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INTRODUCTION — Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized clinically by aching and morning stiffness in the shoulders, hip girdle, and neck. It can be associated with giant cell (temporal) arteritis (GCA), and the two disorders may represent different manifestations of a shared disease process.
As a result of this association, there are two main issues that arise when PMR is considered:
●How is the diagnosis established and distinguished from other disorders that can produce similar symptoms?
●Does the patient also have GCA?
The major features of PMR, with emphasis on clinical manifestations and diagnosis, will be reviewed here. Issues related to the treatment of PMR and to GCA are discussed separately. (See "Treatment of polymyalgia rheumatica" and "Clinical manifestations of giant cell (temporal) arteritis" and "Diagnosis of giant cell (temporal) arteritis" and "Treatment of giant cell (temporal) arteritis".)
EPIDEMIOLOGY — Polymyalgia rheumatica (PMR) is almost exclusively a disease of adults over the age of 50, with a prevalence that increases progressively with advancing age. The peak incidence of PMR occurs between ages 70 and 80 .
PMR is relatively common. The lifetime risk of PMR is second only to rheumatoid arthritis (RA) as a systemic rheumatic disease in adults . Women are affected two to three times more often than men. Cases of familial aggregation are recognized .
The annual incidence varies geographically and is highest in Scandinavian countries and in people of northern European descent . In Europe, for example, the incidence rates for the population ≥50 years are highest in northern regions (113 per 100,000 per year in Norway) and much lower in southern areas (13 per 100,000 per year in Italy) [5,6]. In Olmsted County, Minnesota, where the population is of predominantly Scandinavian descent, the incidence is 59 per 100,000 per year . PMR appears to be uncommon in Asian, African-American, and Latino populations, though all racial and ethnic groups may be affected.
Polymyalgia rheumatica and giant cell (temporal) arteritis — PMR is two to three times more common than giant cell (temporal) arteritis (GCA), and occurs in about 50 percent of patients with GCA . The percentage of patients with PMR who experience GCA at some point varies widely in reported series, ranging from roughly 5 to 30 percent [1,9,10]. However, we have observed that a figure of 10 percent seems most consistent with clinical practice. The two disorders may not be active synchronously.
PATHOGENESIS — The cause of polymyalgia rheumatica (PMR) is unknown; both environmental and genetic factors appear to play a role . In addition to the frequent clinical association between PMR and giant cell (temporal) arteritis (GCA), there is also evidence of a pathogenetic similarity between the two disorders. (See "Pathogenesis of giant cell (temporal) arteritis".)
Both PMR and GCA are associated with specific alleles of human leukocyte antigen (HLA)-DR4. There is also a sequence polymorphism within the hypervariable region of the HLA-DRB1 gene that maps to the antigen-binding cleft of the HLA-DR molecules, suggesting an important role for antigen selection and presentation. Patients with GCA and PMR share this sequence polymorphism, which is not found in patients with rheumatoid arthritis (RA) . Studies of the immunogenetics of PMR contain somewhat limited numbers and need validation in larger patient cohorts.
Although pathological findings in PMR are less prominent than in GCA, a mild synovitis characterized by macrophages and CD4+ T lymphocytes has been described in synovial membranes from involved joints . Evidence of subclinical arterial inflammation, including activated dendritic cells, interleukin (IL)-1, and IL-6 can be detected in the temporal arteries of some patients with PMR who have no clinical evidence of GCA . However, unlike GCA, interferon gamma-producing T cells are not prominent.
Other immunologic abnormalities shared by PMR and GCA include a similar distribution of circulating CD4+ T cell subsets; in both conditions, Th17 cells are increased and Treg cells are decreased . The proinflammatory cytokine IL-6 is elevated in the peripheral blood of patients with PMR and is thought to be responsible for its constitutional manifestations .
Some studies of PMR and GCA have suggested a cyclical pattern in incidence and seasonal variation, indicating a possible environmental infectious trigger . Results of studies of several viruses as potential triggers have been inconclusive.
