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Clinical manifestations and diagnosis of polymyalgia rheumatica
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Mar 2012. | This topic last updated: Feb 3, 2010.

INTRODUCTION — Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized clinically by aching and morning stiffness in the shoulders, hip girdle, and neck [1]. 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 [1]:

  • How is the diagnosis established and distinguished from other disorders that can produce similar symptoms?
  • Does the patient also have GCA?

PMR is two to three times more common than GCA. PMR occurs in about 50 percent of patients with GCA, while approximately 15 to 30 percent of patients with PMR eventually develop GCA [1,2]. Some patients have manifestations of both disorders occurring at different times.

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 prevalence that increases progressively with advancing age. The average age at diagnosis is over 70 years [3].

PMR is relatively common, with a prevalence of about 700 per 100,000 persons over 50 years of age [4]. Women are affected two to three times more often than are men.

The annual incidence varies geographically [3-7]. In Europe, for example, the incidence rates 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]. Rates in Japan are even lower than Europe (less than 2 per 100,000 patients older than 50) [6].

PATHOGENESIS — The cause of PMR is unknown; both environmental and genetic factors appear to play a role [1,3]. In addition to the frequent clinical association between PMR and 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 HLA-DR4. In addition, there is 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 shared by patients with rheumatoid arthritis [8].

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 [1]. Evidence of subclinical arterial inflammation can be detected in some patients, including the presence of activated dendritic cells, interleukin-1, and interleukin-6 [1]. However, unlike GCA, interferon gamma producing T cells are not prominent [1].

Some studies of PMR and GCA have suggested a cyclical pattern in incidence and seasonal variation, indicating a possible environmental infectious trigger [3]. Results of studies of several viruses as potential triggers have been inconclusive.

CLINICAL MANIFESTATIONS

Symptoms and signs — PMR is typically characterized by the subacute or chronic onset of aching and morning stiffness in the shoulders, hip girdles, neck, and torso in patients over the age of 50 [2,9]. The symptoms are usually symmetric [2].

The following features can be seen:

  • Stiffness — Morning stiffness typically lasts at least 30 minutes. Difficulty with activities related to the shoulder and hip stiffness may be described by the patient as trouble dressing (eg, fastening a bra normally, donning a shirt or jacket, or pulling on stockings). In some patients, stiffness may be so severe that rising from or turning over in bed represent major obstacles.
  • Pain — Shoulder pain is more common at presentation (70 to 95 percent of patients) than is hip and neck involvement (50 to 70 percent). Pain is worse with movement and may interfere with sleep.
  • Synovitis and bursitis — About half of patients exhibit distal musculoskeletal manifestations. Palpable synovitis appears to occur in more peripheral joints, such as the knees, wrists, and metacarpophalangeal joints [10-12]. Synovitis is usually mild and 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 are not readily detected on physical examination.
  • Swelling and tenosynovitis — Some patients develop swelling and pitting edema of the hands, wrists, ankles, and top of the feet [10,13]. 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 [13]. Tenosynovitis can also cause carpal tunnel syndrome, which occurs in approximately 10 to 15 percent of patients with PMR [10].
  • Decreased range of motion — There may be decreased active, and sometimes passive, range of motion of the shoulders, neck, and hips.
  • 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 — Muscle strength is usually normal. However, weakness is often reported by patients and may be a problem diagnostically due to diminished effort on examination because of pain, or clinically because of disuse atrophy. Thus, careful testing is required if weakness is reported.
  • Systemic signs and symptoms — Up to 40 percent of patients experience systemic signs or symptoms, including malaise, fatigue, depression, anorexia, weight loss, and fever. However, the high spiking fever that can sometimes occur in GCA is rare in patients who have only PMR [10]. (See "Clinical manifestations of giant cell (temporal) arteritis".)

Laboratory findings — The characteristic laboratory finding in PMR (and in GCA) is an erythrocyte sedimentation rate (ESR) that is above 40 mm/h (78 to 93 percent in some series), with some patients having values that can exceed 100 mm/h [14-16]. In one study, 20 percent of patients had ESR values over 104 mm/hr [17]. Levels below 40 mm/h are most likely to occur in patients with limited disease and fewer systemic symptoms and in those who have been treated with glucocorticoids.

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) [17,18]. In one of these studies, nine of the ten 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 than in the group as a whole (19 versus 68 mg/L) [17]. Among patients with PMR, 90 percent had an ESR greater than 40 mm/h and 90 percent had a CRP greater than 22 mg/L.

