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
SeminarPolymyalgia rheumatica
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
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