease.37 Duplex ultrasonogra- sizes how dry cough, atypical tooth dence of and dissection in giant cell (tem- poral) arteritis. Ann Intern Med. 1995;122:502-507. phy has identified abnormalities in 93% pain, and tongue infarction can all serve 24. Le Hello C, Le´ vesque H, Jeanton M, et al. Lower of patients with TA but the technique as a warning that a patient may have TA limb and temporal arteritis. J Rheu- is operator-dependent and the sensitiv- and be at risk of losing vision. matol. 2001;28:1407-1412. 25. Blockmans D, Stroobants S, Maes A, Mortel- ity and specificity of temporal artery ul- mans L. Positron emission tomography in giant cell ar- Acknowledgment: I thank my patient for sharing her teritis and polymyalgia rheumatica. Am J Med. 2000; trasound outside of a few research cen- story, Roy Zieglestein, MD, and John Stone, MD, for 108:246-249. 38,39 reading and improving the manuscript, and Tambra ters are unknown. It is also too early 26. Kariv R, Sidi Y, Gur H. Systemic vasculitis pre- Noethen for assisting in the word-processing. to tell whether the abnormalities seen on senting as a tumorlike lesion. Medicine. 2000;79:349- positron emission tomographic scan- 359. REFERENCES 27. Caselli RJ, Hunder GG. Neurologic complica- ning will be useful diagnostically. There- tions of giant cell (temporal) arteritis. Semin Neurol. 1. Gonza´ lez-Gay MA, Garcia-Porrua C, Rivas MJ, et 1994;14:349-353. fore, unless the patient has large artery al. Epidemiology of biopsy proven giant cell arteritis 28. Caselli RJ, Hunder GG, Whisnant JP. Neurologic in northwestern Spain. Ann Rheum Dis. 2001;60:367- disease, which is best diagnosed by an- disease in biopsy-proven giant cell (temporal) arteri- 371. tis. Neurology. 1988;38:352-359. giography, all patients suspected of hav- 2. Hunder GG. Giant cell arteritis and polymyalgia 29. Weyand CM, Tetzlaff N, Bjornsson J, et al. Dis- rheumatica. Med Clin North Am. 1997;81:195-219. ing TA should undergo temporal ar- ease patterns and tissue profiles in giant cell 3. Levine SM, Hellmann DB. Giant cell arteritis. Curr arteritis. Arthritis Rheum. 1997;40:19-26. tery biopsy. Opin Rheumatol. 2002;14:3-10. 30. Smetana GW, Shmerling RH. Does this patient 4. Goodman BW Jr. Temporal arteritis. Am J Med. have temporal arteritis? JAMA. 2002;287:92-101. 1979;67:839-852. Approach to Treatment 31. Hunder GG, Bloch DA, Michel BA, et al. The 5. Machado EBV, Michet CJ, Ballard DJ, et al. Trends American College of Rheumatology 1990 criteria for Because the central goal in managing in incidence and clinical presentation of temporal ar- the classification of giant cell arteritis. Arthritis Rheum. TA is to prevent blindness, treatment teritis in Olmsted County, Minnesota, 1950-1985. Ar- thritis Rheum. 1988;31:745-749. 1990;33:1122-1128. should be initiated immediately when- 6. Huston KA, Hunder GG. Giant cell (cranial) arte- 32. Rao JK, Allen NB, Pincus T. Limitations of the 1990 ever the disease is strongly suspected. ritis: a clinical review. Am Heart J. 1980;100:99-105. American College of Rheumatology classification cri- 7. Weyand CM, Hicok KC, Hunder GG, Goronzy JJ. teria in the diagnosis of vasculitis. Ann Intern Med. Most authorities believe that TA re- The HLA-DRB1 locus as a genetic component in gi- 1998;129:345-352. quires starting prednisone in the range ant cell arteritis. J Clin Invest. 1992;90:2355-2361. 33. Achkar AA, Lie JT, Hunder GG, et al. How does previous treatment affect the biopsy find- 2 8. Weyand CM, Goronzy JJ. Arterial wall injury in gi- of 60 mg/d. In contrast, isolated PMR, ant cell arteritis. Arthritis Rheum. 