Symptoms and signs — Polymyalgia rheumatica (PMR) is characterized by aching and morning stiffness in the shoulders, hip girdle, neck, and torso in patients over the age of 50 (figure 1) [11,17-19]. Symptoms are usually symmetric. It is typically a recent, discrete change in musculoskeletal symptoms that prompts the patient to seek medical attention; on occasion, the onset can be startlingly abrupt.
The following features can be seen:
●Stiffness – All patients with PMR experience morning stiffness, which lasts at least 30 minutes, frequently longer. Stiffness at the shoulders and hips may cause trouble with dressing (eg, hooking a bra in the back, donning a shirt or jacket, or pulling on stockings). In some patients, stiffness may be so severe that there is difficulty turning over in bed at night or arising from bed in the morning. The ‘gel’ phenomenon, stiffness after inactivity, is often notably severe in PMR and may result in recrudescent symptoms during the day if the patient is sedentary. An inability to actively abduct shoulders past 90 degrees because of stiffness is a typical finding.
●Pain – Shoulder pain is more common at presentation (70 to 95 percent of patients) than hip and neck involvement (50 to 70 percent), and may dominate the presenting symptomatology. Pain is worse with movement and may interfere with sleep.
●Synovitis and bursitis – About half of patients exhibit distal musculoskeletal manifestations. Clinical synovitis may occur in peripheral joints, such as the knees, wrists, and metacarpophalangeal joints [20-23]. Distal synovitis is usually mild, nonerosive, and may be asymmetric.
Synovitis and bursitis are thought to be the causes of the discomfort and stiffness. However, because the shoulders and hips are surrounded by large muscles, small amounts of synovitis in these joints cannot be detected on physical examination.
●Swelling and tenosynovitis – Some patients develop swelling and pitting edema of the hands, wrists, ankles, and top of the feet (picture 1) . The edema usually occurs with other signs of PMR, but can be the presenting symptom. It appears to represent tenosynovitis and synovitis in regional structures (see 'Differential diagnosis' below). Tenosynovitis can also cause carpal tunnel syndrome, which occurs in approximately 10 to 15 percent of patients with PMR .
●Decreased range of motion – There may be decreased active and passive range of motion of the shoulders, neck, and hips. A characteristic finding is frank limitation of active abduction at the shoulders (picture 2).
●Muscle tenderness – Despite the implications of the name "polymyalgia," muscle tenderness is not a prominent feature, and what tenderness there may be about the shoulders is more likely due to synovial or bursal inflammation than muscle involvement.
●Subjective weakness – Although weakness may be reported by patients, careful motor examination will demonstrate normal muscle strength. This exam may be difficult if there is significant pain with active motion.
●Systemic signs and symptoms – Patients with PMR may experience nonspecific systemic signs or symptoms, including malaise, fatigue, depression, anorexia, weight loss, and low-grade fever. However, the high spiking fever that may accompany giant cell (temporal) arteritis (GCA) is rare in patients who have only PMR. (See "Clinical manifestations of giant cell (temporal) arteritis".)
Laboratory findings — The erythrocyte sedimentation rate (ESR) can be normal, mildly elevated, or even markedly elevated:
●The characteristic laboratory finding in PMR (and GCA) is an elevated ESR, with occasional patients having values that can exceed 100 mm/h. In one study, for example, 20 percent of patients had ESR values over 104 mm/hr .
●Alternatively, studies have found that approximately 5 to 20 percent of patients may have ESRs less than 40 mm/h [26-28].
Some evidence suggests that an elevated serum C-reactive protein (CRP) level is more common than a high ESR. In two reports from the same group, an elevated ESR (greater than 30 mm/h) was noted in 92 to 94 percent of patients at the time of diagnosis of PMR, while 99 percent of such patients had an increased serum CRP level (greater than 5 mg/L) [25,29]. In one of these studies, 9 of the 10 patients with a normal ESR at time of diagnosis had an elevated CRP (median 14 mg/L, range 7 to 47 mg/L), although mean values tended to be lower in these patients than in the group as a whole (19 versus 68 mg/L). In another study, only 1.5 percent of patients had an ESR and CRP within normal limits (defined as less than 20 mm/h and 10 mg/L, respectively) at the time of diagnosis .
