Elsevier

The Lancet

Volume 390, Issue 10103, 7–13 October 2017, Pages 1700-1712
The Lancet

Seminar
Polymyalgia rheumatica

https://doi.org/10.1016/S0140-6736(17)31825-1Get rights and content

Summary

Polymyalgia rheumatica is an inflammatory disease that affects the shoulder, the pelvic girdles, and the neck, usually in individuals older than 50 years. Increases in acute phase reactants are typical of polymyalgia rheumatica. The disorder might present as an isolated condition or in association with giant cell arteritis. Several diseases, including inflammatory rheumatic and autoimmune diseases, infections, and malignancies can mimic polymyalgia rheumatica. Imaging techniques have identified the presence of bursitis in more than half of patients with active disease. Vascular uptake on PET scans is seen in some patients. A dose of 12·5–25·0 mg prednisolone daily or equivalent leads to rapid improvement of symptoms in most patients with isolated disease. However, relapses are common when prednisolone is tapered. Methotrexate might be used in patients who relapse. The effectiveness of biological therapies, such as anti-interleukin 6, in patients with polymyalgia rheumatica that is refractory to glucocorticoids requires further investigation. Most population-based studies indicate that mortality is not increased in patients with isolated disease.

Introduction

Polymyalgia rheumatica is an inflammatory disorder characterised by severe pain and stiffness affecting the shoulders and proximal aspects of the arms bilaterally. Pain and stiffness is also common in the neck. Less frequently these symptoms affect the pelvic girdle and the proximal aspects of the thighs. Patients have morning stiffness that lasts more than 45–60 min and non-specific symptoms such as fatigue and malaise. Increases in acute phase reactants (ie, erythrocyte sedimentation rate and C-reactive protein) is a characteristic feature of the disease.1

Bruce first described this condition in 1888.2 Polymyalgia rheumatica—also known as secondary fibrositis, periarthrosis humeroscapularis, peri-extra-articular rheumatism, myalgic syndrome of the aged, pseudo-polyarthrite rhizomelique, and anarthritic rheumatoid disease—was first reported in the 1940s. The term polymyalgia rheumatica was introduced by Barber in 1957.2

Section snippets

Epidemiology: incidence, genetic components, and environmental factors

Polymyalgia rheumatica is a common disease in elderly patients, but rarely arises in individuals younger than 50 years.3 More than two-thirds of patients are women. Incidence increases progressively with age in both sexes until the age of 80 years.4 The highest incidence is in people older than 65 years, with a peak in the 70–79 year age group.3 Polymyalgia rheumatica is most common in Scandinavian countries.5, 6 The mean annual incidence of polymyalgia rheumatica in individuals aged older than

Pathophysiology

Arthroscopic studies have shown the presence of mild synovitis in the proximal joints (mainly in the shoulders) of patients with polymyalgia rheumatica. The inflammatory infiltrate found in the shoulder synovial membranes and other affected joints was composed mainly of macrophages and CD4 T lymphocytes.19 This mild synovitis does not fully explain the musculoskeletal manifestations and the diffuse pain in the periarticular structures. In view of the prominent inflammatory involvement of

Clinical manifestations

The cardinal clinical feature of polymyalgia rheumatica is pain with restricted range of motion and stiffness of the shoulder girdle in patients older than 50 years. Patients often complain of pain and stiffness in the upper arms, neck, pelvic girdle, hips, and thighs.31 These clinical manifestations are usually bilateral. The onset of symptoms is often rapid, generally over a few days and in some cases acutely overnight. Symptoms are associated with aching and early morning stiffness in the

Laboratory abnormalities

Rises in acute phase reactants is a typical feature in patients with polymyalgia rheumatica. An erythrocyte sedimentation rate of 40 mm or higher per h is regarded as a classification criterion by some34, 43, 44 (panel). Other authors recommend an erythrocyte sedimentation rate of more than 30 mm per h or a C-reactive protein concentration of more than 6 mg/L as a classification criterion for polymyalgia rheumatica diagnosis.45 The 2012 European League Against Rheumatism (EULAR) and the

Polymyalgia rheumatica and giant cell arteritis

Giant cell arteritis is a large blood vessel vasculitis that occurs in elderly people.1, 3 This condition is uncommon in people younger than 50 years and incidence of the disease peaks in the 70–79-year age group.3, 53 Similar to polymyalgia rheumatica, giant cell arteritis has a strong genetic association with the HLA-class region and the disease has a high prevalence in Scandinavian countries and in individuals of Scandinavian descent.3, 13, 14

Giant cell arteritis and polymyalgia rheumatica

Mimics of polymyalgia rheumatica

Symptoms of polymyalgia rheumatica are non-specific and many disease mimics exist. Clinicians should be aware of the most common conditions that mimic polymyalgia rheumatica, including rheumatoid arthritis, shoulder osteoarthritis, polyarticular calcium pyrophosphate deposition disease, rotator cuff disease, and adhesive capsulitis (frozen shoulder).

