Rheumatology and Rehabilitation, 1975, 14, 231
POLYMYALGIA RHEUMATICA AND GIANT CELL ARTERITIS: A SEVEN-YEAR SURVEY* BY A. B. MYLES St. Peter's Hospital, Chertsey, North West Surrey Health District
THE syndrome of polymyalgia rheumatica and its association with giant cell arteritis is now well recognized, and its characteristics are outlined in Table I (Copeman and Myles, 1970; Dixon, 1969; Fauchald et al., 1972; Hamrin, 1972; and Plotz and Spiera, 1969). TABLE I MAIN FEATURES OF POLYMYALGIA RHEUMATICA
Pain and stiffness in girdle and spinal muscles Marked morning stiffness No evidence of polyarthritis No evidence of polymyositis E.s.r. considerably raised Good response to corticosteroids Occurs mainly in the elderly In 1968 it was decided to follow up indefinitely all patients diagnosed as polymyalgia rheumatica or giant cell arteritis in the rheumatology department of St. Peter's Hospital. This paper analyses the findings after seven years, in an attempt to elucidate some of the problems of polymyalgia rheumatica and giant cell arteritis. METHODS Eighty-four patients have been diagnosed as having polymyalgia rheumatica or giant cell arteritis since 1968. Two were lost to follow-up and seven were subsequently shown to have been incorrectly diagnosed (Table II). In one, no diagnosis was made, but continued follow-up did not reveal the presence of any underlying disease. * Paper read at the Annual Meeting of the British Association for Rheumatology and Rehabilitation, London, April 1975. 231
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
SUMMARY The seven-year results of all cases (84) diagnosed as polymyalgia rheumatica or giant cell arteritis are reported. The diagnosis proved to be incorrect in seven, of which six had a polyarthritis. Most cases were treated with prednisolone, starting with 20 mg daily for those with evidence of cranial arteritis, and 10 mg for those without. Fourteen patients were withdrawn from treatment (after three months to 3J years—mean 21 months), but three relapsed and treatment has been restarted. There was no correlation between the presence or absence of arteritis, the starting dose of prednisolone and the subsequent duration of treatment. A small group (7) received higher doses without obvious advantage. Twenty-two started on 5-9 mg daily, but the dose had to be increased in 13 because of inadequate control of symptoms. Objective physical abnormality, particularly painful limitation of shoulder movement, was present in most cases. No patient developed a serious complication of the disease after treatment had been started. Complications of treatment were infrequent. Spinal osteoporosis occurred in seven, but did not cause long-term disability.
232
RHEUMATOLOGY AND REHABILITATION VOL. XTV NO. 4 TABLE II ANALYSIS OF PATIENTS
Initial total = 84 Lost to follow-up = 2
f Polyarthritis 5 Incorrect diagnosis = 7 4 Ankylosing spondylitis 1 I Undiagnosed 1
Polymyalgia rheumatica Cranial arteritis Mixed Total Age: 48-90, mean 69
Temporal artery biopsy
Positive
52 5 18
38 4 15
9 3 11
75
57
23
Male : Female = 1:4 CLINICAL FINDINGS
In the remaining 75, a clinical diagnosis of polymyalgia rheumatica was made in 52, cranial arteritis in five, and features of both conditions were present in 18. Clinical abnormalities frequently found were: (1) Painful limitation of active and passive movement of the shoulder—58. (2) Painful limitation of hip movement—22. (3) Painless knee effusions, without evidence of osteoarthrosis or erosions—6. (4) Dementia, confusion or depression—15. The following investigations were performed in all cases: haemoglobin, erythrocyte sedimentation rate (e.s.r.), urea, alkaline phosphatase (liver function tests since 1973), creatine kinase, plasma proteins and serum electrophoretic strip, autoimmune profile, radiography of chest and pelvis. RESULTS OF INVESTIGATIONS