Other laboratory findings are nonspecific:
●A normocytic anemia may be present, and the white blood cell and platelet counts are usually normal. In some patients, however, the platelet count is increased as part of a general inflammatory response.
●Serologic tests, such as antinuclear antibodies, rheumatoid factor, and cyclic citrullinated peptide antibodies are typically negative. It should be remembered that the prevalence of positive assays for antinuclear antibody and rheumatoid factor rise with age.
●Some patients experience an increase in liver enzymes, especially alkaline phosphatase, although these abnormalities are more common in patients with GCA than PMR alone.
Imaging — Routine radiographs of inflamed joints do not show abnormalities in patients with PMR. By comparison, routine imaging by other techniques (such as ultrasonography [US], magnetic resonance imaging [MRI], and positron emission tomography [PET]) can detect findings suggestive of underlying inflammation in patients with PMR . Over time, the synovitis of PMR is never erosive. However, such advanced imaging is usually not required.
MRI and ultrasound — MRI and US have demonstrated that PMR involves synovial inflammation, with a predilection for extraarticular synovial structures . In a case-control study of 57 patients with untreated PMR and 114 control individuals with bilateral shoulder aching and stiffness, US detected bilateral subacromial/subdeltoid bursitis in nearly all patients with PMR but few controls (96 versus 4 percent) . There was excellent correlation between US and MRI.
Imaging studies of the shoulders frequently identifies glenohumeral synovitis and biceps tenosynovitis. Trochanteric bursitis and synovitis at the hips are common . Additionally, MRI has shown evidence for tenosynovitis at the hands in patients with PMR, as well as for interspinous bursitis in the cervical and lumbar spine [34-36]. MRI and other imaging studies have suggested a potential role for capsular inflammation and involvement of the enthesis (the insertions of tendons, ligaments, and capsules) in the pathogenesis of PMR [37,38].
Other studies have confirmed and extended the prevalent findings on US in PMR [30,39,40], and it has been proposed that US demonstration of subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis in at least one shoulder, and synovitis or trochanteric bursitis in at least one hip, could be included among possible classification criteria for the diagnosis of PMR [41,42]. While it is an imaging hallmark of PMR, subdeltoid/subacromial bursitis is not specific, and is seen in patients with rheumatoid arthritis (RA) and other shoulder pathology . (See "Bursitis: An overview of clinical manifestations, diagnosis, and management", section on 'Subacromial bursitis'.)
PET scanning — PET scanning has no proven clinical value in the care of patients with PMR. PET scanning can identify bursitis, but has been of particular interest in the investigation of underlying vascular involvement . In a series of 13 patients with suspected PMR who underwent 18-fluorodeoxyglucose PET scanning prior to initiation of glucocorticoid therapy, 12 had increased uptake in vascular regions as assessed by visual inspection of the images and by a computer-aided quantitative analysis . Another study of PET scanning demonstrated increased uptake in the subclavian arteries in one-third of 35 patients with PMR, though less intense than that observed in GCA . While increased uptake of radiolabeled glucose is suggestive of large-vessel vasculitis, histologic examination of the aorta and large arteries is obviously not feasible.
The implications of these PET scan findings are unclear, although they indicate that subclinical inflammation of the great vessels is present in PMR. Further study is needed to determine whether PET scanning has any prognostic or therapeutic implications. It is rare for patients with PMR without clinical features of GCA to develop large-vessel complications such as stenoses or aneurysms.
