Polymyalgia rheumatica M35.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

PMR

History
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Bruce 1888

Definition
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Etiologically unexplained inflammatory disease of the connective tissue and the musculature with strong painfulness of the musculature, especially pelvic and shoulder girdle pain such as sore muscles and associated morning stiffness (usually >1h), feeling of illness and weight loss, night sweat, increased BSG (>40mm/h) and/or symptoms of depression.

Polymyalgia rheumatica develops in close association with giant cell arteritis (association in 50-80% of cases) or it is considered its minus variant. The disease can also occur as a paraneoplastic syndrome.

Occurrence/Epidemiology
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Incidence in persons > 50 years (Federal Republic of Germany): 300-700/100,000 inhabitants/year.

Manifestation
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Mostly after the 50th LJ and predominantly occurring in women.

Clinical features
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Remember! Characteristic triad: muscular pain, fall reduction (at least 40 mm/hour), anaemia.

Persistent pain for at least 4 weeks with morning stiffness in the neck, shoulder girdle (70-95%) or pelvic girdle (50-70%), usually lasting > 60 minutes. Oedema on the extensor sides of the extremities (back of the hand, back of the foot) and subdeltoidal bursitis are common.

Often associated (20% of cases) is giant cell arteritis (arteritis cranialis).

Laboratory
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Pronounced acute phase changes are typical: increase of C-reactive protein (most sensitive parameter; increased in > 80% of cases), alpha 1- and alpha 2-globulins and SPA. Characteristically, the muscle enzymes in serum are mostly normal.

Diagnosis
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Polymyalgia rheumatica: ACR/EULAR classification criteria (2012)

Inclusion criteria: =/> 50 years, new shoulder girdle pain on both sides, CFP and/or BSG increased. Additionally:

  • Morning stiffness =/>45 min (2 points)
  • Absence of rheumatoid factor CCP (2 points)
  • Pain/stiffness pelvic girdle (1 point)
  • Absence of other joint manifestations (1 point)

Plus sonography:

  • 1 shoulder joint+1 hip joint (1 point)
  • Both shoulder joints (1 point)

A score of =/>5 points has a sensitivity and specificity 70% for the diagnosis PMR.

Differential diagnosis
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Therapy
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  • Early glucocorticoids such as prednisone (e.g. Decortin) initial 15-20 mg/day p.o. After 14 days gradual reduction, maintenance dose over 1-2 years at 5-10 mg/day p.o. (rule of thumb: continue therapy for at least 1 year beyond symptom relief).
  • Note: Higher doses should not be used: risk of recurrence is increased, danger of masking giant cell arteritis.
  • In case of failure of glucocorticoid monotherapy, trial with additional administration of Methotre1xate (10 to 20 mg/week). Non-steroidal anti-inflammatory drugs are only slightly pain-relieving.
  • Low-dose ASA (100mg/day) prevents ischemic complications in possibly associated giant cell arteritis.
  • Osteoporosis prophylaxis: Estrogen-progestin substitution, e.g. Estraderm TTS = 17-β-estradiol with a daily drug release of 0.05 mg combined with progestin (e.g. Kliogest N). Continuous use after menopause.
  • Sufficient supply of calcium and vitamin D (e.g. frubiase calcium forte, ossofortin).
  • In therapy-refractory cases: cyclophosphamide; alternative: tocilizumab.
  • In cases of bioptically proven giant cell arteritis in complications (deterioration of visual acuity) higher glucocorticoid doses (initial 100-500 mg/day i.v. with gradual dose reduction).

Note(s)
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Remember! Always looking for tumors!

Literature
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  1. Barilla-LaBarca ML et al (2002) Polymyalgia rheumatica/temporal arteritis: recent advances. Curr Rheumatol Rep 4: 39-46
  2. Buttgereit F et al (2016) Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review JAMA 315:2442-2458.
  3. Bruce W (1888) Senile rheumatic gout. Br Med J 2: 811-813

  4. Dejaco C et al (2017) Giant cell arteritis and polymyalgia rheumatica: current challenges and opportunities. Nat Rev Rheumatol 13:578-592.

  5. González-Gay MA et al. (2017) Polymyalgia rheumatica. Lancet. 390:1700-1712.
  6. Matteson EL et al. (2017) Polymyalgia rheumatica. Ann Intern Med 166:ITC65-ITC80. https://www.ncbi.nlm.nih.gov/pubmed/28460395
  7. Panzner M et al (1992) Typical course of a polymyalgia rheumatica. Z Hautkr 67: 997-1000
  8. Salvarani C et al (2003) Treatment of refractory polymyalgia rheumatica with infliximab: a pilot study. J Rheumatol 30: 760-763
  9. Weyand CM et al (2003) Giant-cell arteritis and polymyalgia rheumatica. Ann Internal Med 139: 505-515
  10. Weyand CM et al (2003) Medium and large-vessel vasculitis. N Engl J Med 349: 160-169

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020