1999;42:844-853. ings in giant cell (temporal) arteritis? Ann Intern Med. which is 3 times more common than 9. Huston KA, Hunder GG, Lie JT, et al. Temporal ar- 1994;120:987-992. TA, requires only 10 to 20 mg/d of pred- teritis: a 25-year epidemiologic, clinical, and patho- 34. Danesh-Meyer HV, Savino PJ, Eagle RC Jr, et al. logic study. Ann Intern Med. 1978;88:162-167. Low diagnostic yield with second in sus- nisone at the start of therapy. The rate 10. Cantini F, Salvarani C, Olivieri I, et al. Inflamed pected giant cell arteritis. J Neuroophthalmol. 2000; of tapering prednisone is chiefly deter- structures in polymyalgia rheumatica with nor- 20:213-215. mal erythrocyte sedimentation rate. Arthritis Rheum. 35. Pless M, Rizzo JF 3rd, Lamkin JC, Lessell S. Con- mined by the patient’s symptoms. 2001;44:1155-1159. cordance of bilateral temporal artery biopsy in giant Although prednisone is the corner- 11. Miller NR. Visual manifestations of temporal ar- cell arteritis. J Neuroophthalmol. 2000;20:216-218. teritis. In: Stone JH, Hellmann DB, eds. Rheumatic Dis- 36. Boyev LR, Miller NR, Green WR. Efficacy of uni- stone of therapy, 60% of patients re- ease Clinics of North America: Vasculitis. In press. lateral versus bilateral temporal artery biopsies for the lapse during or after prednisone tapers. 12. Navaez J, Nolla-Sole JM, Navaez JA, et al. Mus- diagnosis of giant cell arteritis. Am J Ophthalmol. 1999; In addition, the long duration of pred- culoskeletal manifestations in polymyalgia rheu- 128:211-215. matica and temporal arteritis. Ann Rheum Dis. 2001; 37. Brack A, Martinez-Taboada V, Stanson A, et al. nisone therapy required to treat TA (of- 60:1060-1063. Disease pattern in cranial and large-vessel giant cell ten Ͼ2 years) and the high rate of pred- 13. Salvarani C, Hunder GG. Giant cell arteritis with arteritis. Arthritis Rheum. 1999;42:311-317. low erythrocyte sedimentation rate. Arthritis Care Res. 38. Schmidt WA, Kraft HE, Vorpahl K, et al. Color du- nisone adverse effects (weight gain, 2001;45:140-145. plex ultrasonography in the diagnosis of temporal ar- , , and compres- 14. Wong RL, Korn JH. Temporal arteritis without an teritis. N Engl J Med. 1997;337:1336-1342. elevated erythrocyte sedimentation rate. Am J Med. 39. Schmidt WA. Doppler ultrasonography in the di- sion fractures) have prompted searches 1986;80:959-964. agnosis of giant cell arteritis. Clin Exp Rheumatol. 2000; for steroid-sparing agents.40 A Spanish 15. Healey LA, Wilske KR. Presentation of occult gi- 18:S40-S42. ant cell arteritis. Arthritis Rheum. 1980;23:641-643. 40. Gabriel SE, Sunku J, Salvarani C, et al. Adverse group conducted a double-masked, pla- 16. Hellmann DB. Occult manifestations of giant cell outcomes of anti-inflammatory therapy among pa- cebo-controlled trial showing that the arteritis. Med Rounds. 1989;2:296-301. tients with polymyalgia rheumatica. Arthritis Rheum. combination of prednisone and metho- 17. Sonnenblick M, Nesher G, Rosin A. Nonclassical 1997;40:1873-1878. involvement in temporal arteritis. Semin Ar- 41. Jover JA, Herna´ ndez-Garcia C, Morado IC, et al. trexate reduced the need for predni- thritis Rheum. 1989;19:183-190. Combined treatment of giant-cell arteritis with metho- sone slightly.41 Unfortunately, the mod- 18. Calamia KT, Hunder GG. Giant cell arteritis (tem- trexate and prednisone. Ann Intern Med. 2001;134: poral arteritis) presenting as fever of undetermined ori- 106-114. est reduction in prednisone did not gin. Arthritis Rheum. 1981;24:1414-1418. 42. Spiera RF, Mitnick HJ, Kupersmith M, et al. A pro- achieve detectable reductions in pred- 19. Walsh SJ, McClelland AJ, Owens CG, Callender spective, double-blind, randomized, placebo con- 41 ME. Fever and dry cough in a patient with a pros- trolled trial of in the treatment of giant nisone-related adverse effects. Other in- thetic heart valve. Rheumatology. 2001;40:714- cell arteritis (GCA). Clin Exp Rheumatol. 2001;19: vestigators have not found that metho- 715. 495-501.

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