EVALUATION — The initial evaluation of patients with suspected polymyalgia rheumatica (PMR) includes a complete history and physical, as well as selected laboratory testing. We suggest particular attention to the following:
●Careful questioning concerning the presence of truncal, shoulder, and hip stiffness and pain; morning stiffness (at least 30 minutes); joint pain and swelling; muscle pain and weakness; disruption of sleep; and systemic symptoms (eg, fever and weight loss). Symptoms that could suggest giant cell (temporal) arteritis (GCA), such as new headache, jaw claudication, scalp tenderness, visual change, fever, or cough, must be sought. (See 'Symptoms and signs' above and 'Evaluation for GCA' below.)
●A review of systems to elicit a history that may suggest another disorder to explain the symptoms. (See 'Differential diagnosis' below.)
●Complete musculoskeletal examination to assess range of motion of the axial joints (shoulders and hips), peripheral joints, neck, and low back; the presence of signs of inflammatory or degenerative arthritis, tendonitis or bursitis; and muscle strength and tenderness. Careful examination of passive range of motion of the shoulders and hips with all muscles relaxed is often greater than active motion of these areas. Patients should be examined for evidence of tender points that could suggest fibromyalgia and other findings that may support an alternative diagnosis, such as hypothyroidism or Parkinson disease. The temporal arteries should be examined for signs of inflammation. (See 'Symptoms and signs' above and 'Differential diagnosis' below and 'Evaluation for GCA' below.)
●Laboratory testing should include a complete blood count (CBC) and platelet count, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels. Additional testing may be required depending upon the patient's symptoms, physical findings, and other diagnoses under consideration. (See 'Laboratory findings' above and 'Differential diagnosis' below.)
●The evaluation includes an assessment of the response to low-dose glucocorticoids. Symptoms are generally 50 to 70 percent better within three days of being started on prednisone (10 to 20 mg/day) and almost all patients respond completely within two weeks of initiation of therapy.
●If there is diagnostic uncertainty, magnetic resonance imaging (MRI) or ultrasound (US) may be helpful to assess whether there is underlying bursitis or other evidence for inflammation. Plain radiographs are not required, unless another diagnosis that may be confirmed or excluded by such studies is being considered. (See 'Imaging' above.)
Diagnosis of PMR — There is no pathognomonic test or established diagnostic criteria for polymyalgia rheumatica (PMR). We use the presence of all of the following empirically formulated criteria for the clinical diagnosis of PMR, in whom another disease to explain the findings is not present [20,45-47]:
●Age 50 years or older at disease onset.
●Proximally and bilaterally distributed aching and morning stiffness (lasting at least 30 minutes or more) persisting for at least two weeks. The stiffness should involve at least two of the following three areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs.
●Erythrocyte sedimentation rate (ESR) (Westergren) ≥40 mm/h.
●Rapid resolution of symptoms with low-dose glucocorticoids. Symptoms are generally 50 to 70 percent better within three days in patients with PMR started on prednisone at a dose of 10 to 20 mg/day. The lack of response to initial therapy strongly suggests an alternative diagnosis.
Establishing the diagnosis is more difficult in patients with atypical presentations. These include patients aged 40 to 50 years, asymmetric symptoms, and an ESR less than 40 mm/h [18,26-28]:
●In younger patients, inflammatory rheumatic diseases that may mimic PMR warrant careful consideration of alternative diagnoses (see 'Differential diagnosis' below). However, occasional patients in practice may be seen with typical PMR beginning in their late 40s.
●Asymmetric symptoms may be present especially early in disease, beginning in one shoulder or hip, but soon become bilateral in most patients .
●As noted above, a small percentage of patients with bona fide PMR have lower ESRs at the time of diagnosis. Although there is less experience with use of serum C-reactive protein (CRP) testing to establish the diagnosis of PMR, it may be reasonable to substitute an elevated serum CRP (above the upper limit of normal for the testing laboratory) as a criterion for PMR if the ESR is less than 40 mm/h, because of the higher sensitivity of CRP elevation than an abnormal ESR (see 'Laboratory findings' above). Thus, a normal or modestly elevated ESR by itself should not deter consideration of a diagnosis of PMR in the appropriate clinical setting, but the combination of a normal ESR and a normal CRP renders the diagnosis much less likely.