Haemoglobin range was 15.5 g/dl to 8.5 g/dl, mean 12.7 g/dl. Only one patient was significantly anaemic at 8.5 g/dl. This was caused by iron deficiency. The e.s.r. range was 135—17 mm/lh, mean 70 mm/lh. The alkaline phosphatase was slightly raised in four, for which no cause was found. This may have resulted from hepatitis, but routine liver function tests were not done until 1973. There was no clinical evidence of hepatitis in any of these cases. Autoimmune screening showed the presence of a high titre of antinuclear factor in two, but DNA binding was normal, and there was no clinical evidence of systemic lupus erythematosus. A small proportion had low titres of other auto-antibodies of doubtful importance. No significant abnormalities were found in the other investigations. Temporal artery biopsy was performed in 57 of the 75 (see Table II). The majority of those with cranial arteritis or features of both conditions had a positive biopsy, whereas slightly less than a quarter of those with polymyalgia rheumatica alone had a positive biopsy. TREATMENT
Corticosteroid treatment was given in the form of prednisolone in all except six patients, but different schedules were used. The most frequently used regime was to start with 20 mg daily for those with clinical evidence of giant cell arteritis, or 10 mg
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
Cases analysed
MYLES: SURVEY OF PR AND GIANT CELL ARTERITIS
233
FOLLOW-UP
Many patients rapidly became free of symptoms after starting steroids and the e.s.r. fell to normal. Corticosteroids were withdrawn in 14 patients when the disease appeared inactive (Table III). Three of these have relapsed and are now on steroids TABLE III CORTICOSTEROID TREATMENT: PRESENT STATE
Years of treatment
No. of patients
7 6 5 4 3 2 1
2 7 6 8 9 14 12
Withdrawn 14 (3 relapsed) (3 months-3$ years, mean 21 months)
No corticosteroid treatment 6
again. More often, after a period of steroid reduction, some symptoms returned and the e.s.r. rose, necessitating an increase to the previous dose level. Table III shows the number of years for which the remainder have been receiving corticosteroid treatment. Twenty-three, about a third, have had continuous treatment for four years or more. Analysis of the results has not shown any correlation between the presence or absence of arteritis, the starting dose of prednisolone and the subsequent length of treatment required. Table IV shows the number of cases in each treatment group who TABLE IV STARTING PREDNISOLONE DOSE
Treatment group
Starting dose mg/day
A. With evidence of arteritis Without evidence of arteritis B. C.
20 10 20-60 5-9
No. of patients
Dose increased
16 24 7 22
3 13
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
daily for those without evidence of arteritis. A small group started on higher doses up to 60 mg daily, and a further group started on a lower dose, 5-10 mg daily. The patients were seen at intervals of roughly four weeks, somewhat more frequently when on the higher doses. The steroid dose was regulated according to the symptoms, clinical findings, and the erythrocyte sedimentation rate, the aim being to keep the patient almost symptom free and the e.s.r. below 20 mm/lh. When this was achieved, the dose was decreased by 1 mg at each visit for those receiving 10 mg a day or less. In those taking higher doses, the reductions were somewhat greater and more frequent. Six patients were not given steroid treatment. In three, the patients and their family doctors gave a consistent story of polymyalgia rheumatica over a period of months with a considerably raised e.s.r. However, the symptoms, signs and e.s.r. were settling by the time they attended and continued to do so without treatment. Two patients had already been started on indomethacin and one on phenylbutazone with reasonably good results. This treatment was, therefore, continued.