Additional information about conditions that mimic polymyalgia rheumatica is shown in the table and appendix.32, 37, 38, 39, 40, 63, 64, 65, 66, 67

Diagnostic investigations and classification criteria

Several sets of criteria for the classification and diagnosis of polymyalgia rheumatica have been proposed (panel). The criteria have common features, such as older age (≥50 years), typical involvement of shoulders, and the presence of raised acute phase reactants.34, 38, 43, 44, 45 The criterion of older age (>50 years) was proposed by Chuang and colleagues,34 Healey,44 and the 2012 EULAR/ACR criteria.38, 78 Bilateral clinical involvement that predominantly involves the shoulders with morning

Management of polymyalgia rheumatica

Oral prednisolone is the mainstay of treatment in polymyalgia rheumatica.89, 90, 91

A prednisolone dose ranging between 12·5 and 25 mg per day is sufficient to yield rapid improvement of polymyalgia features in most cases.28, 51, 89, 92, 93 However, the dose might be individualised according to each patient. Although the biological activity of glucocorticoids is certainly not only bodyweight dependent, generally a heavy person (>80 kg) without risk factors for side-effects such as diabetes

Relapses

Relapses are defined as the recurrence of polymyalgia rheumatica symptoms that are generally associated with elevation rise in erythrocyte sedimentation rate and C-reactive protein concentration. Persistently high concentrations of C-reactive protein and interleukin 6 were significantly associated with an increased risk of relapse in patients with polymyalgia rheumatica.120 This association was especially evident for patients with persistently high concentrations of interleukin 6 during the

Comorbidities and outcomes

Most patients with polymyalgia rheumatica have side-effects associated with glucocorticoid therapy that depend on the duration of treatment and the cumulative dose.126 Nevertheless, overall cardiovascular mortality does not seem increased in this disease.127, 128 Although patients with isolated disease were reported to be at increased risk for peripheral artery disease,129 and polymyalgia rheumatica was associated with higher risk for stroke in Chinese individuals,130 a large registry study of

Controversies in polymyalgia rheumatica management

No unanimous consensus has been reached on the optimum dose of glucocorticoids to prescribe for patients with isolated polymyalgia rheumatica. Experts from the 2015 EULAR and ACR collaborative initiative92, 93 proposed an initial prednisolone dose between 12·5–25·0 mg daily or equivalent. This group of experts recommends a single dose of prednisolone.92, 93 However, we believe that an initial divided dose might be more effective to improve stiffness and pain in the morning when the patient is

Future directions and research questions

Management recommendations should take into account patient perspectives. One way to achieve this is to focus on the severity rather than on the duration of morning stiffness, especially at the time of diagnosis because patients usually find it difficult to measure the exact duration of stiffness.

In addition to a definitive biomarker for diagnosis of polymyalgia rheumatica, better markers of disease activity are needed. These biomarkers could be serological and imaging techniques that might be

Search strategy and selection criteria

We searched the Cochrane Library and PubMed for reports published in English from database inception until Feb 28, 2017, using the term “polymyalgia rheumatica” AND each heading used in our Seminar (eg, “epidemiology”; “genetics”; “pathophysiology”…). We largely selected publications from the past 10 years, but did not exclude commonly referenced and highly regarded older publications. We also searched the reference lists of articles identified by this search strategy and selected those we

References (135)

  • EL Matteson et al.

    Glucocorticoids for management of polymyalgia rheumatica and giant cell arteritis

    Rheum Dis Clin North Am

    (2016)
  • C Salvarani et al.

    Polymyalgia rheumatica and giant-cell arteritis

    N Engl J Med

    (2002)
  • MA Gonzalez-Gay et al.

    Epidemiology of giant cell arteritis and polymyalgia rheumatica

    Arthritis Rheum

    (2009)
  • S Raheel et al.

    Epidemiology of polymyalgia rheumatica 2000–2014 and examination of incidence and survival trends over 45 years: a population based study

    Arthritis Care Res

    (2016)
  • P Boesen et al.

    Giant cell arteritis, temporal arteritis, and polymyalgia rheumatica in a Danish county: a prospective investigation, 1982–1985

    Arthritis Rheum

    (1987)
  • P Elling et al.

    Synchronous variations of the incidence of temporal arteritis and polymyalgia rheumatica in different regions of Denmark; association with epidemics of Mycoplasma pneumoniae infection

    J Rheumatol

    (1996)
  • C Schaufelberger et al.

    Epidemiology and mortality in 220 patients with polymyalgia rheumatica

    Br J Rheumatol

    (1995)
  • MF Doran et al.

    Trends in the incidence of polymyalgia rheumatic over a 30 year period in Olmsted County, Minnesota, USA

    J Rheumatol

    (2002)
  • C Salvarani et al.

    Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in northern Italy

    Arthritis Rheum

    (1991)
  • MA Gonzalez-Gay et al.