Provisional EULAR/ACR classification criteria — Classification criteria have been proposed by the European League Against Rheumatism and the American College of Rheumatology (EULAR/ACR) as a research tool to identify patients with PMR . It is important to emphasize that the proposed criteria are designed as a research tool to distinguish PMR from other conditions, and are not applicable to the diagnosis of the individual patient.
The EULAR/ACR criteria include three required and four additional criteria. Required criteria include age ≥50 years, bilateral shoulder aching, and an abnormal CRP or ESR. A scoring algorithm was developed for four additional criteria: morning stiffness ≥45 minutes (2 points), pain or limited range of motion at the hip (1 point), absence of rheumatoid factor or anti-cyclic citrullinated protein antibody (2 points), and absence of peripheral joint pain (1 point). The presence of all three required criteria, plus a score of ≥4 of the additional criteria, had 78 percent specificity and 68 percent sensitivity for discriminating PMR from a comparator cohort. The imperfect performance of these criteria relative to the EULAR/ACR classification criteria for other diseases underscores the challenging nature of categorizing patients as having PMR.
One study compared the performance of the EULAR/ACR criteria with other previously published classification criteria in 136 patients with PMR and 149 controls with rheumatoid arthritis and other inflammatory arthritides . The EULAR/ACR criteria were the most sensitive (93 percent), although they were not the most specific (82 percent). The inclusion of shoulder and hip ultrasound in the EULAR/ACR criteria increased specificity to 91 percent.
Evaluation for GCA — Patients with clinically "pure" PMR lack the classic findings of giant cell (temporal) arteritis (GCA), eg, temporal artery tenderness, headache, jaw pain, visual symptoms, and other evidence of ischemia such as arm claudication. In the absence of symptoms or signs, we do not routinely perform a temporal artery biopsy, since a positive result is rare [21,49].
GCA may become manifest at any point during the clinical course of PMR, including at presentation, during treatment with glucocorticoids, or after treatment has been discontinued. Risk factors for the clinical expression of GCA in patients with PMR have not been clearly established. In patients on 20 mg of prednisone/day, the persistence of constitutional symptoms (such as fever), or significantly elevated acute phase reactants could indicate underlying GCA.
At every follow-up visit, we recommend monitoring for symptoms or physical findings suggestive of GCA in patients with PMR. Evaluation, including biopsy, should be performed if symptoms of GCA develop, even if patients are on glucocorticoids. The yield of temporal artery biopsy does not appear to be affected by previous glucocorticoid therapy . The biopsy may be negative if there is large artery involvement by GCA, in which case diagnosis could require vascular imaging . (See "Diagnosis of giant cell (temporal) arteritis".)
DIFFERENTIAL DIAGNOSIS — The most challenging consideration in the differential diagnosis of polymyalgia rheumatica (PMR) is seronegative rheumatoid arthritis (RA). A variety of other disorders can usually be easily distinguished clinically from PMR [11,17,18]. Limited laboratory testing may be helpful depending on the specific clinical presentation.
●Rheumatoid arthritis – Patients with RA classically have symmetric polyarthritis of the small joints of the hands and feet, which is persistent and only partially responsive to low doses of prednisone. These findings differ from PMR, in which fewer joints are swollen, and swelling subsides completely in response to low-dose prednisone. (See "Clinical manifestations of rheumatoid arthritis" and "Use of glucocorticoids in the treatment of rheumatoid arthritis".)
There is, however, considerable overlap between PMR and seronegative RA in older adults who present with symmetric synovitis. Such patients have also been referenced under the rubrics of “late-onset rheumatoid arthritis” and “elderly-onset rheumatoid arthritis” . The following are illustrative:
•In a report of 159 patients with PMR, seronegative RA, or features of both, who were followed for at least 30 months, recurrent synovitis was observed in 57 patients . Twenty of the 57 patients had both an episode of symptoms and signs consistent with PMR and another that looked like RA. All patients responded to prednisone. Twenty-four patients later developed giant cell (temporal) arteritis (GCA): 19 had been given the diagnosis of PMR and 5 of RA. (See "Diagnosis and differential diagnosis of rheumatoid arthritis".)
