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Prognosis of polymyalgia rheumatica and giant cell arteritis ALAN
B. M Y L E S
Polymyalgia rheumatica and giant cell arteritis are so closely related that it might be thought that the prognosis would be similar, and this is probably correct. There is quite good evidence concerning the prognosis of giant cell arteritis but very much less for polymyalgia rheumatica; they will be considered individually, while realizing that there may be features of both conditions in many individuals and that some starting with one condition may develop features of the other. There are differences, however, partly related to the use of different classifications of the two conditions.
PROGNOSIS OF GIANT CELL ARTERITIS
Mortality Although commonly presenting in the temporal or cranial arteries, giant cell arteritis affects other arteries widely and it might be supposed that this involvement would often have serious consequences, but survival statistics have usually shown that patients with giant cell arteritis have the same expectation for life as the general population (Hauser et al, 1971; Huston et al, 1978; Andersson et al, 1986). Nordberg and Bengtsson (1989), however, followed up 284 patients diagnosed histologically as having giant cell arteritis. The mortality over a 10-year period did not differ from the general population but death from vascular disease in the first year was considerably higher than expected, 17 dying within the first 4 months (eight from cerebral vascular disease and the others from myocardial infarction and other disease of major blood vessels). They suggested that although all the patients had ieceived corticosteroid treatment, the dose had been inadequate in 13 of them and that this was a contributory factor. Save-Soderbergh (1986) described nine patients who had died as a result of giant cell arteritis (five with cerebral involvement, two from myocardial infarction and two from aortic dissection) and also suggested that inadequate corticosteroid treatment contributed to the deaths. Bailli~re's ClinicalRheumatology-Vol. 5, No. 3, December 1991 ISBN 0-7020--1537-7
493 Copyright 9 1991, by Bailli~re Tindall All rights of reproduction in any form reserved
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Neurological involvement A wide range of neurological abnormalities have been described, mainly resulting from involvement of the cranial arteries and nerves (Mehler and Rabinowich, 1988; Reich et al, 1990), many having a good prognosis. Major vessel involvement usually affects the vertebral arteries and is seldom diagnosed during life (Wilkinson and Russell, 1972). Caselli (1990) described three patients with multi-infarct dementia attributable to giant cell arteritis who responded well to treatment with corticosteroids.
Large artery involvement Even in those patients who are known to have giant cell arteritis, it is difficult to be certain whether major vessel involvement is a result of giant cell arteritis or of atherosclerotic disease, common in this age group, which explains the disparity of some of the papers (Sonnenblick et al, 1989). Thus Russell (1959) regarded giant cell arteritis as a serious disease, seven of 28 patients developing severe systemic complications, whereas Mosher (1959) followed 32 patients, 11 of whom died but in none of whom giant cell arteritis was thought to be relevant to the death. Graham et al (1981) investigated 32 deaths in 58 patients; eight died early and, in three, giant cell arteritis was the probable cause of death. Large artery involvement, other than affecting the cranial arteries, is described in 34 of 248 patients by Klein et al (1975), who considered that the prognosis in adequately treated patients was good, although three of their patients died of complications related to giant cell aortitis. Bengtsson and Malmvall (1981) found the aortic arch syndrome in six patients in a group of 90 but it did not seem to affect the mortality.
Ocular complications The first clear description of temporal arteritis was made by Horton et al (1932), but they did not recognize the serious ocular effects, which became apparent in the next two decades (Cooke et al, 1945). Crosby and Wadsworth (1948) found that three of 25 patients with temporal arteritis became blind and it became apparent that this was the most serious consequence of temporal arteritis. Birkhead et al (1957), reviewing their own cases and the literature up to date, concluded that blindness occurred in 22% of patients with temporal arteritis and about half of these developed blindness in the other eye, usually within a few weeks. A lower incidence of blindness at presentation (7%) has subsequently been described by Bengtsson and Malmvall (1982), which doubtless represents earlier diagnosis of giant cell arteritis and recognition of the complications, and earlier corticosteroid treatment. The earlier figures are unlikely to be accurate and may have underestimated the real incidence in the past. Sudden loss of vision, which would now be recognized as resulting from giant cell arteritis, was at that time considered to be ischaemic due to optic neuritis and would have been classified differently if there were no obvious evidence of temporal arteritis.
VRO6NOSm
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It subsequently became apparent, as a result of estimating the ESR and, if raised, doing a temporal artery biopsy, that many of these patients did have temporal arteritis (Cullen, 1967). Beri et al (1987) found that 19 out of 50 patients with anterior ischaemic optic neuropathy attributed to giant cell arteritis had evidence of involvement of both eyes at presentation. Loss of vision resulting from ocular involvement by giant cell arteritis is usually permanent. Treatment is given to suppress the disease and avoid further blindness, particularly in the other eye, which usually occurs early in the course of the disease and seldom after 6 months from the first symptoms. The degree of recovery of vision depends upon the amount of vision that is lost. If it is considerable, there is usually no significant return of vision but, if it is partial, some recovery of vision has often been described and attributed to prompt corticosteroid treatment (Whitfield et al, 1953; Schneider et al, 1971; Cullen and Coleiro, 1976). Profound visual loss is almost always permanent, although there are a small number of reports of return of vision in patients given intravenous steroids within a short time after onset. Enthusiastic treatment of this nature has doubtless been used quite often but the failures are not reported (Clearkin and Watts, 1990). The main object of treatment is to protect the uninvolved eye, and earlier reports suggested that this was likely to occur within a few weeks, some patients presenting with blindness in both eyes. This tendency ceased abruptly as soon as corticosteroid treatment was used and this protective effect has been apparent in all subsequent studies.
Prevention of further visual loss Relatively high corticosteroid doses, i.e. cortisone 300 mg daily, were used in the earlier studies and doses of this nature have been used commonly. It is frequently stated that these high doses are needed to provide this degree of protection but there have been no long-term controlled studies designed to elucidate this matter. Lower doses have been used by Myles (1975), Behn et al (1983), Delecoeuillerie et al (1988), Lundberg and Hedfors (1990) and Ridley et al (1991) without any disadvantage. It remains, however, the practice of most clinicians, particularly ophthalmologists, to use high starting doses of prednisolone, such as 80-120 mg daily in the belief that this is necessary to prevent blindness (Beri et al, 1987). Table 1 shows the reports in the world literature of blindness occurring in giant cell arteritis after corticosteroid treatment has been started, indicating the starting dose, the dose at the time of the onset of blindness, and the duration after starting treatment. It will be seen that the numbers are small, 19 in all, over a period of nearly 30 years. In most cases high starting doses have been used; the blindness has occurred within the first 4 weeks in nine of the cases, although the timing was not stated in another five. It is clear that the high doses, when used, did not always prevent blindness but have done so in a very high proportion. It is possible that the prognosis may be equally as good with the lower starting doses, although they do not always provide the same degree of symptomatic relief from other symptoms of giant cell arteritis (Kyle and Hazleman, 1989a).
496
A.B. MYL~S Table 1. Occurrence of blindness during cortieosteroid treatment (individual cases).