234
RHEUMATOLOGY AND REHABILITATION VOL. XIV NO. 4
COMPLICATIONS
Complications starting after treatment has been initiated have been infrequent, and not necessarily related to giant cell arteritis. Three patients developed intermittent claudication, two had non-fatal myocardial infarction. Mild hepatitis occurred in one. Seven patients have died (Table V). These patients died at home, and the full details TABLE V CAUSE OF DEATH
Myocardial infarction Stroke Bronchopneumonia Pulmonary embolus Carcinoma of stomach Suicide Total
2 1 1 1 1 1 7
are not available, but there was no evidence that arteritis or corticosteroid treatment had been responsible. The only significant complication possibly related to treatment, was the development of marked spinal osteoporosis in seven with vertebral fractures in five. After a period of back pain, they all became virtually free of symptoms. Recurrence has been infrequent, and no patient has experienced continuing disability from this. DISCUSSION The results of this study are broadly similar to others, but show some interesting differences. In some patients the disease becomes inactive after a few years and corticosteroids can be stopped, but this is very variable. In others, prolonged treatment is necessary, and a few have remitted spontaneously over the course of a few months without treatment. Unfortunately there are no features from which a prediction can be made. The therapeutic results in this study have been satisfactory. No patient developed a severe complication of the disease after treatment was started and complications of treatment were infrequent. Many authorities recommend much higher starting doses of prednisolone such as 40-60 mg daily, not only for giant cell arteritis, but also for polymyalgia rheumatica (Fauchald et al., 1972; and Hamilton et ah, 1971). This study
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
required to have their dose increased. No-one starting on 20 mg or more of prednisolone required an increase. Three starting on 10 mg a day required an increase because of a suspicion that cranial arteritis had developed. In the lowest dose group, none of whom had clinical evidence of arteritis, over half had the dose increased, mainly because the polymyalgic symptoms were not controlled adequately. In five, as the dose was reduced, the e.s.r. rose slightly to between 30 and 40 mm/lh but the patients remained free of symptoms. The steroid dose was increased slightly but usually made no difference, Subsequently, provided that the e.s.r. did not rise further, a more gradual steroid reduction has been made. Seven developed a recurrence of pain and stiffness in girdle muscles, usually the shoulders, and sometimes only one, but the e.s.r. remained normal. A local steroid injection often remedied this situation. It should not be assumed however, that the disease was, therefore, localized. Intra-articular steroid administration is one method of giving systemic steroids.
MYLES: SURVEY OF PR AND GIANT CELL ARTERITIS
235
REFERENCES
COPEMAN, W.
S. C. and MYLES, A. B. (1970) British Encyclopaedia of Medical Practice: Medical Progress 1969/70, p. 368. London: Butterworths. DIXON, A. ST. J. (1969) "Polymyalgia Rheumatica". /. R. Coll. Physicians, 4, 55. FAUCHALD, P., RYGVOLD, O. and 0YSTESE, B. (1972) "Temporal Arteritis and Polymyalgia Rheumatica: Clinical and Biopsy Findings". Ann. intern. Med., 77, 845. FERNANDES-HERLIHY, L. (1971) "Polymyalgia Rheumatica". Semin. Arthritis Rheum., 3, 236. HAMILTON, C. R., SHELLEY, W. M. and TUMULTY, P. A. (1971) "Giant Cell Arteritis: Including Temporal Arteritis and Polymyalgia Rheumatica". Medicine, 50, 1. HAMRIN, B. (1972) "Polymyalgia Arteritica". Ada Med. Scand. Suppl. 533, 1. HAUSER, W. A., FERGUSON, R. A., HOLLEY, K. E. and KURLAND, L. T. (1971) "Temporal Arteritis in Rochester, Minnesota, 1951 to 1967". Mayo Clin. Proc, 46, 597. HEALEY, L. A. (1972) In Arthritis and Allied Conditions. Ed. J. L. Hollander. Philadelphia: Lea and Febiger. p. 885. PLOTZ, C. M. and SPIERA, M. D. (1969) "Polymyalgia Rheumatica". Bull. Rheum. Dis., 20, 578. GENERAL DISCUSSION (ABRIDGED) The evidence of abnormality of liver function was discussed. The speaker was not able to answer this in detail, because liver function tests had not been performed routinely until 1973, but he had noted that 5% of all his patients had slightly raised alkaline phosphatase levels. The speaker did not favour the use of ACTH as opposed to oral corticosteroids, partly because the side effects have disadvantages in the elderly, and also because it is more difficult to 'tailor' the dose accurately with adrenal stimulant. He did not think that the diagnosis of polymyalgia rheumatica could be ruled out if the patients developed one or two swollen joints in the course of their disease. One speaker had satisfactorily maintained some cases for as long as 25 years using as small a dose as 2.5 mg of prednisone per day.