•A prospective study including 116 patients found that after one year of follow-up, the diagnosis was changed to RA in 20 percent of patients initially diagnosed with PMR .
•Although one study found that acute phase reactants (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP], interleukin [IL]-6) were higher in patients with PMR and responded more dramatically to treatment with glucocorticoids than patients with RA , others have identified no clinical or laboratory findings that reliably differentiated PMR from RA, apart from vigilant clinical follow-up [54,56]. Measurement of anti-citrullinated protein antibodies may assist in the recognition of an older adult with seropositive RA .
We initially treat those patients in whom PMR cannot be clinically distinguished from seronegative RA with glucocorticoids in the doses used for PMR and carefully monitor the clinical response. It may take several months to differentiate PMR from rheumatoid disease with certainty. Patients who respond well are continued on this treatment regimen, and glucocorticoids are tapered accordingly. Treatment for RA should be instituted if symptoms of arthritis respond incompletely or evolve in a pattern more characteristic of RA during initial glucocorticoid therapy. (See "Treatment of polymyalgia rheumatica".)
●RS3PE syndrome – The RS3PE syndrome  refers to Remitting Seronegative Symmetrical Synovitis with Pitting Edema, also described as the puffy edematous hand syndrome or distal extremity swelling with pitting edema. Patients with RS3PE, who are frequently over 50 years of age, typically present with the sudden onset of polyarthritis associated with prominent distal symptoms, and are rheumatoid factor-negative . The dominant finding on magnetic resonance imaging (MRI) and ultrasound (US) is an often exuberant extensor tenosynovitis, with lesser amounts of flexor tenosynovitis and synovitis of the metacarpophalangeal and proximal interphalangeal joints . Symptoms and signs may respond briskly to treatment with low-dose glucocorticoids and accordingly may be construed as having a variant of PMR with prominent edema [23,61]. In other patients with this syndrome, a more specific disease may evolve, such as RA. An association with solid tumors and hematologic disorders has also been reported, though, in clinical practice, such an occurrence is rare [62,63].
●Multifocal local musculoskeletal disease – The symptoms and signs at the shoulders in PMR may be similar to those that result from subacromial/subdeltoid bursitis without PMR, or from rotator cuff tendinitis. Local pathology of this sort is common in older adults, may be bilateral, and, when combined with symptoms and signs of degenerative disc disease at the cervical spine and/or osteoarthritis at the hips, could suggest PMR. However, in such patients, morning stiffness is brief, constitutional symptoms are absent, and the acute phase reactants are not elevated. (See 'MRI and ultrasound' above.)
●Bone disease – The differential diagnosis of diffuse achiness in an older adult includes osseous disease. Multiple myeloma can present with bone pain and an elevated ESR, thereby mimicking PMR. Such patients can be identified by the presence of a monoclonal protein in the serum or urine (see "Clinical features, laboratory manifestations, and diagnosis of multiple myeloma"). Widespread skeletal metastases may present with diffuse pain, so details of a prior history of malignancy should not be overlooked. Hyperparathyroidism may present with proximal stiffness and aching, often in older patients, usually in the context of hypercalcemia with elevated parathyroid hormone (see "Primary hyperparathyroidism: Diagnosis, differential diagnosis, and evaluation"). Osteomalacia, with multiple microfractures, may be associated with widespread musculoskeletal pain.
●Drug-induced myalgias or myositis – Statins may be associated a variety of myopathic syndromes, including statin-related myopathy and myalgias. Myopathy results in elevations of the serum creatine kinase levels and weakness, which are not features of PMR. Statin-related myalgias can be distinguished from PMR by the absence of prominent morning stiffness and by the lack of proximally and symmetrically distributed symptoms. (See "Statin myopathy".)