    The spectrum of polymyalgia rheumatica in northwestern Spain: incidence and analysis of variables associated with relapse in a 10 year study

    J Rheumatol

    (1999)
  • CS Crowson et al.

    Contemporary prevalence estimates for giant cell arteritis and polymyalgia rheumatic

    Semin Arthritis Rheum

    (2017)
  • M Yates et al.

    The prevalence of giant cell arteritis and polymyalgia rheumatica in a UK primary care population

    BMC Musculoskelet Disord

    (2016)
  • L Boiardi et al.

    Relationship between interleukin 6 promoter polymorphism at position -174, IL-6 serum levels, and the risk of relapse/recurrence in polymyalgia rheumatica

    J Rheumatol

    (2006)
  • MA Gonzalez-Gay et al.

    IL-6 promoter polymorphism at position-174 modulates the phenotypic expression of polymyalgia rheumatica in biopsy-proven giant cellarteritis

    Clin Exp Rheumatol

    (2002)
  • P Peris

    Polymyalgia rheumatica is not seasonal in pattern and is unrelated to parvovirus b19 infection

    J Rheumatol

    (2003)
  • RL Rhee et al.

    Infections and the risk of incident giant cell arteritis: a population-based, case-control study

    Ann Rheum Dis

    (2017)
  • R Meliconi et al.

    Leukocyte infiltration in synovial tissue from the shoulder of patients with polymyalgia rheumatica. Quantitative analysis and influence of corticosteroid treatment

    Arthritis Rheum

    (1996)
  • C Salvarani et al.

    Polymyalgia rheumatica: a disorder of extraarticular synovial structures?

    J Rheumatol

    (1999)
  • C Salvarani et al.

    Proximal bursitis in active polymyalgia rheumatica

    Ann Intern Med

    (1997)
  • F Cantini et al.

    Shoulder ultrasonography in the diagnosis of polymyalgia rheumatica: a case-control study

    J Rheumatol

    (2001)
  • F Cantini et al.

    Inflammatory changes of hip synovial structures in polymyalgia rheumatica

    Clin Exp Rheumatol

    (2005)
  • C Salvarani et al.

    Cervical interspinous bursitis in active polymyalgia rheumatica

    Ann Rheum Dis

    (2008)
  • C Salvarani et al.

    Lumbar interspinous bursitis in active polymyalgia rheumatica

    Clin Exp Rheumatol

    (2013)
  • MA Gonzalez-Gay

    The clinical implication of cervical interspinous bursitis in the diagnosis of polymyalgia rheumatica

    Ann Rheum Dis

    (2008)
  • F Kreiner et al.

    Increased muscle interstitial levels of inflammatory cytokines in polymyalgia rheumatica

    Arthritis Rheum

    (2010)
  • MA González-Gay et al.

    Giant cell arteritis and polymyalgia rheumatica: an update

    Curr Rheumatol Rep

    (2015)
  • M Samson et al.

    Th1 and Th17 lymphocytes expressing CD161 are implicated in giant cell arteritis and polymyalgia rheumatica pathogenesis

    Arthritis Rheum

    (2012)
  • KS van der Geest et al.

    Disturbed B cell homeostasis in newly diagnosed giant cell arteritis and polymyalgia rheumatica

    Arthritis Rheumatol

    (2014)
  • C Masson et al.

    Polymyalgia rheumatica and giant cell arteritis

  • MA González-Gay et al.

    Polymyalgia rheumatica in biopsy proven giant cell arteritis does not constitute a different subset but differs from isolated polymyalgia rheumatica

    J Rheumatol

    (1998)
  • TY Chuang et al.

    Polymyalgia rheumatica: a 10-year epidemiologic and clinical study

    Ann Intern Med

    (1982)
  • MA Gonzalez-Gay et al.

    Fever in biopsy-proven giant cell arteritis: clinical implications in a defined population

    Arthritis Rheum

    (2004)
  • MA González-Gay et al.

    Polymyalgia rheumatica vs rheumatoid arthritis in the elderly

    Arch Intern Med

    (1997)
  • CT Pease et al.

    Polymyalgia rheumatica can be distinguished from late onset rheumatoid arthritis at baseline: results of a 5-yr prospective study

    Rheumatology

    (2009)
  • B Dasgupta et al.

    2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative

    Arthritis Rheum

    (2012)
  • C Salvarani et al.

    Distal musculoskeletal manifestations in polymyalgia rheumatica

    Arthritis Rheum

    (1998)
  • MA Gonzalez-Gay et al.

    Diagnostic approach in a patient presenting with polymyalgia

    Clin Exp Rheumatol

    (1999)
  • C Salvarani et al.

    Distal extremity swelling with pitting edema in polymyalgia rheumatica. Report of nineteen cases

    Arthritis Rheum

    (1996)
  • DJ McCarty et al.

    Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome

    JAMA

    (1985)
  • HA Bird et al.

    An evaluation of criteria for polymyalgia rheumatica

    Ann Rheum Dis

    (1979)
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