Hands on 1 s5

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POLYMYALGIA RHEUMATICA
Gillian Hosie, MB, ChB, FRCP(Glas), DA
General Practitioner, Glasgow
Past President of the Primary Care Rheumatology Society
Introduction
The presence of any 3 of these criteria gives a sensitivity of 92% and a specificity of 80% for the diagnosis of PMR.2 If The diagnosis of polymyalgia rheumatica (PMR) can present an additional criterion of a rapid response to oral steroid a difficult challenge in primary care as there are no specific therapy is added, this increases the sensitivity to 99%.2 diagnostic tests and diagnosis depends on having a high index of suspicion supported by history, examination and The Bird criteria are very useful and easy to apply in primary raised inflammatory markers. On the other hand, PMR care, although it should be noted that they do not yet have can be a very satisfying condition to treat in primary care universal acceptance in the rheumatological community as as patients often have a very dramatic response to steroid therapy and are very grateful for the prompt relief of their symptoms.
Clinical features of PMR
Epidemiology of PMR
Clinical features include:• stiffness and pain in shoulder and pelvic girdles The epidemiology of PMR varies according to different stud- ies with the incidence per 100,000 of the population aged • often systemic features of debility, weight loss, tiredness over 50 reported between 13 and 68. The incidence incr- eases with increasing age and there is an M:F ratio of 1:2.
• either: dramatic onset of pain and stiffness
or: insidious onset with less stiffness and pain and
Diagnostic criteria for PMR
more systemic features.
Stiffness is usually the main feature. Affected muscles Various authors have tried to set out diagnostic criteria for may be tender but the joints themselves are not usually PMR, all of which include a combination of age, clinical features and a raised erythrocyte sedimentation rate (ESR). Bird et al1 list six criteria: The diagnosis can be straightforward in patients who pres-ent with a sudden onset of symptoms associated with a TABLE 1. Criteria for diagnosis of polymyalgia
raised ESR. Some patients, however, present with a more rheumatica.1
insidious onset. In these patients systemic features may be more prominent, making the diagnosis much more difficult, and in these cases a wide range of differential diagnoses may need to be considered. These include: The presence of 3 or more of the above 6 criteria is ESR erythrocyte sedimentation rate; PMR polymyalgia rheumatica ������� ������� ���� �������� ���������� ������� ������� ����� ������ ���� ����������
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• fibromyalgia• shoulder problems (e.g. capsulitis or tears of the Straightforward cases of PMR can be managed successfully within primary care. Unless there are any contraindications patients should be started on 15 mg prednisolone per day, which should bring rapid relief of symptoms within a few Investigations for suspected PMR
There is no specific test for PMR, although most patients If this does not happen, reconsider your diagnosis.
will have a significantly raised ESR. If you suspect PMR it is worthwhile doing the following investigations: The dose of prednisolone should then be titrated down slowly according to symptoms rather than ESR. The dose TABLE 2. Investigations for suspected PMR.
can often be reduced fairly rapidly down to 10 mg per day and then reduced by 1 mg daily per 4–6 weeks over Investigation
6–12 months down to 5 mg daily. Then the dose should be reduced more slowly, aiming to have the patient off steroid therapy by around 2 years, although some patients may require a small dose of steroids for 3–4 years.
As they are weaned off corticosteroid therapy some patients may require a small dose of NSAIDs at this stage to reduce ESR erythrocyte sedimentation rate; FBC full blood count; LFT liver the muscle pain that sometimes occurs.
function tests; PMR polymyalgia rheumatica Remember that steroid therapy has risks and side-effects Although the ESR is usually significantly raised in PMR there are some patients in whom the ESR is normal or only a little • risk of osteoporosis – bone protection therapy should be
raised. In these patients and in those presenting with sys- initiated at the time of starting steroid therapy. Bone is temic features other causes of illness should be excluded.
lost rapidly in the early stages of steroid therapy so treatment should not be delayed while awaiting a Rheumatoid arthritis in an older patient may present with dual-energy x-ray absorptiometry (DEXA) scan. In those symptoms of PMR with shoulder girdle pain and stiffness. patients aged over 65 and in those with a previous frac- Be aware of the possibility of a polymyalgic presentation of ture a DEXA scan is not necessary as these patients are at considerable increased risk of osteoporosis and sub- • there are peripheral joint signs such as synovitis sequent fracture and require continued bone protection • there is a failure to respond to an adequate dose of • gastric irritation – gastro-protection may be required.
• the initial dose of steroid cannot be reduced without exacerbating the symptoms.
Relapses
If the diagnosis remains unclear, re-examine the patient Relapses are common in PMR and should be diagnosed on thoroughly with other diagnoses in mind. At this stage other clinical grounds rather than relying on ESR. They are usually investigations may be useful. Consider the following : caused by an overly rapid decrease in the dose of cortico-steroid and should be treated by increasing the dose again and then titrating down more slowly. If the steroid dose TABLE 3. Useful investigations to aid differential
cannot be reduced without exacerbating the symptoms diagnosis.
in PMR, or if the patient suffers several relapses, consider Investigation
another diagnosis – especially rheumatoid arthritis.
Relapses are commoner in the first 6–12 months of treat- ment and relapse rates are between 30 and 60%.
Prognosis
The long-term prognosis of PMR is good. Many patients can stop their steroid treatment at around 2 years, although some patients may require a small dose of steroids over several years. This is more likely to occur in females, and in ANA antinuclear antibodies; CPK creatine phosphokinase those who were older and with a higher ESR at diagnosis.