●Inflammatory myopathy – Patients with dermatomyositis or polymyositis present with symmetric proximal muscle weakness, and shoulder or hip pain is not as prominent as in PMR. Elevated muscle enzymes, abnormal electromyography, abnormal MRI, and evidence of myositis on muscle biopsy establish the diagnosis of an inflammatory myopathy. (See "Clinical manifestations of dermatomyositis and polymyositis in adults".)
●Fibromyalgia – Patients with fibromyalgia syndrome (FM) experience widespread musculoskeletal pain, stiffness, aching, and fatigue. Although such patients are often younger than 50 years, FM can be seen in older patients. Patients with FM have symptoms of long-standing duration, as contrasted with PMR, in which symptoms are of recent, subacute onset. Physical examination in FM discloses no abnormalities, apart from characteristic tender points, and unlike most patients with PMR, those with FM have a normal ESR, CRP, and hematocrit. (See "Clinical manifestations and diagnosis of fibromyalgia in adults".)
●Infective endocarditis – The occurrence of fever in suspected PMR is always of concern and must prompt consideration for infection, as endocarditis and other infectious diseases may occasionally mimic PMR. The presence of a heart murmur, positive blood cultures, and vegetation on a heart valve on echocardiography are the diagnostic features of this infection (see "Clinical manifestations and evaluation of adults with suspected native valve endocarditis"). Patients with early, localized, or disseminated Lyme disease may present with nonspecific constitutional symptoms that include myalgias and arthralgias.
●Other – Other conditions whose initial presentations may occasionally prompt diagnostic concern for PMR include:
•Parkinson disease – Parkinson disease may present with stiffness in an older patient, but also with tremor and rigidity, including cogwheel rigidity, on examination. (See "Diagnosis and differential diagnosis of Parkinson disease".)
•Hypothyroidism – Patients with hypothyroidism may present with aching, stiffness, and arthralgias. Slow relaxation of deep tendon reflexes, a low serum thyroxine (T4) concentration, and an elevated serum thyrotropin (TSH) concentration are characteristic of hypothyroidism and are not seen in PMR. (See "Clinical manifestations of hypothyroidism", section on 'Musculoskeletal symptoms' and "Diagnosis of and screening for hypothyroidism in nonpregnant adults".)
•Malignancy – There is no association between the occurrence of PMR alone and malignancy . Very rarely, patients with malignancy have a paraneoplastic syndrome with diffuse muscle and joint pains which may resemble PMR . There is increasing recognition of the varied rheumatologic manifestations of the myelodysplastic syndromes, among which are symptoms and signs typical of PMR . The therapeutic response to low-dose glucocorticoids is brisk, as in PMR. The presence of leukopenia or thrombocytopenia in a patient with otherwise typical PMR should lead to consideration of further hematologic evaluation (see "Malignancy and rheumatic disorders", section on 'Malignant diseases: Musculoskeletal manifestations'). It must be emphasized that the presence of atypical clinical features (eg, fever, adenopathy, or a pulmonary infiltrate) calls for careful investigation before a diagnosis of PMR can be endorsed.
•Vasculitis – Vasculitic disorders other than GCA can present with arthralgias and an elevated ESR. This was illustrated in a retrospective study of 86 patients with systemic vasculitis affecting small vessels; 11 (13 percent) were thought to have PMR prior to the recognition of renal involvement . All 11 were found to have antineutrophil cytoplasmic antibodies (ANCA). Clinical and laboratory features that suggest ANCA-associated vasculitis include symptoms of upper respiratory tract involvement, pulmonary hemorrhage, renal disease, neuropathy, and positive ANCA. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis".)
•Crowned dens syndrome – “Crowned dens” refers to the radiologic appearance of the dens resulting from soft-tissue calcifications of the periodontoid tissues, presumably due to hydroxyapatite crystals or calcium pyrophosphate deposition disease. An accompanying clinical syndrome of posterior neck pain and stiffness, which can be acute and severe; shoulder girdle stiffness; fever; and elevations of the acute phase reactants could suggest PMR. Plain films may or may not show calcifications. CT examination of C1/C2 is necessary to confirm the periodontoid localization of calcification (image 1). Symptomatic relief is obtained with nonsteroidal antiinflammatory drugs (NSAIDs) or colchicine [67,68].