Authors
Year
Prednisolone: dose at time (rag)
Prednisolone: starting dose (mg)
Whitfield et al Cullen Sorensen and Lorenzen Sorensen and Lorenzen Healy and Wilske Huston et al Hugod and Schebel Calamia and Hunder Herrick Herrick Jones and Hazleman Jones and Hazleman Bengtsson and Malmvall Bengtsson and Malmvall Beri et al Delecoeuillerie et al Delecoeuillerie et al Faarvang and PontoppidanThyssen Kyle and Hazleman
1963 1972 1977 1977 1977 1976 1979 1980 1980 1980 1981 1981 1982 1982 1987 1988 1988 1989
Adequate 5 2.5 1.5 10 25 60 60 60 60 10 8 20 30 80-120 >60 60 10
Not stated Not stated 30-60 30-60 40 60 60 50 60 60 Not stated Not stated 20 30 80-120 >60 30 80
1989a
40
40
Duration from starting Not stated 7 years Not stated Not stated 2 months 7 days 5 days 4 weeks 1 day 4 days 1 year 8 months 2 days 5 days 2 days Not stated Not stated 2 months 1 week
Clinicians are u n d e r s t a n d a b l y worried that stopping steroids too soon m a y allow giant cell arteritis to b e c o m e active again and blindness to occur, and for this reason p r o l o n g e d corticosteroid t r e a t m e n t or p r o l o n g e d followup are s o m e t i m e s suggested. T h e r e is, h o w e v e r , little evidence f r o m the literature that this is a significant danger. Blindness occurring m o r e than one year after corticosteroid t r e a t m e n t was started was only described in one patient (Cullen, 1972) and, f u r t h e r m o r e , this patient had continued to receive 5 m g p r e d n i s o l o n e daily as m a i n t e n a n c e t r e a t m e n t , p r e s u m a b l y to prevent this possible risk (Kyle and H a z l e m a n , 1990). Thus the prognosis for small vessel arteritis, as in the eye, is very g o o d after corticosteroid t r e a t m e n t has been started. It is also g o o d for large vessel involvement, but there is less information a b o u t this and it is occasionally found late in the course of the disease, in spite of apparently a d e q u a t e treatment. T h e histological changes are t h o u g h t to resolve quite rapidly; for this reason t e m p o r a l artery biopsy is r e c o m m e n d e d within a week of starting treatment (Allison and Gallagher, 1984), but changes m a y persist and Fauchald et al (1972) s h o w e d evidence of active arteritis m a n y years after presentation of the disease.
PROGNOSIS OF POLYMYALGIA RHEUMATICA A l t h o u g h Bruce, in 1888, is usually accepted as the first p e r s o n to have described polymyalgia rheumatica, it was not until 1957 that B a r b e r pro-
PROGNOSIS
497
vided the name, and it was not widely accepted for a further 10 years or so. As recently as 1966 an American textbook stated: 'thus the proponents of a syndrome of polymyalgia rheumatica may have selected those early patients with fibrositis who happened to have a high sedimentation rate, arbitrarily given their illness a new pseudo specific name and exposed this high risk group to unnecessary and unwise steroid therapy' (Smyth, 1966). In the light of these difficulties in acceptance of the diagnosis, it is not surprising that there were few clear accounts of the natural history of this condition. Its recognition came with the realization that corticosteroids in quite small amounts produced almost complete symptomatic relief, and most patients are treated in this way. Almost all studies, therefore, of the duration of polymyalgia rheumatica are studies of the length of time for which corticosteroid treatment has been necessary to provide adequate symptomatic relief and to protect from the possible complications of the disease. The results of these studies have varied considerably but in a fairly consistent manner. The later studies have shown a longer duration of treatment, and relapses occurred in those who had been treated for an inadequate length of time in the earlier studies. Originally thought to be a benign condition, which was self-limiting and recovered in the course of a few years, the reported outcome has subsequently varied considerably. Bagratuni (1963), describing the anarthritic rheumatoid syndrome which was subsequently to be called polymyalgia rheumatica, found a duration of about 7 years before corticosteroid treatment was used. Since corticosteroid treatment has been available and stopping the treatment has been considered to be the end-point of the disease, the reported duration has generally increased with the passage of time. Von Knorring (1979) reported a mean time to withdrawal of 3 years, but there were 20 subsequent relapses. Coomes et al (1976) found that treatment for 5 years was necessary in 84% of their patients. Behn et al (1983) found that only 72 of 176 patients had stopped steroids at a mean of 31 months, but 30 subsequently relapsed. Similar findings were reported by Bengtsson and Malmvall (1981) and Ayoub et al (1985). Delecoeuillerie et al (1988) reported that only 65 of 132 patients had been able to stop steroids after a mean of 25 months. Kyle and Hazleman (1988) found that a quarter were able to stop after 2 years. These papers are all from Europe. In the United States, however, Chuan et al (1982) reported a mean duration of treatment of 11 months, and in other studies most patients had stopped treatment within 2 years (Huston et al, 1978; Huston and Hunder, 1980). Using lower than usual steroid doses, Lundberg and Hedfors (1990) reported a mean duration of treatment of 17 months and concluded that corticosteroid treatment could be stopped in most cases after 2 years. The disparity concerning the length of treatment is not easy to explain. There is no suggestion that the severity of the disease differed in these series or that corticosteroid regimens make any difference to the ultimate length of treatment. It is possible that clinical practice differs considerably in these countries; complications of the disease at the late stages are unusual, and the need for continuing treatment is usually the clinical judgement of the need to
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provide corticosteroids to relieve symptoms, rather than being made on the basis of any objective test. Unfortunately, such tests do not exist but possibly may do so in the future. Dasgupta et al (1989) found a selective reduction of CD8+ suppressor cytotoxic cells in polymyalgia rheumatica and giant cell arteritis, which persisted for about 2 years and was not related to symptomatic control of the disease. Such tests may, in due course, be able to give an indication of when corticosteroid withdrawal could be considered. Non-steroidal anti-inflammatory drugs have been suggested for treatment of uncomplicated polymyalgia rheumatica (Chuan et al, 1982). The literature is not clear on this matter but it is possible that they are used for symptomatic suppression in the late stages of polymyalgia rheumatica at a stage when continued low dose corticosteroid treatment would be recommended in most European countries. There is no adequate evidence about this practice and it is by no means certain that non-steroidal antiinflammatory analgesics are less likely to cause side-effects. Other disease associations
There do not seem to be any important consequences of polymyalgia rheumatica, other than the possibility of developing giant cell arteritis and its complications. There is some evidence to suggest that these conditions are autoimmune diseases, and overlap with other conditions might have been expected. There has been shown to be an increased risk of developing hypothyroidism but there is no evidence to suggest that there is an increased risk of any other autoimmune disease (Dent and Edwards, 1978; Bowness et al, 1991). FACTORS WHICH MAY INFLUENCE PROGNOSIS Unfortunately, there does not seem to be any method of assessing the prognosis in an individual patient. Complications of giant cell arteritis tend to occur early in the disease and seem to be more common in men than women. The main complication of polymyalgia rheumatica is the development of giant cell arteritis, which may occur at any time but often late in the course of the disease at a time when the steroid dose is being reduced. Factors such as the height of the ESR at onset (Ellis and Ralston, 1983) or the presence and degree of histological evidence of giant cell arteritis do not affect the ultimate prognosis (Bevan et al, 1968; Hall et al, 1983; Vilaseca, 1987). Some patients with polymyalgia rheumatica and negative biopsies have developed giant cell arteritis in spite of corticosteroid treatment (Jones and Hazleman, 1981). Delecoeuillerie et al (1988) found a very significant increase in complications of temporal arteritis in males compared with females, which has not previously been reported. It was originally suggested that Negroes had a low instance of polymyalgia rheumatica and giant cell arteritis, but both have been found (Gonzalez et al, 1989), and it was suggested by Love et al (1986) that blindness occurred more commonly in Negroes affected by this con-
PROGNOSIS
499
dition. Friedman and Friedman (1988) noted that non-western Jews seem to have a worse prognosis with regard to recurrence of giant cell arteritis than western Jews. PROGNOSIS WITH REGARD TO CORTICOSTEROID TREATMENT
