Review article
Update on polymyalgia rheumatica

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Abstract

Polymyalgia rheumatica is an inflammatory disease of unknown etiology affecting individuals aged fifty years and older, mainly of Caucasian ethnicity. Polymyalgia rheumatica is associated with giant cell arteritis more frequently than expected by chance alone. In both conditions, females are affected two to three times more often than males. The clinical hallmark manifestations of polymyalgia rheumatica are aching and morning stiffness in the shoulder girdle and often in the pelvic girdle and neck. Serum inflammatory markers are typically elevated, while the most consistent abnormal finding on imaging studies is bursitis in the symptomatic areas. A dramatic response to glucocorticoids is characteristic of polymyalgia rheumatica. Many patients are able to discontinue glucocorticoids six months to two years after the onset of clinical symptoms, but some patients may require longstanding glucocorticoid treatment. Glucocorticoid-sparing agents may be helpful in patients with chronic relapsing courses and those at high risk of glucocorticoid-related adverse events.

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.

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