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
suggests that a starting dose of 20 mg is adequate for most cases of giant cell arteritis and 10 mg for polymyalgia rheumatica. Most clinicians agree that a gradual reduction of the steroid dose is desirable. If, however, the starting dose is 60 mg, inevitably there will be a prolonged period during which the patient receives moderately high doses and it is this period of high dose treatment which gives rise to corticosteroid complications. A very rapid response to corticosteroid treatment is said to be typical of polymyalgia rheumatica and to have some diagnostic significance (Fernandes-Herlihy, 1971; and Healey, 1972). This rapid response has not been observed in this study, possibly because lower doses have been used. The six patients who subsequently proved to have a polyarthritis responded initially as well as the patients with polymyalgia rheumatica. Rheumatologists have frequently been worried that elderly patients with nonspecific symptoms and a raised e.s.r. from some other cause such as multiple myelomatosis, scattered bone metastases or chronic infection would be incorrectly diagnosed as polymyalgia rheumatica (Hamilton et ah, 1971; and Hauser et al, 1971). This has not occurred in any cases in this study in which the diagnostic errors were confined to patients with polyarthritis, affecting central joints only in the initial stages. It has also been said that the patient with polymyalgia has many complaints and a very high e.s.r. but that no physical abnormalities are found, whereas objective abnormalities particularly of shoulder movement, are present in most patients. Thesefindings,and careful attention to the history, in which marked morning stiffness is the most important feature, contribute to avoiding these diagnostic errors. The management of polymyalgia rheumatica is reasonably straightforward. Nevertheless, there remain some unsolved problems which include the patient with a persistently raised e.s.r. but no other evidence of active disease, and the patient with recurrent pain and stiffness who has a normal e.s.r.
POLYMYALGIA RHEUMATICA AND GIANT CELL ARTERITIS: A SEVEN-YEAR SURVEY* BY A. B. MYLES St. Peter's Hospital, Chertsey, North West Surrey Health District
THE syndrome of polymyalgia rheumatica and its association with giant cell arteritis is now well recognized, and its characteristics are outlined in Table I (Copeman and Myles, 1970; Dixon, 1969; Fauchald et al., 1972; Hamrin, 1972; and Plotz and Spiera, 1969). TABLE I MAIN FEATURES OF POLYMYALGIA RHEUMATICA
Pain and stiffness in girdle and spinal muscles Marked morning stiffness No evidence of polyarthritis No evidence of polymyositis E.s.r. considerably raised Good response to corticosteroids Occurs mainly in the elderly In 1968 it was decided to follow up indefinitely all patients diagnosed as polymyalgia rheumatica or giant cell arteritis in the rheumatology department of St. Peter's Hospital. This paper analyses the findings after seven years, in an attempt to elucidate some of the problems of polymyalgia rheumatica and giant cell arteritis. METHODS Eighty-four patients have been diagnosed as having polymyalgia rheumatica or giant cell arteritis since 1968. Two were lost to follow-up and seven were subsequently shown to have been incorrectly diagnosed (Table II). In one, no diagnosis was made, but continued follow-up did not reveal the presence of any underlying disease. * Paper read at the Annual Meeting of the British Association for Rheumatology and Rehabilitation, London, April 1975. 231
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
SUMMARY The seven-year results of all cases (84) diagnosed as polymyalgia rheumatica or giant cell arteritis are reported. The diagnosis proved to be incorrect in seven, of which six had a polyarthritis. Most cases were treated with prednisolone, starting with 20 mg daily for those with evidence of cranial arteritis, and 10 mg for those without. Fourteen patients were withdrawn from treatment (after three months to 3J years—mean 21 months), but three relapsed and treatment has been restarted. There was no correlation between the presence or absence of arteritis, the starting dose of prednisolone and the subsequent duration of treatment. A small group (7) received higher doses without obvious advantage. Twenty-two started on 5-9 mg daily, but the dose had to be increased in 13 because of inadequate control of symptoms. Objective physical abnormality, particularly painful limitation of shoulder movement, was present in most cases. No patient developed a serious complication of the disease after treatment had been started. Complications of treatment were infrequent. Spinal osteoporosis occurred in seven, but did not cause long-term disability.