It has often been assumed that the relatively low corticosteroid doses used in these conditions would not result in an important incidence of corticosteroid side-effects, and it has even been suggested that these patients were in some way resistant to the side-effects. Unfortunately, this has not proved to be correct. Rubinow et al (1984) described serious corticosteroid complications in 11 patients receiving a relatively high maintenance dose of prednisone, averaging 26.3 mg daily, which included fractures in six, three with steroid myopathy, two with psychosis, two with diabetes and three with severe infection; four of these patients died. Another group with similar severity of disease received an average maintenance dose of 13 mg daily and did not have any severe complications. Ayoub et al (1985) described significant corticosteroid related side-effects in 17 of 76 patients, although there were no deaths. Kyle and Hazleman (1989b) undertook a specific study of the relationship between steroid dosage and steroid side-effects. They found that significant side-effects were present in 36%; this figure rose to 76% if weight gain was included. The side-effects were significantly more common in those starting with an initially high dose of prednisolone of more than 30mg daily, and in those who had a high cumulative total dose. Using somewhat lower starting doses, Behn et al (1983) reported a lower incidence of corticosteroid side-effects at 16%. The most important expected corticosteroid side-effect of long-term treatment is osteoporosis, but Andersson et al (1990), studying radiological osteoporosis and the mineral content of heel bone in patients with giant cell arteritis treated with prednisolone for 5 years on average doses, did not show an increased incidence of osteoporosis over a control group. There are now many contradictory papers concerning effects of low dose corticosteroid treatment on the skeleton, and the contradictions have not yet been satisfactorily answered. Possibly newer radiological techniques will solve this problem. Corticosteroid related side-effects have usually been found where they have been looked for, and may become a greater source of morbidity and mortality than the disease for which the treatment was originally given. INTERCHANGE BETWEEN POLYMYALGIA RHEUMATICA AND GIANT CELL ARTERITIS It is clear that some patients with giant cell arteritis develop polymyalgia rheumatica and it is usually accepted that patients with polymyalgia rheumatica are at risk of developing giant cell arteritis, which is one of the
500
a.U. MYLES
reasons for using corticosteroid treatment, most clinicians using a higher dose in those thought to have giant cell arteritis. In my series of 367 patients with polymyalgia rheumatica and giant cell arteritis, 15 of those with giant cell arteritis only at presentation developed polymyatgia rheumatica subsequently, and seven patients with apparently uncomplicated polymyalgia at onset developed giant cell arteritis (unpublished). However, Sorensen and Lorenzen (1977), studying 63 patients with polymyalgia rheumatica and giant cell arteritis, considered that there was no justification for making a distinction between the two diseases with regard to treatment. Delecoeuillerie et al (1988) examined retrospectively 210 patients with polymyalgia or giant cell arteritis; 132 patients were considered to have 'pure' polymyalgia rheumatica requiring low dose corticosteroid treatment, and it was thought that this distinction could be made at the original presentation, regardless of biopsy findings. Chuan et al, however, in 1982, also concluded that there was a group of patients with polymyalgic symptoms but without evidence of giant cell arteritis, judged by extensive temporal artery biopsy, and concluded that these patients could be treated with non-steroidal anti-inflammatory drugs or low doses of corticosteroids. Most clinicians, however, have patients with initially 'pure' temporal arteritis who subsequently develop polymyalgic symptoms, often while the corticosteroid dose is being withdrawn, and patients with polymyalgia rheumatica may develop features of giant cell arteritis (Hamilton et al, 1971). Jones and Hazleman (1981) did not find that polymyalgia rheumatica was a benign disease, finding some evidence of giant cell arteritis in 55% and that neurological or visual complications occurred in 26%. There is therefore some disparity concerning the prognosis of polymyalgia rheumatica, which is probably more apparent than real: those favouring the benign prognosis for 'pure' polymyalgia rheumatica excluding patients with any features of giant cell arteritis from their studies. It is clear, however, that patients with features of both conditions tend to have the worst prognosis with regard to duration of treatment and complications of the disease (Delecoeuillerie et al, 1988; Kyle and Hazleman, 1988; Lundberg and Hedfors, 1990). SUMMARY
Polymyalgia rheumatica and giant cell arteritis are amongst the most satisfying conditions for clinicians to diagnose and treat because the unpleasant effects and serious consequences of these conditions can be almost entirely prevented by corticosteroid treatment; the fact that the side-effects of this treatment sometimes seem to be more serious than the complications of the disease is an indication of its effectiveness. Unfortunately, there is no objective way of determining the prognosis in the individual, and decisions concerning duration of treatment remain empirical. REFERENCES
Allison MC & Gallagher PJ (1984) Temporal artery biopsy and corticosteroid treatment. Annals of the Rheumatic Diseases 43: 416-417.
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Andersson R, Malmvall BO & Bengtsson BA (1986) Long term survival in giant cell arteritis. Acta Medica Scandinavica 220: 361-364. Andersson R, Rundgren A, Rosengren K, Bengtsson BA, Malmvall BE & Mellstrom D (1990) Osteoporosis after long-term corticosteroid treatment of giant cell arteritis. Journal of Internal Medicine 272: 391-395. Ayoub WT, Franklin CM & Torretti D (1985) Polymyalgia rheumatica-~turation of therapy and long term outcome. American Journal of Medicine 79: 309-315. Bagratuni L (1963) Prognosis in the anarthritic rheumatoid syndrome. British Medical Journal 1: 513--518. Barber HS (1957) Myalgic syndrome with constitutional effects. Polymyalgia Rheumatica. Annals of the Rheumatic Diseases 16: 230-237. Behn AR, Perera T & Myles AB (1983) Polymyalgia rheumatica and corticosteroids: how much for how long? Annals of the Rheumatic Diseases 42: 374-378. Bengtsson BA & Malmvall BO (1981) Prognosis of giant cell arteritis including temporal arteritis and polymyalgia rheumatica. Acta Medica Scandinavica 209: 337-345. Bengtsson BA & Malmvall BO (1982) Eye complications in giant cell arteritis. Acta Medica Scandinavica, Supplement 658: 38-43. Beri M, Klugman MR, Kohler J & Hayreh SS (1987) Anterior ischaemic optic neuropathy. Ophthalmology 94: 1020-1028. Bevan AT, Dunnill MS & Harrison MJG (1968) Clinical and biopsy findings in temporal arteritis. Annals of the Rheumatic Diseases 27: 271-277. Birkhead NC, Wagener HP & Shick RM (1957) Treatment of temporal arteritis with adrenal corticosteroids. Journal of the American Medical Association 163: 821-827. Bowness P, Shotliff K, Middlemiss A & Myles AB (1991) Prevalence of hypothyroidism in patients with polymyalgia rheumatica and giant cell arteritis. British Journal of Rheumatology 30: 349-351. Bruce W (1888) Senile rheumatic gout. British Medical Journal li: 811-813. Calamia KT & Hunder GG (1980) Manifestations of giant cell (temporal) arteritis. Clinics in Rhei~matic Diseases 6: 389-403. Caselli RJ (1990) Giant cell (temporal) arteritis: a treatable cause of multi-infarct dementia. Neurology 40: 753-755. Chuan TY, Hunder GG, Ilstrup DM & Kurland LT (1982) Polymyalgia rheumatica--a 10-year epidemiologic and clinical study. Annals oflnternal Medicine 97: 672-680. Clearkin LG & Watts MT (1990) Ocular involvement in giant cell arteritis. British Journal of Hospital Medicine 43: 373-376. Cooke WT, Cloake PCP, Govan ADT & Colbeck JC (1945) Temporal arteritis: a generalised vascular disease. Quarterly Journal of Medicine 57: 47-75. Coomes EN, Ellis RM & Kay AG (1976) A prospective study of 102 patients with the polymyalgia rheumatica syndrome. Rheumatology and Rehabilitation 15: 270-276. Crosby RC & Wadsworth R (1948) Temporal arteritis. Archives of Internal Medicine 81: 431-464. Cullen JF (1967) Occult temporal arteritis. British Journal of Ophthalmology 51: 513-525. Cullen J (1972) Temporal arteritis. British Journal of Ophthalmology 56: 584-588. Cullen JF & Coleiro JA (1976) Ophthalmic complications of giant cell arteritis. Survey of Ophthalmology 20: 247-259. Dasgupta B, Duke O, Timms AM, Pitzalis C & Panayi GS (1989) Selective depletion and activation of CD8+ lymphocytes from peripheral blood of patients with polymyalgia rheumatica and giant cell arteritis. Annals of the Rheumatic Diseases" 48: 30%311. Delecoeuitlerie G, Joly P, Cohen De Lara A & Paolaggi JB (1988) Polymyalgia rheumatica and giant cell arteritis: a retrospective analysis of prognostic features and different corticosteroid regimens (11 year survey of 210 patients). Annals of the Rheumatic Diseases 47: 733-739. Dent RG & Edwards OM (1978) Autoimmune thyroid disease and the polymyalgia rheumatica-giant cell arteritis syndrome. Clinical Endocrinology 9: 215-219. Ellis ME & Ralston S (1983) The ESR in the diagnosis and management of the polymyalgia rheumatica/giant cell arteritis syndrome. Annals of the Rheumatic Diseases 42: 168170. Faarvang KL & Pontoppidan-Thyssen E (1989) Giant cell arteritis. Journal of Internal Medicine 225: 215-216.