Referral to secondary care
• in whom the diagnosis is unclear• who have repeated relapses.
Most straightforward cases of PMR can be managed within REFERENCES
primary care. In others however diagnosis and manage- 1. Bird HA, Esselinckx W, Dixon AS, Mowat AG, Wood PHN. An eval- ment may be difficult and these cases should be referred to uation of criteria for polymyalgia rheumatica. Ann Rheum Dis a rheumatologist. Such patients may include those: • who have an inadequate response to steroids 2. Bird H. European diagnostic criteria for polymyalgia rheumatica. • in whom it is difficult to reduce the steroid dose with- 3. Samanta A, Kendall J. A fresh look at polymyalgia rheumatica. Rheumatology (Oxford) 2002;41:1455-6.
Brian Hazleman, MA, FRCP
Rheumatology Research Unit, Addenbrooke’s Hospital, Cambridge Past President of the British Society for Rheumatology The cardinal symptoms of PMR – prolonged morning stiffness jaw claudication, as the catastrophic sequela of untreated in the shoulder and pelvic girdles with an elevated ESR – are GCA is irreversible blindness. In such cases higher doses of generally straightforward. However PMR is essentially a diag- prednisolone are required with possible referral for temporal nosis of exclusion. As the differential diagnosis is extensive it is important to exclude more sinister conditions at the outset with screening blood investigations. PMR is exceedingly rare Education is paramount in PMR and patients should be below the age of 50 and occasionally the ESR is misleading, warned that treatment is usually necessary for at least 2 being normal or only slightly raised. years, with some requiring long-term maintenance therapy. In prednisolone-resistant cases response to steroid-sparing Of particular importance is the link between PMR and giant cell arteritis (also known as temporal arteritis). Many authori-ties believe the two represent opposite ends of the spectrum If diagnosis is proving difficult referral to a rheumatologist is of the same disease. In any patient who presents with PMR it prudent. However the treatment of a classic case of PMR is is mandatory to ask about headache, scalp tenderness and highly effective and one of the most satisfying in medicine. PMR case study
• Mrs X, aged 88, presented with a 2-week history of pain perhaps starting at a dose of 15 mg daily on the presump- and stiffness in her neck and shoulders and to a lesser tive diagnosis of PMR? On the other hand if the diagnosis extent around her hips. On examination she had a reason- turned out to be RA perhaps oral steroids would not be the able range of movement of her neck but some difficulty in raising her arms above her head. Past history included • Following discussion with a consultant rheumatologist col- osteoarthritis of the spine and hip with a hip replacement league we decided that the best option was to give a dose of intramuscular steroid and review at hospital out- • At this stage it was thought that she probably had PMR and investigations of ESR, CRP, FBC and rheumatoid factor were • Because of her on-going symptoms we also added a COX-2 undertaken. While awaiting the ESR result she was treated inhibitor and amitriptyline to her medication at this stage.
with analgesics rather than anti-inflammatory drugs • She responded well to the steroid injection with resultant because of a history of hiatus hernia and oesophageal decrease in stiffness and pain allowing her to attend her • Initial results showed a normal ESR of 5, raised CRP of 32 • Subsequent ESR was reported as 99 and to date the patient with a normal FBC, with rheumatoid factor still awaited.
has not developed any signs of synovitis. She is still await- • At this stage the patient’s stiffness and pain worsened to the extent that she was unable to dress herself and spent her nights sitting in a chair as she felt unable to lie down in This case study illustrates the difficulty we often have in bed because of her symptoms. She complained of feeling Primary Care in making a diagnosis between PMR and generally unwell, with loss of appetite.
• The differential diagnosis remained between PMR and RA. Signs and symptoms seemed more in favour of PMR but Confounding factors in this instance were: ESR was only 5. At this stage we received the rheumatoid factor result which was 382 (normal 0–22). Although care- • high level of rheumatoid factor ful examination of the patient showed no evidence of synovitis it was possible that this was a case of RA with a Often the situation only clarifies as time goes on. Perhaps this patient should have been treated with oral • This patient was miserable and needed treatment to allevi- ate her symptoms. Should we treat her with oral steroids, Suggested PMR audit
It is a useful exercise to take a retrospective look at the care and clinical course of patients within your practice with a diagnosis of PMR. Useful data to collect includes: • age at diagnosis• presenting symptoms and signs• initial ESR• starting dose of prednisolone• whether there was a dramatic response to treatment• whether patients received osteoporosis advice or treatment• number of relapses• total length of treatment with steroid therapy• referrals to secondary care• any laboratory tests such as thyroid function and rheumatoid factor.
An audit of PMR was undertaken in 1993 by GP members of the Primary Care Rheumatology Society. Data from 47 patients in primary care were analysed retrospectively. The M:F ratio was 17:30 and the mean age at diagnosis was 69.6 years.
Questionnaire
• 18 (38%) suffered 2 or more (1 patient had 6) Mean initial starting dose of prednisolone Audits undertaken today should show a lower initial starting dose of prednisolone (around 15 mg) and a much higher percen-tage of patients counselled regarding, or treated for, osteoporosis. Only 38% of patients in the audit above were noted to have been given treatment or counselling about the risk of osteoporosis. The new Guidelines on corticosteroid induced osteoporosis1 advise starting bone protective therapy at the same time as starting steroid therapy in all patients aged over 65 or with ahistory of fragility fracture.
REFERENCE
1. Glucocorticoid-induced osteoporosis: guidelines for prevention and treatment. London: Royal College of Physicians of London; 2002.
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