•Spondyloarthropathy – Proximal symptoms similar to PMR have been described in occasional patients with late-onset spondyloarthropathy (SpA) . Axial skeletal involvement, oligoarthritis, distal pitting edema, and constitutional symptoms such as fever, anorexia, and weight loss may be present along with an elevated ESR. In these patients, SpA can be differentiated from PMR by the presence of enthesitis, dactylitis, anterior uveitis, or sacroiliitis on imaging and by a high prevalence of human leukocyte antigen (HLA)-B27. (See "Clinical manifestations and diagnosis of peripheral spondyloarthritis in adults" and "Overview of the clinical manifestations and classification of spondyloarthritis".)
•Depression – Depression, which occurs in some patients with PMR, may be associated with weight loss and somatic symptoms, including musculoskeletal pain. Patients with depression do not have typical proximal aching and protracted morning stiffness. (See "Unipolar depression in adults: Assessment and diagnosis".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, “The Basics” and “Beyond the Basics.” The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on “patient info” and the keyword(s) of interest.)
●Basics topics (see "Patient education: Polymyalgia rheumatica and giant cell arteritis (The Basics)")
●Beyond the Basics topics (see "Patient education: Polymyalgia rheumatica and giant cell (temporal) arteritis (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
●Polymyalgia rheumatica (PMR) is an inflammatory rheumatologic condition characterized clinically by aching and morning stiffness about the shoulders, hip girdle, neck, and torso. It can be associated with giant cell (temporal) arteritis (GCA). PMR is almost exclusively a disease of adults over the age of 50; the incidence increases progressively with advancing age. The average age at diagnosis is over 70 years. (See 'Introduction' above and 'Epidemiology' above and 'Clinical manifestations' above.)
●Physical examination may reveal decreased active range of motion of the shoulders, neck, and hips. Muscle strength is normal when carefully tested. (See 'Symptoms and signs' above.)
●The characteristic laboratory finding is an elevation in the erythrocyte sedimentation rate (ESR) that occasionally exceeds 100 mm/h, but can also be less than 40 in a minority of patients. Elevated serum C-reactive protein (CRP) may be more sensitive than a high ESR for the diagnosis. (See 'Laboratory findings' above.)
●The diagnosis of PMR can be made in patients with the following features, in whom another disease to explain the findings is not present:
•Age 50 years or older at onset of symptoms
•Bilateral aching and morning stiffness (lasting 30 minutes or more) persisting for at least two weeks, and involving at least two of the following three areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs
•ESR increased to 40 mm/h or more
•Prompt response of symptoms to low-dose glucocorticoids
Although there is less experience with use of serum CRP testing to establish the diagnosis of PMR, it may be reasonable to substitute an elevated serum CRP as a criterion for PMR if the ESR is less than 40 mm/h. (See 'Diagnosis of PMR' above.)
●Patients should be routinely monitored for signs and symptoms suggestive of GCA.
●Temporal artery biopsy is NOT required in the absence of signs or symptoms of GCA. Evaluation, including biopsy, should be performed if symptoms of GCA develop, even if patients are on glucocorticoids. (See 'Evaluation for GCA' above.)
●Early seronegative rheumatoid arthritis (RA) may initially be difficult to differentiate from PMR. The differential diagnosis of PMR also includes a variety of other disorders that can usually be distinguished clinically from PMR. (See 'Differential diagnosis' above.)
- Salvarani C, Gabriel SE, O'Fallon WM, Hunder GG. Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991. Arthritis Rheum 1995; 38:369.
- Crowson CS, Matteson EL, Myasoedova E, et al. The lifetime risk of adult-onset rheumatoid arthritis and other inflammatory autoimmune rheumatic diseases. Arthritis Rheum 2011; 63:633.
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