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Fauchald P, Rygvold O & Oystese B (1972) Temporal arteritis and polymyalgia rheumatica: clinical and biopsy findings. Annals oflnternal Medicine 77: 845-852. Friedman G & Friedman B (1988) Prolonged corticosteroid treatment in the management of temporal arteritis. Klinische Wochenschrift 66: 1167-1170. Gonzalez EB, Varner WT, Lisse JR, Daniels JC & Hokanson JA (1989) Giant cell arteritis in the southern United States. Archives of Internal Medicine 149: 1561-1565. Graham E, Holland A, Avery A & Russell RW (1981) Prognosis in giant cell arteritis. British Medical Journal 282: 269-271. Hall S, Lie JT, Kurland LT, Persellin S, O'Brien PC & Hunder G G (1983) The therapeutic impact of temporal artery biopsy. Lancet ih 1217-1220. Hamilton CR, Shelley WM & Tumulty PA (1971) Giant cell arteritis. Medicine 50: 1-27. Hauser WA, Ferguson RH, Holley KE & Kurland LT (1971) Temporal arteritis in Rochester, Minnesota. Mayo Clinic Proceedings 46: 597-602. Healy LA & Wilske KR (1977) Manifestations of giant cell arteritis. Medical Clinics of North America 61: 261-269. Herrick PC (1980) Cranial arteritis. A preventable cause of blindness. Transactions of the Ophthalmological Society of New Zealand 32: 106-109. Horton BT, Magath TB & Brown GE (1932) Undescribed form of arteritis of temporal vessels. Mayo Clinic Proceedings 7: 700-701. Hugod C & Schebel M (1979) Temporal arteritis. Acta Medica Scandinavica 205: 445-446. Huston KA, Hunder GG, Lie JT, Kennedy R H & Elveback LR (1978) Temporal arteritis. A 25 year epidemiologic, clinical and pathologic study. Annals of Internal Medicine 88: 162167. Jones JG & Hazleman BL (1981) Prognosis and management of polymyalgia rheumatica. Annals of the Rheumatic Diseases 40: 1-5. Klein RG, Hunder GG, Stanson AW & Sheps SG (1975) Larger artery involvement in giant cell arteritis. Annals of Internal Medicine 83: 806-812. Kyle V & Hazleman BL (1988) The clinical and laboratory course of polymyalgia rheumatica/ giant cell arteritis. British Journal of Rheumatology 27(supplement 1): 7. Kyle V & Hazleman B (1989a) Treatment of polymyalgia rheumatica and giant cell arteritis. I. Steroid regimens in the first two months. Annals of the Rheumatic Diseases 48" 658-661. Kyle V & Hazleman BL (1989b) Treatment of polymyalgia rheumatica and giant cell arteritis. II. Relation between steroid dose and steroid associated side effects. Annals of the Rheumatic Diseases 48: 662-666. Kyle V & Hazleman BL (1990) Stopping steroids in polymyalgia rheumatica and giant cell arteritis. British Medical Journal 300: 344-345. Love D, Rapkin J, Lesser R et al (1986) Temporal arteritis in Blacks. Annals of Internal Medicine 105: 387-389. Lundberg I & Hedfors E (1990) Restricted dose and duration of corticosteroid treatment in patients with polymyalgia rheumatica and temporal arteritis. Journal of Rheumatology 17: 1340-1345. Mehler MF & Rabinowich L (1988) The clinical neuro-ophthalmologic spectrum of temporal arteritis. American Journal of Medicine 85: 83%844. Mosher HA (1959) The prognosis in temporal arteritis. Archives of Ophthalmology 62: 641-644. Myles AB (1975) Polymyalgia rheumatica and giant cell arteritis; a seven year survey. Rheumatology and Rehabilitation 14: 231-235. Nordberg E & Bengtsson B A (1989) Death rates and causes of death in 284 consecutive patients with giant cell arteritis confirmed by biopsy. British Medical Journal 299: 549-550. Reich KA, Giansiracusa DF & Strongwater SL (1990) Neurologic manifestations of giant cell arteritis. American Journal of Medicine 89: 67-72. Ridley MR, Perera T & Myles AB (1991) Prevention of blindness in giant cell arteritis by corticosteroid treatment. British Journal of Rheumatology (in press). Rubinow A, Brandt KD, Cohen AS & Sack B (1984) Iatrogenic morbidity accompanying suppression of temporal arteritis by adrenal corticosteroids. Annals of Ophthalmology 16: 258--265. Russell RWR (1959) Giant cell arteritis. Quarterly Journal of Medicine 28: 471-489. Save-Soderbergh J, Malmvall BO, Andersson R & Bengtsson BA (1986) Giant cell arteritis and cause of death. Journal of the American Medical Association 255: 493-496.
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Schneider H, Weber A & Ballen P (1971) Visual prognosis in temporal arteritis. Annals of Ophthalmology 3: 1215-1227. Smyth CJ (1966) Non-articular rheumatism and the fibrositis syndrome. In Holland JL (ed.) Arthritis and Allied Conditions, p 775. London: Kimpton. Sonnenblick M, Nesher G & Rosin A (1989) Nonclassical organ involvement in temporal arteritis. Seminars in Arthritis and Rheumatism 19: 183-190. Sorensen PR & Lorenzen I (1977) Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. Acta Medica Scandinavica 201: 207-213. Vilaseca J, Gonzalez A, Cid MC, Lopez-Vivancos J & Ortega A (1987) Clinical usefulness of temporal artery biopsy. Annals of the Rheumatic Diseases 46: 282-285. Von Knorring J (1979) Treatment and prognosis in polymyalgia rheumatica and temporal arteritis. Acta Medica Scandinavica 205: 429-435. Whitfield AGW, Trevor Cooke W, Jameson-Evans P & Rudd C (1953) Temporal arteritis and its treatment with cortisone. Lancet i: 408-412. Whitfield AGW, Bateman M & Cooke T (1963) Temporal arteritis. British Journal of Ophthalmology 47: 555-566. Wilkinson IMS & Russell RWR (1972) Arteries of the head and neck in giant cell arteritis. Archives of Neurology 27: 378-391.