232
RHEUMATOLOGY AND REHABILITATION VOL. XTV NO. 4 TABLE II ANALYSIS OF PATIENTS
Initial total = 84 Lost to follow-up = 2
f Polyarthritis 5 Incorrect diagnosis = 7 4 Ankylosing spondylitis 1 I Undiagnosed 1
Polymyalgia rheumatica Cranial arteritis Mixed Total Age: 48-90, mean 69
Temporal artery biopsy
Positive
52 5 18
38 4 15
9 3 11
75
57
23
Male : Female = 1:4 CLINICAL FINDINGS
In the remaining 75, a clinical diagnosis of polymyalgia rheumatica was made in 52, cranial arteritis in five, and features of both conditions were present in 18. Clinical abnormalities frequently found were: (1) Painful limitation of active and passive movement of the shoulder—58. (2) Painful limitation of hip movement—22. (3) Painless knee effusions, without evidence of osteoarthrosis or erosions—6. (4) Dementia, confusion or depression—15. The following investigations were performed in all cases: haemoglobin, erythrocyte sedimentation rate (e.s.r.), urea, alkaline phosphatase (liver function tests since 1973), creatine kinase, plasma proteins and serum electrophoretic strip, autoimmune profile, radiography of chest and pelvis. RESULTS OF INVESTIGATIONS
Haemoglobin range was 15.5 g/dl to 8.5 g/dl, mean 12.7 g/dl. Only one patient was significantly anaemic at 8.5 g/dl. This was caused by iron deficiency. The e.s.r. range was 135—17 mm/lh, mean 70 mm/lh. The alkaline phosphatase was slightly raised in four, for which no cause was found. This may have resulted from hepatitis, but routine liver function tests were not done until 1973. There was no clinical evidence of hepatitis in any of these cases. Autoimmune screening showed the presence of a high titre of antinuclear factor in two, but DNA binding was normal, and there was no clinical evidence of systemic lupus erythematosus. A small proportion had low titres of other auto-antibodies of doubtful importance. No significant abnormalities were found in the other investigations. Temporal artery biopsy was performed in 57 of the 75 (see Table II). The majority of those with cranial arteritis or features of both conditions had a positive biopsy, whereas slightly less than a quarter of those with polymyalgia rheumatica alone had a positive biopsy. TREATMENT
Corticosteroid treatment was given in the form of prednisolone in all except six patients, but different schedules were used. The most frequently used regime was to start with 20 mg daily for those with clinical evidence of giant cell arteritis, or 10 mg
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
Cases analysed
MYLES: SURVEY OF PR AND GIANT CELL ARTERITIS
233
FOLLOW-UP
Many patients rapidly became free of symptoms after starting steroids and the e.s.r. fell to normal. Corticosteroids were withdrawn in 14 patients when the disease appeared inactive (Table III). Three of these have relapsed and are now on steroids TABLE III CORTICOSTEROID TREATMENT: PRESENT STATE
Years of treatment
No. of patients
7 6 5 4 3 2 1
2 7 6 8 9 14 12
Withdrawn 14 (3 relapsed) (3 months-3$ years, mean 21 months)
No corticosteroid treatment 6
again. More often, after a period of steroid reduction, some symptoms returned and the e.s.r. rose, necessitating an increase to the previous dose level. Table III shows the number of years for which the remainder have been receiving corticosteroid treatment. Twenty-three, about a third, have had continuous treatment for four years or more. Analysis of the results has not shown any correlation between the presence or absence of arteritis, the starting dose of prednisolone and the subsequent length of treatment required. Table IV shows the number of cases in each treatment group who TABLE IV STARTING PREDNISOLONE DOSE
