Review articleUpdate on polymyalgia rheumatica☆
Introduction
Polymyalgia rheumatica (PMR) is an inflammatory disease characterized by aching and prolonged morning stiffness in the shoulder girdle and often in the pelvic girdle and neck. The first case of PMR was described 1888 [8], but the term “polymyalgia rheumatica” was coined by Barber in 1957 [3].
Section snippets
Epidemiology
PMR typically affects people 50 years of age or older, mostly Caucasians. Its incidence increases progressively with age, peaking between 70 and 80 years of age [60]. Women are affected two to three times more often than men [31]. A population-based study from Olmsted County, Minnesota, US, estimated the prevalence of PMR to be as high as 1 case for 133 persons over 50 years [67]. In Italy, a study based in Reggio Emilia revealed that average annual incidence rates of PMR from 1981 to 1985 were
Classification criteria
In clinical practice, the diagnosis of PMR rests on its characteristic manifestations, raised inflammatory markers, a dramatic response to GC, and exclusion of other disorders that may present with similar features [62]. For classification purposes, a number of criteria have been proposed over time, including those by Chuang [16], Healey [34], and Bird [4] (Table 1). However, none of these criteria has been properly validated or received universal acceptance. More recently, new classification
Etiology and pathogenesis
The etiology of PMR is still obscure. A seasonal pattern with higher incidence rates in winter has been reported [54], suggesting that an infectious agent could act as trigger. The odd case of PMR simultaneously affecting both spouses would also fit with an infectious etiology [57]. However, a bacterial etiology seems unlikely because procalcitonin levels are not raised in PMR [71]. In addition, no specific microorganism has consistently been linked to PMR [56], [74].
The higher incidence of PMR
Clinical manifestations
The hallmark manifestations of PMR are aching and prolonged morning stiffness in the shoulder girdle and often in the neck and pelvic girdle [62]. Nearly all patients develop shoulder pain, while the neck and pelvic girdle are involved in approximately 70% and 50% of patients, respectively [60], [62]. The pain is inflammatory in nature i.e. worse at night and radiates distally toward the elbows and knees. Shoulder and hip pain may initially be unilateral, but as a rule becomes soon bilateral.
Laboratory tests
Inflammatory markers, such as the ESR and CRP, are elevated in most patients with PMR [62]. Other laboratory abnormalities associated with active inflammation may include normochromic normocytic anemia, thrombocytosis, hypoalbuminemia, and raised α-2 globulin proteins [73]. However, in a small number of cases the ESR and, rarely, the CRP may be normal despite clinically active disease [22], [78]. The CRP is more specific than the ESR in reflecting inflammation and should thus preferentially be
Association between polymyalgia rheumatica and giant cell arteritis
PMR and GCA are both inflammatory diseases of the elderly that occur together more frequently than expected by chance [60], [62]. Circa 16–21% of PMR patients develop GCA; conversely, 40–60% of GCA patients have features of PMR [20], [52], [68]. PMR may appear before, together with, or after GCA [62]. Mild arteritis has been demonstrated using PET in 1/3 of patients with isolated PMR (i.e. PMR without clinical evidence of GCA) [5], while about 4% of patients with isolated PMR have histological
Glucocorticoids
Glucocorticoids remain to date the cornerstone of treatment of PMR. There is evidence that initial prednisone doses higher than 10 mg daily are associated with fewer relapses and shorter glucocorticoid requirements than lower dosages [35]. On the other hand, starting prednisone doses higher than 15 mg daily have been linked to higher cumulative glucocorticoid doses and more frequent glucocorticoid-related adverse events [35]. In practice, most patients with PMR respond to a prednisone dosage of 15
Differentials of polymyalgia rheumatica
A number of diseases can potentially present with clinical features mimicking PMR [55]. PMR mimickers include elderly-onset rheumatoid arthritis, late-onset seronegative spondyloarthropathy, myositis, fibromyalgia, calcium pyrophosphate disease, viral myalgia, bilateral rotator cuff syndrome, bilateral adhesive capsulitis, osteoarthritis of the cervical spine and shoulders, tumors including multiple myeloma, hypothyroidism, and neurological disorders such as Parkinson's disease.
Elderly-onset
Conclusions
Various criteria have been proposed to classify PMR, but in practice the diagnosis of PMR still eminently rests on its characteristic clinical features, elevated inflammatory markers, and a dramatic response to glucocorticoids. Imaging findings may support the clinical diagnosis of PMR, but their role in the individual diagnostic process is not fully clarified. Glucocorticoids remain the mainstay of treatment of PMR, but immunosuppressants may be useful in relapsing cases and in those at high
Learning points
- •
Various criteria have been proposed to classify PMR, but in practice the diagnosis of PMR still eminently rests on its characteristic clinical features, elevated inflammatory markers, and a dramatic response to glucocorticoids.
- •
Imaging findings may support the clinical diagnosis of PMR, but their role in the individual diagnostic process is not fully clarified.
- •
Glucocorticoids remain the mainstay of treatment of PMR, but immunosuppressants may be useful in relapsing cases and in those at high
Conflict of interests
We declare no conflicts of interest.
References (78)
- et al.
Interleukin-1RN gene polymorphisms in elderly patients with rheumatic inflammatory chronic conditions: association of IL-1RN*2/2 genotype with polymyalgia rheumatica
Hum Immunol
(2009) - et al.
C-reactive protein in giant cell (cranial, temporal) arteritis
Ophthalmology
(1980) - et al.