Prognosis of polymyalgia rheumatica and giant cell arteritis ALAN
B. M Y L E S
Polymyalgia rheumatica and giant cell arteritis are so closely related that it might be thought that the prognosis would be similar, and this is probably correct. There is quite good evidence concerning the prognosis of giant cell arteritis but very much less for polymyalgia rheumatica; they will be considered individually, while realizing that there may be features of both conditions in many individuals and that some starting with one condition may develop features of the other. There are differences, however, partly related to the use of different classifications of the two conditions.
PROGNOSIS OF GIANT CELL ARTERITIS
Mortality Although commonly presenting in the temporal or cranial arteries, giant cell arteritis affects other arteries widely and it might be supposed that this involvement would often have serious consequences, but survival statistics have usually shown that patients with giant cell arteritis have the same expectation for life as the general population (Hauser et al, 1971; Huston et al, 1978; Andersson et al, 1986). Nordberg and Bengtsson (1989), however, followed up 284 patients diagnosed histologically as having giant cell arteritis. The mortality over a 10-year period did not differ from the general population but death from vascular disease in the first year was considerably higher than expected, 17 dying within the first 4 months (eight from cerebral vascular disease and the others from myocardial infarction and other disease of major blood vessels). They suggested that although all the patients had ieceived corticosteroid treatment, the dose had been inadequate in 13 of them and that this was a contributory factor. Save-Soderbergh (1986) described nine patients who had died as a result of giant cell arteritis (five with cerebral involvement, two from myocardial infarction and two from aortic dissection) and also suggested that inadequate corticosteroid treatment contributed to the deaths. Bailli~re's ClinicalRheumatology-Vol. 5, No. 3, December 1991 ISBN 0-7020--1537-7
493 Copyright 9 1991, by Bailli~re Tindall All rights of reproduction in any form reserved
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A.B. MYLES
Neurological involvement A wide range of neurological abnormalities have been described, mainly resulting from involvement of the cranial arteries and nerves (Mehler and Rabinowich, 1988; Reich et al, 1990), many having a good prognosis. Major vessel involvement usually affects the vertebral arteries and is seldom diagnosed during life (Wilkinson and Russell, 1972). Caselli (1990) described three patients with multi-infarct dementia attributable to giant cell arteritis who responded well to treatment with corticosteroids.
Large artery involvement Even in those patients who are known to have giant cell arteritis, it is difficult to be certain whether major vessel involvement is a result of giant cell arteritis or of atherosclerotic disease, common in this age group, which explains the disparity of some of the papers (Sonnenblick et al, 1989). Thus Russell (1959) regarded giant cell arteritis as a serious disease, seven of 28 patients developing severe systemic complications, whereas Mosher (1959) followed 32 patients, 11 of whom died but in none of whom giant cell arteritis was thought to be relevant to the death. Graham et al (1981) investigated 32 deaths in 58 patients; eight died early and, in three, giant cell arteritis was the probable cause of death. Large artery involvement, other than affecting the cranial arteries, is described in 34 of 248 patients by Klein et al (1975), who considered that the prognosis in adequately treated patients was good, although three of their patients died of complications related to giant cell aortitis. Bengtsson and Malmvall (1981) found the aortic arch syndrome in six patients in a group of 90 but it did not seem to affect the mortality.
Ocular complications The first clear description of temporal arteritis was made by Horton et al (1932), but they did not recognize the serious ocular effects, which became apparent in the next two decades (Cooke et al, 1945). Crosby and Wadsworth (1948) found that three of 25 patients with temporal arteritis became blind and it became apparent that this was the most serious consequence of temporal arteritis. Birkhead et al (1957), reviewing their own cases and the literature up to date, concluded that blindness occurred in 22% of patients with temporal arteritis and about half of these developed blindness in the other eye, usually within a few weeks. A lower incidence of blindness at presentation (7%) has subsequently been described by Bengtsson and Malmvall (1982), which doubtless represents earlier diagnosis of giant cell arteritis and recognition of the complications, and earlier corticosteroid treatment. The earlier figures are unlikely to be accurate and may have underestimated the real incidence in the past. Sudden loss of vision, which would now be recognized as resulting from giant cell arteritis, was at that time considered to be ischaemic due to optic neuritis and would have been classified differently if there were no obvious evidence of temporal arteritis.
VRO6NOSm
495
It subsequently became apparent, as a result of estimating the ESR and, if raised, doing a temporal artery biopsy, that many of these patients did have temporal arteritis (Cullen, 1967). Beri et al (1987) found that 19 out of 50 patients with anterior ischaemic optic neuropathy attributed to giant cell arteritis had evidence of involvement of both eyes at presentation. Loss of vision resulting from ocular involvement by giant cell arteritis is usually permanent. Treatment is given to suppress the disease and avoid further blindness, particularly in the other eye, which usually occurs early in the course of the disease and seldom after 6 months from the first symptoms. The degree of recovery of vision depends upon the amount of vision that is lost. If it is considerable, there is usually no significant return of vision but, if it is partial, some recovery of vision has often been described and attributed to prompt corticosteroid treatment (Whitfield et al, 1953; Schneider et al, 1971; Cullen and Coleiro, 1976). Profound visual loss is almost always permanent, although there are a small number of reports of return of vision in patients given intravenous steroids within a short time after onset. Enthusiastic treatment of this nature has doubtless been used quite often but the failures are not reported (Clearkin and Watts, 1990). The main object of treatment is to protect the uninvolved eye, and earlier reports suggested that this was likely to occur within a few weeks, some patients presenting with blindness in both eyes. This tendency ceased abruptly as soon as corticosteroid treatment was used and this protective effect has been apparent in all subsequent studies.
Prevention of further visual loss Relatively high corticosteroid doses, i.e. cortisone 300 mg daily, were used in the earlier studies and doses of this nature have been used commonly. It is frequently stated that these high doses are needed to provide this degree of protection but there have been no long-term controlled studies designed to elucidate this matter. Lower doses have been used by Myles (1975), Behn et al (1983), Delecoeuillerie et al (1988), Lundberg and Hedfors (1990) and Ridley et al (1991) without any disadvantage. It remains, however, the practice of most clinicians, particularly ophthalmologists, to use high starting doses of prednisolone, such as 80-120 mg daily in the belief that this is necessary to prevent blindness (Beri et al, 1987). Table 1 shows the reports in the world literature of blindness occurring in giant cell arteritis after corticosteroid treatment has been started, indicating the starting dose, the dose at the time of the onset of blindness, and the duration after starting treatment. It will be seen that the numbers are small, 19 in all, over a period of nearly 30 years. In most cases high starting doses have been used; the blindness has occurred within the first 4 weeks in nine of the cases, although the timing was not stated in another five. It is clear that the high doses, when used, did not always prevent blindness but have done so in a very high proportion. It is possible that the prognosis may be equally as good with the lower starting doses, although they do not always provide the same degree of symptomatic relief from other symptoms of giant cell arteritis (Kyle and Hazleman, 1989a).
496
A.B. MYL~S Table 1. Occurrence of blindness during cortieosteroid treatment (individual cases).
