Elsevier

The Lancet

Volume 381, Issue 9860, 5–11 January 2013, Pages 63-72
The Lancet

Seminar
Polymyalgia rheumatica

https://doi.org/10.1016/S0140-6736(12)60680-1Get rights and content

Summary

Polymyalgia rheumatica is a chronic, inflammatory disorder of unknown cause that affects people over age 50 years. Classic symptoms include pain and long-term morning stiffness of the neck, shoulders, hips, upper arms, and thighs. Although markers of inflammation are often raised, no specific laboratory test exists for the disorder and the diagnosis is based on clinical assessment. Provisional classification criteria were published in April, 2012, by a collaborative initiative of the European League Against Rheumatism and the American College of Rheumatology. Several other disorders can mimic polymyalgia rheumatica. In particular, clinical manifestations can be difficult to differentiate from other forms of inflammatory arthritis such as spondyloarthritis and rheumatoid arthritis. Imaging studies such as ultrasonography and MRI typically show a predominantly periarticular inflammatory process. A subset of patients has an associated inflammatory vasculopathy affecting large arteries (giant cell arteritis). The standard treatment is low-dose glucocorticoids, which provide symptomatic relief for most patients. However, disease relapses are common, and treatment with glucocorticoids is associated with substantial morbidity. Improved understanding of disease pathogenesis might allow for more targeted immunotherapy.

Introduction

Polymyalgia rheumatica is a chronic, inflammatory disorder of unknown cause. It is not typically seen in people under age 50 years. Clinically, the disorder is characterised by pain and long-term morning stiffness affecting the neck, shoulders, hips, upper arms, and thighs. There are no specific diagnostic tests; diagnosis is typically based on clinical presentation and evidence of systemic inflammation. Ultrasonography of the shoulders and hips is a useful imaging modality in the initial assessment and often shows findings of bursitis, tenosynovitis, or synovitis. The differential diagnosis is broad and clinicians need to consider several disorders that can mimic the disease. In particular, late-onset spondyloarthritis and rheumatoid arthritis can present with polymyalgic symptoms. A well known association exists between polymyalgia rheumatica and giant cell arteritis, a systemic, granulomatous vasculitis that affects the aorta and its branches.

Section snippets

Epidemiology

711 000 people aged 50 years or older in the USA have polymyalgia rheumatica, according to one estimate.1 From population-based incidence studies in Olmsted County, MN, USA, the lifetime risk of developing the disease has been estimated at 2·43% for women and 1·66% for men.2 Polymyalgia rheumatica occurs almost exclusively in people older than age 50 years, and the mean age of onset is about 73 years. In general, its incidence increases with advancing age,3 and varies by geographical region.

Aetiology and pathogenesis

The cause of polymyalgia rheumatica is unknown. Epidemiological studies suggest that both genetic and environmental factors might be important in disease pathogenesis. Although familial aggregation is rare, it has been described.13 Several studies have reported an association between the disorder and specific polymorphisms in genes related to immune regulation.11 Genetic polymorphisms associated with disease risk or severity include intercellular adhesion molecule 1, interleukin 1 receptor

Polymyalgia rheumatica and giant cell arteritis

Polymyalgia rheumatica and giant cell arteritis share many similarities, including age at onset, female predominance, and similar geographical distribution, suggesting that they might represent different types of the same disease. Clinically, 40–60% of patients with giant cell arteritis have polymyalgia rheumatica symptoms at diagnosis.35 Additionally, 16–21% of patients with polymyalgia rheumatica have giant cell arteritis.35 Both diseases are characterised by a chronic inflammatory state,

Clinical features

Patients present with characteristic pain and stiffness affecting the shoulder girdle, hip girdle, and neck muscles. The pain can radiate to the elbows or knees. Patients often report morning stiffness lasting 30 min or longer and worsening stiffness after periods of rest. Symptoms are usually bilateral. Constitutional symptoms, including fatigue, malaise, anorexia, weight loss, and fever (usually low-grade), can occur in 40–50% of patients.40 In view of the association between polymyalgia

Diagnosis

Polymyalgia rheumatica is a clinical diagnosis. At initial presentation, to think of it as a polymyalgia syndrome is helpful, and a careful history and physical examination are crucial in distinguishing it from other disorders that mimic it (panel 1).52 Several sets of diagnostic criteria have been proposed (panel 2).40, 53, 54, 55 An age cutoff is used in most of these systems. Other common criteria are the presence of bilateral shoulder girdle and hip girdle aching, morning stiffness, and a

Treatment and follow-up

The standard treatment is low-dose glucocorticoids, which characteristically induce rapid resolution of symptoms. There have been no controlled clinical trials assessing the efficacy of glucocorticoids compared with placebo. A systematic review of studies of treatment has been reported.77 Although the high degree of variation between studies precluded the researchers from calculating pooled estimates, they were able to make several treatment recommendations.77 A set of guidelines for management

Prognosis

Response to treatment is assessed on the basis of symptoms and markers of inflammation. Despite therapy, relapses are common and arise in roughly 50% of patients.94, 95 Factors that have been associated with relapses or long-term glucocorticoid therapy are a higher initial dose of glucocorticoids,94 rapid glucocorticoid taper,94 and female sex.31, 80 Persistently raised CRP and interleukin 6 might also be associated with risk of relapses.95 Several studies have shown increased circulating

Future perspectives

Disease-specific biomarkers for polymyalgia rheumatica are currently unavailable. Additionally, heterogeneity exists between patients and some have an underlying inflammatory vasculopathy. Therefore, diagnostic biomarkers that allow prompt and accurate diagnosis are eagerly awaited. Discovery and validation of such markers will require collaborative, prospective studies in which patients are assessed and treated in a standardised way, such as that recently undertaken by the EULAR-ACR

Search strategy and selection criteria

Using the term “polymyalgia rheumatica”, we searched Medline, Embase, Web of Science, and Scopus. We largely selected publications from the past 5 years, but did not exclude widely referenced and highly regarded older publications. The reference lists of articles identified by the search strategy were also reviewed, and articles judged relevant were included. Case reports, scientific abstracts, and articles that included only patients with giant cell arteritis were excluded. Review articles are

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