Treatment group
Starting dose mg/day
A. With evidence of arteritis Without evidence of arteritis B. C.
20 10 20-60 5-9
No. of patients
Dose increased
16 24 7 22
3 13
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
daily for those without evidence of arteritis. A small group started on higher doses up to 60 mg daily, and a further group started on a lower dose, 5-10 mg daily. The patients were seen at intervals of roughly four weeks, somewhat more frequently when on the higher doses. The steroid dose was regulated according to the symptoms, clinical findings, and the erythrocyte sedimentation rate, the aim being to keep the patient almost symptom free and the e.s.r. below 20 mm/lh. When this was achieved, the dose was decreased by 1 mg at each visit for those receiving 10 mg a day or less. In those taking higher doses, the reductions were somewhat greater and more frequent. Six patients were not given steroid treatment. In three, the patients and their family doctors gave a consistent story of polymyalgia rheumatica over a period of months with a considerably raised e.s.r. However, the symptoms, signs and e.s.r. were settling by the time they attended and continued to do so without treatment. Two patients had already been started on indomethacin and one on phenylbutazone with reasonably good results. This treatment was, therefore, continued.
234
RHEUMATOLOGY AND REHABILITATION VOL. XIV NO. 4
COMPLICATIONS
Complications starting after treatment has been initiated have been infrequent, and not necessarily related to giant cell arteritis. Three patients developed intermittent claudication, two had non-fatal myocardial infarction. Mild hepatitis occurred in one. Seven patients have died (Table V). These patients died at home, and the full details TABLE V CAUSE OF DEATH
Myocardial infarction Stroke Bronchopneumonia Pulmonary embolus Carcinoma of stomach Suicide Total
2 1 1 1 1 1 7
are not available, but there was no evidence that arteritis or corticosteroid treatment had been responsible. The only significant complication possibly related to treatment, was the development of marked spinal osteoporosis in seven with vertebral fractures in five. After a period of back pain, they all became virtually free of symptoms. Recurrence has been infrequent, and no patient has experienced continuing disability from this. DISCUSSION The results of this study are broadly similar to others, but show some interesting differences. In some patients the disease becomes inactive after a few years and corticosteroids can be stopped, but this is very variable. In others, prolonged treatment is necessary, and a few have remitted spontaneously over the course of a few months without treatment. Unfortunately there are no features from which a prediction can be made. The therapeutic results in this study have been satisfactory. No patient developed a severe complication of the disease after treatment was started and complications of treatment were infrequent. Many authorities recommend much higher starting doses of prednisolone such as 40-60 mg daily, not only for giant cell arteritis, but also for polymyalgia rheumatica (Fauchald et al., 1972; and Hamilton et ah, 1971). This study
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
required to have their dose increased. No-one starting on 20 mg or more of prednisolone required an increase. Three starting on 10 mg a day required an increase because of a suspicion that cranial arteritis had developed. In the lowest dose group, none of whom had clinical evidence of arteritis, over half had the dose increased, mainly because the polymyalgic symptoms were not controlled adequately. In five, as the dose was reduced, the e.s.r. rose slightly to between 30 and 40 mm/lh but the patients remained free of symptoms. The steroid dose was increased slightly but usually made no difference, Subsequently, provided that the e.s.r. did not rise further, a more gradual steroid reduction has been made. Seven developed a recurrence of pain and stiffness in girdle muscles, usually the shoulders, and sometimes only one, but the e.s.r. remained normal. A local steroid injection often remedied this situation. It should not be assumed however, that the disease was, therefore, localized. Intra-articular steroid administration is one method of giving systemic steroids.
MYLES: SURVEY OF PR AND GIANT CELL ARTERITIS
235
REFERENCES
COPEMAN, W.