Authors
Year
Prednisolone: dose at time (rag)
Prednisolone: starting dose (mg)
Whitfield et al Cullen Sorensen and Lorenzen Sorensen and Lorenzen Healy and Wilske Huston et al Hugod and Schebel Calamia and Hunder Herrick Herrick Jones and Hazleman Jones and Hazleman Bengtsson and Malmvall Bengtsson and Malmvall Beri et al Delecoeuillerie et al Delecoeuillerie et al Faarvang and PontoppidanThyssen Kyle and Hazleman
1963 1972 1977 1977 1977 1976 1979 1980 1980 1980 1981 1981 1982 1982 1987 1988 1988 1989
Adequate 5 2.5 1.5 10 25 60 60 60 60 10 8 20 30 80-120 >60 60 10
Not stated Not stated 30-60 30-60 40 60 60 50 60 60 Not stated Not stated 20 30 80-120 >60 30 80
1989a
40
40
Duration from starting Not stated 7 years Not stated Not stated 2 months 7 days 5 days 4 weeks 1 day 4 days 1 year 8 months 2 days 5 days 2 days Not stated Not stated 2 months 1 week
Clinicians are u n d e r s t a n d a b l y worried that stopping steroids too soon m a y allow giant cell arteritis to b e c o m e active again and blindness to occur, and for this reason p r o l o n g e d corticosteroid t r e a t m e n t or p r o l o n g e d followup are s o m e t i m e s suggested. T h e r e is, h o w e v e r , little evidence f r o m the literature that this is a significant danger. Blindness occurring m o r e than one year after corticosteroid t r e a t m e n t was started was only described in one patient (Cullen, 1972) and, f u r t h e r m o r e , this patient had continued to receive 5 m g p r e d n i s o l o n e daily as m a i n t e n a n c e t r e a t m e n t , p r e s u m a b l y to prevent this possible risk (Kyle and H a z l e m a n , 1990). Thus the prognosis for small vessel arteritis, as in the eye, is very g o o d after corticosteroid t r e a t m e n t has been started. It is also g o o d for large vessel involvement, but there is less information a b o u t this and it is occasionally found late in the course of the disease, in spite of apparently a d e q u a t e treatment. T h e histological changes are t h o u g h t to resolve quite rapidly; for this reason t e m p o r a l artery biopsy is r e c o m m e n d e d within a week of starting treatment (Allison and Gallagher, 1984), but changes m a y persist and Fauchald et al (1972) s h o w e d evidence of active arteritis m a n y years after presentation of the disease.
PROGNOSIS OF POLYMYALGIA RHEUMATICA A l t h o u g h Bruce, in 1888, is usually accepted as the first p e r s o n to have described polymyalgia rheumatica, it was not until 1957 that B a r b e r pro-
PROGNOSIS
497
vided the name, and it was not widely accepted for a further 10 years or so. As recently as 1966 an American textbook stated: 'thus the proponents of a syndrome of polymyalgia rheumatica may have selected those early patients with fibrositis who happened to have a high sedimentation rate, arbitrarily given their illness a new pseudo specific name and exposed this high risk group to unnecessary and unwise steroid therapy' (Smyth, 1966). In the light of these difficulties in acceptance of the diagnosis, it is not surprising that there were few clear accounts of the natural history of this condition. Its recognition came with the realization that corticosteroids in quite small amounts produced almost complete symptomatic relief, and most patients are treated in this way. Almost all studies, therefore, of the duration of polymyalgia rheumatica are studies of the length of time for which corticosteroid treatment has been necessary to provide adequate symptomatic relief and to protect from the possible complications of the disease. The results of these studies have varied considerably but in a fairly consistent manner. The later studies have shown a longer duration of treatment, and relapses occurred in those who had been treated for an inadequate length of time in the earlier studies. Originally thought to be a benign condition, which was self-limiting and recovered in the course of a few years, the reported outcome has subsequently varied considerably. Bagratuni (1963), describing the anarthritic rheumatoid syndrome which was subsequently to be called polymyalgia rheumatica, found a duration of about 7 years before corticosteroid treatment was used. Since corticosteroid treatment has been available and stopping the treatment has been considered to be the end-point of the disease, the reported duration has generally increased with the passage of time. Von Knorring (1979) reported a mean time to withdrawal of 3 years, but there were 20 subsequent relapses. Coomes et al (1976) found that treatment for 5 years was necessary in 84% of their patients. Behn et al (1983) found that only 72 of 176 patients had stopped steroids at a mean of 31 months, but 30 subsequently relapsed. Similar findings were reported by Bengtsson and Malmvall (1981) and Ayoub et al (1985). Delecoeuillerie et al (1988) reported that only 65 of 132 patients had been able to stop steroids after a mean of 25 months. Kyle and Hazleman (1988) found that a quarter were able to stop after 2 years. These papers are all from Europe. In the United States, however, Chuan et al (1982) reported a mean duration of treatment of 11 months, and in other studies most patients had stopped treatment within 2 years (Huston et al, 1978; Huston and Hunder, 1980). Using lower than usual steroid doses, Lundberg and Hedfors (1990) reported a mean duration of treatment of 17 months and concluded that corticosteroid treatment could be stopped in most cases after 2 years. The disparity concerning the length of treatment is not easy to explain. There is no suggestion that the severity of the disease differed in these series or that corticosteroid regimens make any difference to the ultimate length of treatment. It is possible that clinical practice differs considerably in these countries; complications of the disease at the late stages are unusual, and the need for continuing treatment is usually the clinical judgement of the need to
498
A . B . MYLES
provide corticosteroids to relieve symptoms, rather than being made on the basis of any objective test. Unfortunately, such tests do not exist but possibly may do so in the future. Dasgupta et al (1989) found a selective reduction of CD8+ suppressor cytotoxic cells in polymyalgia rheumatica and giant cell arteritis, which persisted for about 2 years and was not related to symptomatic control of the disease. Such tests may, in due course, be able to give an indication of when corticosteroid withdrawal could be considered. Non-steroidal anti-inflammatory drugs have been suggested for treatment of uncomplicated polymyalgia rheumatica (Chuan et al, 1982). The literature is not clear on this matter but it is possible that they are used for symptomatic suppression in the late stages of polymyalgia rheumatica at a stage when continued low dose corticosteroid treatment would be recommended in most European countries. There is no adequate evidence about this practice and it is by no means certain that non-steroidal antiinflammatory analgesics are less likely to cause side-effects. Other disease associations
There do not seem to be any important consequences of polymyalgia rheumatica, other than the possibility of developing giant cell arteritis and its complications. There is some evidence to suggest that these conditions are autoimmune diseases, and overlap with other conditions might have been expected. There has been shown to be an increased risk of developing hypothyroidism but there is no evidence to suggest that there is an increased risk of any other autoimmune disease (Dent and Edwards, 1978; Bowness et al, 1991). FACTORS WHICH MAY INFLUENCE PROGNOSIS Unfortunately, there does not seem to be any method of assessing the prognosis in an individual patient. Complications of giant cell arteritis tend to occur early in the disease and seem to be more common in men than women. The main complication of polymyalgia rheumatica is the development of giant cell arteritis, which may occur at any time but often late in the course of the disease at a time when the steroid dose is being reduced. Factors such as the height of the ESR at onset (Ellis and Ralston, 1983) or the presence and degree of histological evidence of giant cell arteritis do not affect the ultimate prognosis (Bevan et al, 1968; Hall et al, 1983; Vilaseca, 1987). Some patients with polymyalgia rheumatica and negative biopsies have developed giant cell arteritis in spite of corticosteroid treatment (Jones and Hazleman, 1981). Delecoeuillerie et al (1988) found a very significant increase in complications of temporal arteritis in males compared with females, which has not previously been reported. It was originally suggested that Negroes had a low instance of polymyalgia rheumatica and giant cell arteritis, but both have been found (Gonzalez et al, 1989), and it was suggested by Love et al (1986) that blindness occurred more commonly in Negroes affected by this con-
PROGNOSIS
499
dition. Friedman and Friedman (1988) noted that non-western Jews seem to have a worse prognosis with regard to recurrence of giant cell arteritis than western Jews. PROGNOSIS WITH REGARD TO CORTICOSTEROID TREATMENT