Genetic markers of disease susceptibility and severity in giant cell arteritis and polymyalgia rheumatica
Semin Arthritis Rheum
(2003) Long-term follow-up of polymyalgia rheumatica: evidence for synovitis
Semin Arthritis Rheum
(1984)- et al.
Hypothalamic–pituitary–adrenocortical axis function in patients with polymyalgia rheumatica and giant cell arteritis
Semin Arthritis Rheum
(2003) - et al.
The role of infectious agents in the pathogenesis of vasculitis
Best Pract Res Clin Rheumatol
(2008) - et al.
Polymyalgia rheumatica and giant-cell arteritis
Lancet
(2008) Vasculitis: a collection of pearls and myths
Rheum Dis Clin North Am
(2007)- et al.
Intercellular adhesion molecule-1 gene polymorphisms in isolated polymyalgia rheumatica
J Rheumatol
(2002) Myalgic syndrome with constitutional effects: polymyalgia rheumatica
Ann Rheum Dis
(1957)
An evaluation of criteria for polymyalgia rheumatica
Ann Rheum Dis
Repetitive 18-fluorodeoxyglucose positron emission tomography in isolated polymyalgia rheumatica: a prospective study in 35 patients
Rheumatology (Oxford)
Giant cell arteritis, temporal arteritis, and polymyalgia rheumatica in a Danish county. A prospective investigation, 1982–1985
Arthritis Rheum
Relationship between interleukin 6 promoter polymorphism at position -174, IL-6 serum levels, and the risk of relapse/recurrence in polymyalgia rheumatica
J Rheumatol
Senile rheumatic gout
BMJ
Imaging of polymyalgia rheumatica: indications on its pathogenesis, diagnosis and prognosis
Rheumatology (Oxford)
Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study
Ann Rheum Dis
Inflamed shoulder structures in polymyalgia rheumatica with normal erythrocyte sedimentation rate
Arthritis Rheum
Shoulder ultrasonography in the diagnosis of polymyalgia rheumatica: a case–control study
J Rheumatol
Prednisone plus methotrexate for polymyalgia rheumatica: a randomized, double-blind, placebo-controlled trial
Ann Intern Med
Treatment of refractory polymyalgia rheumatica with etanercept: an open pilot study
Arthritis Rheum
Successful use of tocilizumab in polymyalgic onset biopsy positive GCA with large vessel involvement
BMJ Case Rep
Polymyalgia rheumatica: a 10-year epidemiologic and clinical study
Ann Intern Med
BSR and BHPR guidelines for the management of polymyalgia rheumatica
Rheumatology (Oxford)
2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative
Ann Rheum Dis
Interleukin-6 in serum of patients with polymyalgia rheumatica and giant cell arteritis
Br J Rheumatol
The clinical features of elderly-onset rheumatoid arthritis. A comparison with younger-onset disease of similar duration
Arthritis Rheum
Definition of remission and relapse in polymyalgia rheumatica: data from a literature search compared with a Delphi-based expert consensus
Ann Rheum Dis
Evidence for synovitis in active polymyalgia rheumatica: sonographic study in a large series of patients
J Rheumatol
Current understanding and management of giant cell arteritis and polymyalgia rheumatica
Expert Rev Clin Immunol
Genetic epidemiology. Giant cell arteritis and polymyalgia rheumatica
Arthritis Res
Giant cell arteritis without clinically evident vascular involvement in a defined population
Arthritis Rheum
Polymyalgia rheumatica and biopsy-proven giant cell arteritis exhibit different HLA-DRB1* associations
J Rheumatol
The spectrum of conditions mimicking polymyalgia rheumatica in Northwestern Spain
J Rheumatol
IL-6 promoter polymorphism at position -174 modulates the phenotypic expression of polymyalgia rheumatica in biopsy-proven giant cell arteritis
Clin Exp Rheumatol
Epidemiology of giant cell arteritis and polymyalgia rheumatica
Arthritis Rheum
Tocilizumab ameliorates clinical symptoms in polymyalgia rheumatica
J Rheumatol
Laboratory investigations useful in the evaluation of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA)
Clin Exp Rheumatol
Treatment of polymyalgia rheumatica: a systematic review
Arch Intern Med
Cited by (34)
Polymyalgia Rheumatica
2017, The Heart in Rheumatic, Autoimmune and Inflammatory Diseases: Pathophysiology, Clinical Aspects and Therapeutic ApproachesPolymyalgia rheumatica
2017, The LancetCitation Excerpt :Patients with polymyalgia rheumatica and low erythrocyte sedimentation rates have similar clinical characteristics and disease course to those with high erythrocyte sedimentation rates.46,47 However, those with low erythrocyte sedimentation rates are usually younger with a lower frequency of fever, weight loss, and anaemia than those with erythrocyte sedimentation rates of 40 mm or higher per h.46 C-reactive protein might be more specific and more sensitive than the erythrocyte sedimentation rate for the detection of inflammation and assessing disease activity.48,49 Whether the measurement of C-reactive protein concentration is more useful than erythrocyte sedimentation rate for the initial diagnosis of polymyalgia rheumatic is unclear.
Predictors of complete 24-month remission and flare in patients with polymyalgia rheumatica
2023, Clinical and Experimental MedicineImmune complex-mediated neutrophil activation in patients with polymyalgia rheumatica
2023, Rheumatology (United Kingdom)Challenges in the diagnosis of polymyalgia rheumatica and related giant cell arteritis
2023, Expert Review of Clinical Immunology
- ☆
The authors have no disclosures.