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JoonasRautavaara

Polymyalgia Rheumatica

Essentials

  • Among the vast number of patients with myalgia or arthralgia, those whose symptoms can be effectively treated with low-dose glucocorticoids should be identified.
  • Patients with typical polymyalgia rheumatica who respond well to glucocorticoids can be treated in primary care.
  • Suspicion of giant cell arteritis (previously known as temporal arteritis) Giant Cell (Temporal) Arteritis, atypical clinical features and non-satisfactory response to treatment are indications for consulting specialized care.

General remarks

  • Polymyalgia rheumatica is a chronic inflammatory disease of unknown aetiology.
  • Muscular pain and stiffness as well as systemic symptoms characterize the clinical picture.
  • Giant cell arteritis Giant Cell (Temporal) Arteritis can be demonstrated in 10-20% of patients with polymyalgia rheumatica.

Epidemiology

  • The incidence of polymyalgia rheumatica is approximately 500 cases per million people.
  • There is a female preponderance.
  • The disease is encountered almost solely in patients over 50 years of age. Peak incidence is at the age of 70.

Symptoms

  • Symmetrical pain, stiffness and restricted motion in the neck-shoulder region and upper arms (in 75-99%) as well as pelvic region (in 50-90%).
    • Asymmetrical pain such as pain in one shoulder only, is not typical of polymyalgia.
  • The pain is accentuated during the night and in the morning and is associated with prolonged morning stiffness normally lasting at least one hour.
  • General symptoms: fatigue, weight loss, loss of appetite, fever, depression
  • Onset is usually fairly acute with symptoms typically reaching their peak within a few days or a week. The patient is often able to tell the exact day when the symptoms started.
  • Because of the association between polymyalgia rheumatica and giant cell arteritis it is important to recognize the clinical picture that includes headache, jaw claudication, tenderness of the scalp, visual disturbances and claudication in the limbs. These symptoms should trigger diagnostic exploration with giant cell arteritis Giant Cell (Temporal) Arteritis in mind.

Findings

  • Painful restriction of movement of the shoulder and hip joints in nearly all patients
  • No tender spots as in fibromyalgia, but there may be tenderness on palpation in the upper arms and thighs.
  • In some cases there may be arthritides of the wrists or fingers and inflammation of finger flexor tendons resembling rheumatoid arthritis. In elderly people, rheumatoid arthritis may have a polymyalgic onset.
  • Ultrasonography may sometimes show effusion in the subdeltoid bursa and the sheath of the long head of the biceps tendon and synovitis in hip joints.

Investigations

  • The following are necessary laboratory investigations at the diagnostic stage: ESR, CRP and basic blood count with platelet count.
  • For the differential diagnostics, other laboratory tests may also be necessary; see sections Diagnosis and Differential diagnosis.

Diagnosis

  • Diagnosis based on clinical findings is often sufficient: age over 50, typical clinical picture, increased ESR and CRP and rapid subjective response to a glucocorticoid (prednisolone or prednisone, 12.5-25 mg/day,) within 3 days.
  • ESR and CRP are goodscreening tests when polymyalgia rheumatica is suspected as the cause of pain (cf. fibromyalgia Fibromyalgia).
    • ESR is typically clearly increased, > 40 mm/h (in 80%), but it may also be lower.
    • In a small proportion of patients, ESR may be normal but in such cases CRP is typically increased.
    • In about 1-2% only, both ESR and CRP are normal; therefore, if inflammatory markers are not increased from the patient's previous levels at all, another disease causing the symptoms should primarily be sought.
  • Laboratory findings demonstrate typical features of a generalized inflammation.
    • In addition to increased ESR and CRP, the findings include mild to moderate normocytic anaemia and thrombocytosis.
    • Plasma alkaline phosphatase may be increased (in 30-60% of patients) but it is usually rapidly normalized after beginning glucocorticoid treatment.
    • Rheumatoid factor and anti-citrulline antibodies are usually negative.
    • Creatine kinase concentration is normal (cf. polymyositis Myositis).
  • Ultrasonography of the shoulders or hips can be considered case by case as part of the initial investigations.
    • It may often show bilateral subdeltoid bursitis, tenosynovitis of the long head of the biceps or synovitis in the hip joint.
    • Soft tissue inflammation may also be visualized by MRI or PET scanning. The latter investigation may show subclinical inflammation in the walls of large arteries.
  • Temporal artery ultrasonography or biopsy (video Temporal Artery Biopsy) is only indicated if symptoms or signs suggest the possibility of giant cell arteritis Giant Cell (Temporal) Arteritis.
  • It is not considered necessary to try to exclude cancer in patients with polymyalgia rheumatica with typical symptoms and responding well to treatment.