It has often been assumed that the relatively low corticosteroid doses used in these conditions would not result in an important incidence of corticosteroid side-effects, and it has even been suggested that these patients were in some way resistant to the side-effects. Unfortunately, this has not proved to be correct. Rubinow et al (1984) described serious corticosteroid complications in 11 patients receiving a relatively high maintenance dose of prednisone, averaging 26.3 mg daily, which included fractures in six, three with steroid myopathy, two with psychosis, two with diabetes and three with severe infection; four of these patients died. Another group with similar severity of disease received an average maintenance dose of 13 mg daily and did not have any severe complications. Ayoub et al (1985) described significant corticosteroid related side-effects in 17 of 76 patients, although there were no deaths. Kyle and Hazleman (1989b) undertook a specific study of the relationship between steroid dosage and steroid side-effects. They found that significant side-effects were present in 36%; this figure rose to 76% if weight gain was included. The side-effects were significantly more common in those starting with an initially high dose of prednisolone of more than 30mg daily, and in those who had a high cumulative total dose. Using somewhat lower starting doses, Behn et al (1983) reported a lower incidence of corticosteroid side-effects at 16%. The most important expected corticosteroid side-effect of long-term treatment is osteoporosis, but Andersson et al (1990), studying radiological osteoporosis and the mineral content of heel bone in patients with giant cell arteritis treated with prednisolone for 5 years on average doses, did not show an increased incidence of osteoporosis over a control group. There are now many contradictory papers concerning effects of low dose corticosteroid treatment on the skeleton, and the contradictions have not yet been satisfactorily answered. Possibly newer radiological techniques will solve this problem. Corticosteroid related side-effects have usually been found where they have been looked for, and may become a greater source of morbidity and mortality than the disease for which the treatment was originally given. INTERCHANGE BETWEEN POLYMYALGIA RHEUMATICA AND GIANT CELL ARTERITIS It is clear that some patients with giant cell arteritis develop polymyalgia rheumatica and it is usually accepted that patients with polymyalgia rheumatica are at risk of developing giant cell arteritis, which is one of the
500
a.U. MYLES
reasons for using corticosteroid treatment, most clinicians using a higher dose in those thought to have giant cell arteritis. In my series of 367 patients with polymyalgia rheumatica and giant cell arteritis, 15 of those with giant cell arteritis only at presentation developed polymyatgia rheumatica subsequently, and seven patients with apparently uncomplicated polymyalgia at onset developed giant cell arteritis (unpublished). However, Sorensen and Lorenzen (1977), studying 63 patients with polymyalgia rheumatica and giant cell arteritis, considered that there was no justification for making a distinction between the two diseases with regard to treatment. Delecoeuillerie et al (1988) examined retrospectively 210 patients with polymyalgia or giant cell arteritis; 132 patients were considered to have 'pure' polymyalgia rheumatica requiring low dose corticosteroid treatment, and it was thought that this distinction could be made at the original presentation, regardless of biopsy findings. Chuan et al, however, in 1982, also concluded that there was a group of patients with polymyalgic symptoms but without evidence of giant cell arteritis, judged by extensive temporal artery biopsy, and concluded that these patients could be treated with non-steroidal anti-inflammatory drugs or low doses of corticosteroids. Most clinicians, however, have patients with initially 'pure' temporal arteritis who subsequently develop polymyalgic symptoms, often while the corticosteroid dose is being withdrawn, and patients with polymyalgia rheumatica may develop features of giant cell arteritis (Hamilton et al, 1971). Jones and Hazleman (1981) did not find that polymyalgia rheumatica was a benign disease, finding some evidence of giant cell arteritis in 55% and that neurological or visual complications occurred in 26%. There is therefore some disparity concerning the prognosis of polymyalgia rheumatica, which is probably more apparent than real: those favouring the benign prognosis for 'pure' polymyalgia rheumatica excluding patients with any features of giant cell arteritis from their studies. It is clear, however, that patients with features of both conditions tend to have the worst prognosis with regard to duration of treatment and complications of the disease (Delecoeuillerie et al, 1988; Kyle and Hazleman, 1988; Lundberg and Hedfors, 1990). SUMMARY
Polymyalgia rheumatica and giant cell arteritis are amongst the most satisfying conditions for clinicians to diagnose and treat because the unpleasant effects and serious consequences of these conditions can be almost entirely prevented by corticosteroid treatment; the fact that the side-effects of this treatment sometimes seem to be more serious than the complications of the disease is an indication of its effectiveness. Unfortunately, there is no objective way of determining the prognosis in the individual, and decisions concerning duration of treatment remain empirical. REFERENCES
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PROGNOSIS
501
Andersson R, Malmvall BO & Bengtsson BA (1986) Long term survival in giant cell arteritis. Acta Medica Scandinavica 220: 361-364. Andersson R, Rundgren A, Rosengren K, Bengtsson BA, Malmvall BE & Mellstrom D (1990) Osteoporosis after long-term corticosteroid treatment of giant cell arteritis. Journal of Internal Medicine 272: 391-395. Ayoub WT, Franklin CM & Torretti D (1985) Polymyalgia rheumatica-~turation of therapy and long term outcome. American Journal of Medicine 79: 309-315. Bagratuni L (1963) Prognosis in the anarthritic rheumatoid syndrome. British Medical Journal 1: 513--518. Barber HS (1957) Myalgic syndrome with constitutional effects. Polymyalgia Rheumatica. Annals of the Rheumatic Diseases 16: 230-237. Behn AR, Perera T & Myles AB (1983) Polymyalgia rheumatica and corticosteroids: how much for how long? Annals of the Rheumatic Diseases 42: 374-378. Bengtsson BA & Malmvall BO (1981) Prognosis of giant cell arteritis including temporal arteritis and polymyalgia rheumatica. Acta Medica Scandinavica 209: 337-345. Bengtsson BA & Malmvall BO (1982) Eye complications in giant cell arteritis. Acta Medica Scandinavica, Supplement 658: 38-43. Beri M, Klugman MR, Kohler J & Hayreh SS (1987) Anterior ischaemic optic neuropathy. Ophthalmology 94: 1020-1028. Bevan AT, Dunnill MS & Harrison MJG (1968) Clinical and biopsy findings in temporal arteritis. Annals of the Rheumatic Diseases 27: 271-277. Birkhead NC, Wagener HP & Shick RM (1957) Treatment of temporal arteritis with adrenal corticosteroids. Journal of the American Medical Association 163: 821-827. Bowness P, Shotliff K, Middlemiss A & Myles AB (1991) Prevalence of hypothyroidism in patients with polymyalgia rheumatica and giant cell arteritis. British Journal of Rheumatology 30: 349-351. Bruce W (1888) Senile rheumatic gout. British Medical Journal li: 811-813. Calamia KT & Hunder GG (1980) Manifestations of giant cell (temporal) arteritis. Clinics in Rhei~matic Diseases 6: 389-403. Caselli RJ (1990) Giant cell (temporal) arteritis: a treatable cause of multi-infarct dementia. Neurology 40: 753-755. Chuan TY, Hunder GG, Ilstrup DM & Kurland LT (1982) Polymyalgia rheumatica--a 10-year epidemiologic and clinical study. Annals oflnternal Medicine 97: 672-680. Clearkin LG & Watts MT (1990) Ocular involvement in giant cell arteritis. British Journal of Hospital Medicine 43: 373-376. Cooke WT, Cloake PCP, Govan ADT & Colbeck JC (1945) Temporal arteritis: a generalised vascular disease. Quarterly Journal of Medicine 57: 47-75. Coomes EN, Ellis RM & Kay AG (1976) A prospective study of 102 patients with the polymyalgia rheumatica syndrome. Rheumatology and Rehabilitation 15: 270-276. Crosby RC & Wadsworth R (1948) Temporal arteritis. Archives of Internal Medicine 81: 431-464. Cullen JF (1967) Occult temporal arteritis. British Journal of Ophthalmology 51: 513-525. Cullen J (1972) Temporal arteritis. British Journal of Ophthalmology 56: 584-588. Cullen JF & Coleiro JA (1976) Ophthalmic complications of giant cell arteritis. Survey of Ophthalmology 20: 247-259. Dasgupta B, Duke O, Timms AM, Pitzalis C & Panayi GS (1989) Selective depletion and activation of CD8+ lymphocytes from peripheral blood of patients with polymyalgia rheumatica and giant cell arteritis. Annals of the Rheumatic Diseases" 48: 30%311. Delecoeuitlerie G, Joly P, Cohen De Lara A & Paolaggi JB (1988) Polymyalgia rheumatica and giant cell arteritis: a retrospective analysis of prognostic features and different corticosteroid regimens (11 year survey of 210 patients). Annals of the Rheumatic Diseases 47: 733-739. Dent RG & Edwards OM (1978) Autoimmune thyroid disease and the polymyalgia rheumatica-giant cell arteritis syndrome. Clinical Endocrinology 9: 215-219. Ellis ME & Ralston S (1983) The ESR in the diagnosis and management of the polymyalgia rheumatica/giant cell arteritis syndrome. Annals of the Rheumatic Diseases 42: 168170. Faarvang KL & Pontoppidan-Thyssen E (1989) Giant cell arteritis. Journal of Internal Medicine 225: 215-216.