S. C. and MYLES, A. B. (1970) British Encyclopaedia of Medical Practice: Medical Progress 1969/70, p. 368. London: Butterworths. DIXON, A. ST. J. (1969) "Polymyalgia Rheumatica". /. R. Coll. Physicians, 4, 55. FAUCHALD, P., RYGVOLD, O. and 0YSTESE, B. (1972) "Temporal Arteritis and Polymyalgia Rheumatica: Clinical and Biopsy Findings". Ann. intern. Med., 77, 845. FERNANDES-HERLIHY, L. (1971) "Polymyalgia Rheumatica". Semin. Arthritis Rheum., 3, 236. HAMILTON, C. R., SHELLEY, W. M. and TUMULTY, P. A. (1971) "Giant Cell Arteritis: Including Temporal Arteritis and Polymyalgia Rheumatica". Medicine, 50, 1. HAMRIN, B. (1972) "Polymyalgia Arteritica". Ada Med. Scand. Suppl. 533, 1. HAUSER, W. A., FERGUSON, R. A., HOLLEY, K. E. and KURLAND, L. T. (1971) "Temporal Arteritis in Rochester, Minnesota, 1951 to 1967". Mayo Clin. Proc, 46, 597. HEALEY, L. A. (1972) In Arthritis and Allied Conditions. Ed. J. L. Hollander. Philadelphia: Lea and Febiger. p. 885. PLOTZ, C. M. and SPIERA, M. D. (1969) "Polymyalgia Rheumatica". Bull. Rheum. Dis., 20, 578. GENERAL DISCUSSION (ABRIDGED) The evidence of abnormality of liver function was discussed. The speaker was not able to answer this in detail, because liver function tests had not been performed routinely until 1973, but he had noted that 5% of all his patients had slightly raised alkaline phosphatase levels. The speaker did not favour the use of ACTH as opposed to oral corticosteroids, partly because the side effects have disadvantages in the elderly, and also because it is more difficult to 'tailor' the dose accurately with adrenal stimulant. He did not think that the diagnosis of polymyalgia rheumatica could be ruled out if the patients developed one or two swollen joints in the course of their disease. One speaker had satisfactorily maintained some cases for as long as 25 years using as small a dose as 2.5 mg of prednisone per day.
Downloaded from http://rheumatology.oxfordjournals.org/ at University of California, Santa Barbara on September 7, 2015
suggests that a starting dose of 20 mg is adequate for most cases of giant cell arteritis and 10 mg for polymyalgia rheumatica. Most clinicians agree that a gradual reduction of the steroid dose is desirable. If, however, the starting dose is 60 mg, inevitably there will be a prolonged period during which the patient receives moderately high doses and it is this period of high dose treatment which gives rise to corticosteroid complications. A very rapid response to corticosteroid treatment is said to be typical of polymyalgia rheumatica and to have some diagnostic significance (Fernandes-Herlihy, 1971; and Healey, 1972). This rapid response has not been observed in this study, possibly because lower doses have been used. The six patients who subsequently proved to have a polyarthritis responded initially as well as the patients with polymyalgia rheumatica. Rheumatologists have frequently been worried that elderly patients with nonspecific symptoms and a raised e.s.r. from some other cause such as multiple myelomatosis, scattered bone metastases or chronic infection would be incorrectly diagnosed as polymyalgia rheumatica (Hamilton et ah, 1971; and Hauser et al, 1971). This has not occurred in any cases in this study in which the diagnostic errors were confined to patients with polyarthritis, affecting central joints only in the initial stages. It has also been said that the patient with polymyalgia has many complaints and a very high e.s.r. but that no physical abnormalities are found, whereas objective abnormalities particularly of shoulder movement, are present in most patients. Thesefindings,and careful attention to the history, in which marked morning stiffness is the most important feature, contribute to avoiding these diagnostic errors. The management of polymyalgia rheumatica is reasonably straightforward. Nevertheless, there remain some unsolved problems which include the patient with a persistently raised e.s.r. but no other evidence of active disease, and the patient with recurrent pain and stiffness who has a normal e.s.r.