Differential diagnosis

Treatment Bisphosphonates for Steroid Induced Osteoporosis

  • Polymyalgia rheumatica is treated with a low-dose glucocorticoid that typically eliminates or essentially relieves the symptoms within a few days.
  • The initial dose of prednisolone(or prednisone) is 12.5-25 mg/day.
    • If the symptoms do not subside within 3-5 days, the accuracy of the diagnosis should be suspected.
    • Higher glucocorticoid doses are seldom needed.
  • The initial dose should be continued for 2 to 4 weeks. After that, the dose may be gradually tapered while monitoring symptoms and CRP (CRP is a more sensitive and rapidly-reacting parameter than ESR).
    • The dose should be reduced in such a way that the dose is 10-15 mg/day 4-8 weeks after the treatment was begun.
    • Later on, the dose should be reduced more slowly, such as by 2.5 mg/day every 3 months.
    • Should the disease be reactivated when glucocorticoid dosage is reduced, the dose should again be increased to the preceding effective dose. After 4-8 weeks, an attempt should be made to reduce the dose again to the level where the relapse occurred.
  • If a relapse is suspected, inflammatory markers (ESR, CRP) should be defined before increasing the glucocorticoid dose.
    • If inflammatory markers are normal or at the patient's previous level, the possibility of symptoms of other causes must be kept in mind.
    • Reactivation of polymyalgia with perfectly normal inflammatory markers is possible but rare.
  • Glucocorticoid treatment should be continued for at least one year, typically 12-24 months. More rapid withdrawal significantly increases the risk of disease reactivation and ultimately increases the cumulative glucocorticoid dose.
  • The aim should always be to withdraw glucocorticoids completely. For a small proportion of patients, however, it must, due to repeated relapses, be continued for several years.
  • If withdrawal is repeatedly unsuccessful or tried after several years of glucocorticoid therapy, the possibility of glucocorticoid-induced adrenal suppression must be kept in mind. In such cases, the morning cortisol level should be measured 48 hours after withdrawing glucocorticoids; see Pharmacological Glucocorticoid Treatment.
  • Glucocorticoid therapy eliminates the symptoms but the duration of the disease is not reduced.
  • If the response to the glucocorticoid remains insufficient or if the drug is harmful (osteoporosis, diabetes), methotrexate may be combined with it. If methotrexate introduction is considered, specialized care, either a rheumatology unit or internal diseases unit, should be consulted.
  • In the USA, the interleukin-6 inhibitor sarilumab has been approved for the treatment of polymyalgia rheumatica in patients with insufficient response to glucocorticoids or if glucocorticoid treatment cannot be withdrawn as planned. In Europe, sarilumab is not yet officially indicated in the treatment of polymyalgia.
  • Another IL-6 inhibitor, tocilizumab, has also been used to treat severe cases of polymyalgia rheumatica but it is not officially indicated in the treatment of the disease.
  • Prophylaxis against osteoporosis should be started concomitantly with the decision to start glucocorticoid treatment.
    • See Osteoporosis.
    • Sufficient calcium and vitamin D intake must always be guaranteed.
    • Since the patients are elderly and it is likely that the glucocorticoid therapy is needed for a lengthy period, it is in practice almost always necessary to start a bisphosphonate or some other specific anti-osteoporotic medication as well.
    • Discontinuation of the anti-osteoporotic medication may be considered when the glucocorticoid therapy ends.
  • Physical activity and maintenance of good muscle fitness should be encouraged as soon as the worst symptoms subside. In elderly people, muscle fitness is rapidly affected by physical inactivity.

Levels of care and indications for consulting specialized care

  • Most cases of polymyalgia rheumatica can be diagnosed and treated in primary health care.
  • Consulting specialized care is recommended in cases listed below.
    • There are atypical clinical symptoms, such as stiffness mainly distal to the elbows or knees.
    • There are clear synovites associated with the disease.
    • The response to daily doses of no more than 25 mg prednisolone (or prednisone) remains poor or there are repeated relapses at a dose of 10 mg/day or more.
    • The patient develops symptoms suggestive of giant cell arteritis Giant Cell (Temporal) Arteritis.

Follow-up

  • The aim is to find the smallest dose of glucocorticoid that is still effective. The dose is defined individually in each patient by monitoring the inflammatory activity of the disease.
  • During glucocorticoid therapy, laboratory tests and an assessment by a physician are recommended at 4-8-week intervals at the beginning and later at 8-12-week intervals.
  • In addition to the clinical assessment, CRP provides a good picture of the inflammatory activity of the disease.
  • During follow-up visits, attention should also be paid to any adverse effects of glucocorticoid therapy (see Pharmacological Glucocorticoid Treatment) as well as to any associated diseases, such as osteoporosis and diabetes.
  • Laboratory tests to be monitored include ESR, CRP, basic blood count with platelet count, and plasma creatinine, glucose and electrolytes.

Prognosis

  • Glucocorticoids are highly effective in the treatment of polymyalgia, and if the clinical picture does not improve with a glucocorticoid, another diagnosis should be sought.
  • Polymyalgia rheumatica is typically a disease that will resolve. The aim is to ultimately withdraw all medication.
  • Reactivation of the disease after withdrawing glucocorticoids is very common, and as many as 43% of patients have at least one reactivation requiring increased glucocorticoid dosage within one year of diagnosis.
  • In practice, glucocorticoid treatment is often prolonged and according to studies, about half of patients still take a glucocorticoid at 2 years and one in four at 5 years from diagnosis.
  • The disease has a tendency to relapse. The patient is usually able to identify the symptoms and readily seek medical advice.

    References

    • Lundberg IE, Sharma A, Turesson C, et al. An update on polymyalgia rheumatica. J Intern Med 2022;292(5):717-732 [PubMed]
    • González-Gay MA, Matteson EL, Castañeda S. Polymyalgia rheumatica. Lancet 2017;390(10103):1700-1712 [PubMed]
    • Dejaco C, Singh YP, Perel P ym. 2015 Recommendations for the management of polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis 2015;74(10):1799-807. [PubMed]
    • Dasgupta B, Borg FA, Hassan N, et al. BSR and BHPR guidelines for the management of polymyalgia rheumatica. Rheumatology (Oxford) 2010;49(1):186-90 [PubMed]