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Fauchald P, Rygvold O & Oystese B (1972) Temporal arteritis and polymyalgia rheumatica: clinical and biopsy findings. Annals oflnternal Medicine 77: 845-852. Friedman G & Friedman B (1988) Prolonged corticosteroid treatment in the management of temporal arteritis. Klinische Wochenschrift 66: 1167-1170. Gonzalez EB, Varner WT, Lisse JR, Daniels JC & Hokanson JA (1989) Giant cell arteritis in the southern United States. Archives of Internal Medicine 149: 1561-1565. Graham E, Holland A, Avery A & Russell RW (1981) Prognosis in giant cell arteritis. British Medical Journal 282: 269-271. Hall S, Lie JT, Kurland LT, Persellin S, O'Brien PC & Hunder G G (1983) The therapeutic impact of temporal artery biopsy. Lancet ih 1217-1220. Hamilton CR, Shelley WM & Tumulty PA (1971) Giant cell arteritis. Medicine 50: 1-27. Hauser WA, Ferguson RH, Holley KE & Kurland LT (1971) Temporal arteritis in Rochester, Minnesota. Mayo Clinic Proceedings 46: 597-602. Healy LA & Wilske KR (1977) Manifestations of giant cell arteritis. Medical Clinics of North America 61: 261-269. Herrick PC (1980) Cranial arteritis. A preventable cause of blindness. Transactions of the Ophthalmological Society of New Zealand 32: 106-109. Horton BT, Magath TB & Brown GE (1932) Undescribed form of arteritis of temporal vessels. Mayo Clinic Proceedings 7: 700-701. Hugod C & Schebel M (1979) Temporal arteritis. Acta Medica Scandinavica 205: 445-446. Huston KA, Hunder GG, Lie JT, Kennedy R H & Elveback LR (1978) Temporal arteritis. A 25 year epidemiologic, clinical and pathologic study. Annals of Internal Medicine 88: 162167. Jones JG & Hazleman BL (1981) Prognosis and management of polymyalgia rheumatica. Annals of the Rheumatic Diseases 40: 1-5. Klein RG, Hunder GG, Stanson AW & Sheps SG (1975) Larger artery involvement in giant cell arteritis. Annals of Internal Medicine 83: 806-812. Kyle V & Hazleman BL (1988) The clinical and laboratory course of polymyalgia rheumatica/ giant cell arteritis. British Journal of Rheumatology 27(supplement 1): 7. Kyle V & Hazleman B (1989a) Treatment of polymyalgia rheumatica and giant cell arteritis. I. Steroid regimens in the first two months. Annals of the Rheumatic Diseases 48" 658-661. Kyle V & Hazleman BL (1989b) Treatment of polymyalgia rheumatica and giant cell arteritis. II. Relation between steroid dose and steroid associated side effects. Annals of the Rheumatic Diseases 48: 662-666. Kyle V & Hazleman BL (1990) Stopping steroids in polymyalgia rheumatica and giant cell arteritis. British Medical Journal 300: 344-345. Love D, Rapkin J, Lesser R et al (1986) Temporal arteritis in Blacks. Annals of Internal Medicine 105: 387-389. Lundberg I & Hedfors E (1990) Restricted dose and duration of corticosteroid treatment in patients with polymyalgia rheumatica and temporal arteritis. Journal of Rheumatology 17: 1340-1345. Mehler MF & Rabinowich L (1988) The clinical neuro-ophthalmologic spectrum of temporal arteritis. American Journal of Medicine 85: 83%844. Mosher HA (1959) The prognosis in temporal arteritis. Archives of Ophthalmology 62: 641-644. Myles AB (1975) Polymyalgia rheumatica and giant cell arteritis; a seven year survey. Rheumatology and Rehabilitation 14: 231-235. Nordberg E & Bengtsson B A (1989) Death rates and causes of death in 284 consecutive patients with giant cell arteritis confirmed by biopsy. British Medical Journal 299: 549-550. Reich KA, Giansiracusa DF & Strongwater SL (1990) Neurologic manifestations of giant cell arteritis. American Journal of Medicine 89: 67-72. Ridley MR, Perera T & Myles AB (1991) Prevention of blindness in giant cell arteritis by corticosteroid treatment. British Journal of Rheumatology (in press). Rubinow A, Brandt KD, Cohen AS & Sack B (1984) Iatrogenic morbidity accompanying suppression of temporal arteritis by adrenal corticosteroids. Annals of Ophthalmology 16: 258--265. Russell RWR (1959) Giant cell arteritis. Quarterly Journal of Medicine 28: 471-489. Save-Soderbergh J, Malmvall BO, Andersson R & Bengtsson BA (1986) Giant cell arteritis and cause of death. Journal of the American Medical Association 255: 493-496.
PROGNOSIS
503
Schneider H, Weber A & Ballen P (1971) Visual prognosis in temporal arteritis. Annals of Ophthalmology 3: 1215-1227. Smyth CJ (1966) Non-articular rheumatism and the fibrositis syndrome. In Holland JL (ed.) Arthritis and Allied Conditions, p 775. London: Kimpton. Sonnenblick M, Nesher G & Rosin A (1989) Nonclassical organ involvement in temporal arteritis. Seminars in Arthritis and Rheumatism 19: 183-190. Sorensen PR & Lorenzen I (1977) Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. Acta Medica Scandinavica 201: 207-213. Vilaseca J, Gonzalez A, Cid MC, Lopez-Vivancos J & Ortega A (1987) Clinical usefulness of temporal artery biopsy. Annals of the Rheumatic Diseases 46: 282-285. Von Knorring J (1979) Treatment and prognosis in polymyalgia rheumatica and temporal arteritis. Acta Medica Scandinavica 205: 429-435. Whitfield AGW, Trevor Cooke W, Jameson-Evans P & Rudd C (1953) Temporal arteritis and its treatment with cortisone. Lancet i: 408-412. Whitfield AGW, Bateman M & Cooke T (1963) Temporal arteritis. British Journal of Ophthalmology 47: 555-566. Wilkinson IMS & Russell RWR (1972) Arteries of the head and neck in giant cell arteritis. Archives of Neurology 27: 378-391.