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 [2].
  • Some patients experience an increase in liver enzymes, especially alkaline phosphatase, although this is more common in patients with GCA than PMR alone.

Imaging — Routine radiographs of inflamed joints rarely reveal any abnormalities in patients with PMR. By comparison, routine imaging by other techniques (such as magnetic resonance imaging or ultrasonography) can detect underlying inflammation in patients with PMR. However, this is usually not required.

MRI and ultrasound — Magnetic resonance imaging (MRI) studies have confirmed that PMR involves inflammation of extraarticular synovial structures. As an example, MRI of the hands and feet of affected patients frequently demonstrates inflammation of the tenosynovial sheaths; flexor, posterior tibial, and peroneal tenosynovitis have also been observed [10]. In addition, shoulder MRI reveals subacromial and subdeltoid bursitis (typically bilateral) in almost all patients with active PMR [19]. (See "Bursitis: An overview of clinical manifestations, diagnosis, and management".)

Bursa ultrasonography (US) often reveals effusions within both shoulder bursae in patients with PMR. 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 most patients but few controls (96 versus 4 percent) [20]. In contrast, there was no difference between the groups in the incidence of glenohumeral joint synovitis or biceps tenosynovitis. There was a good correlation between US and MRI.

PET scanning — Positron emission tomography (PET scanning) has no proven clinical value in the care of patients with PMR, but has been of interest in the investigation of underlying vascular involvement. Data are limited on PET scanning in patients with PMR. 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 [21]. While increased uptake of radiolabeled glucose is suggestive of large vessel vasculitis, histologic examination of the aorta and large arteries was not feasible.

The clinical implications of these PET scan findings are unclear, although they may indicate that subclinical inflammation of the great vessels is more common than previously supposed in PMR. Further study is needed to determine whether PET scanning has any prognostic or therapeutic implications.

EVALUATION — Important elements in the evaluation of the patient suspected of having PMR include:

  • Focusing on key elements that are consistent with the diagnosis
  • Careful attention to the possible presence of disorders that closely resemble PMR
  • Particular attention to the possible presence of GCA, which is found in 15 to 30 percent of those with PMR.

The initial evaluation of patients suspected of having 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). The history should also include whether there are symptoms of GCA, including new headache, jaw claudication, scalp tenderness, visual change, fever, or cough. (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 in the truncal and peripheral joints and spine; the presence of signs of inflammatory or degenerative arthritis, tendonitis or bursitis; and muscle strength and tenderness. Patients should be examined for evidence of tender points suggestive of fibromyalgia and other findings that may also 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 CBC and platelet count, erythrocyte sedimentation rate, and CRP levels. Additional testing may be required depending upon the patient's symptoms and findings and other diagnoses being considered. (See 'Laboratory findings' above and 'Differential diagnosis' below.)
  • Imaging studies such as an MRI or ultrasound of the shoulder region may be useful to determine if an inflammatory process is present in patients with signs and symptoms of PMR, but a normal ESR and CRP, if uncertainty remains regarding the diagnosis. Plain radiographs are not required, unless another diagnosis that may be confirmed or excluded by such studies is being considered. (See 'Imaging' above.)

DIAGNOSIS

Diagnosis of PMR — In the absence of any pathognomonic test, we use the presence of each of the following three criteria for the clinical diagnosis of PMR [1]:

  • Age 50 years or older at disease onset
  • Bilateral aching and morning stiffness (lasting 30 minutes or more) persisting for at least one month. 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 (Westergren) ≥40 mm/h

Some experts add a prompt response of symptoms to glucocorticoids as an additional criterion. Symptoms are generally 50 to 70 percent better within three days in patients with PMR started on prednisone (15 to 20 mg/day). The presence of another disease to explain the findings excludes the diagnosis.

Establishing the diagnosis is more difficult in patients with atypical presentations. These include age 40 to 50 years, asymmetric symptoms, and ESR less than 40 mm/h [2,14-16]:

  • In younger patients, inflammatory rheumatic diseases that may mimic PMR are more prevalent than PMR itself; the diagnosis in these patients thus necessitates careful consideration of alternative diagnoses [22]. (See 'Differential diagnosis' below.)
  • Asymmetric symptoms may be present especially early in disease, beginning in one shoulder or hip, but soon become bilateral in most patients [1].
  • Although there is less experience with use of serum CRP testing to establish the diagnosis of PMR [17], 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.)

Evaluation for GCA — As noted above, there is considerable overlap in the diagnoses of PMR and GCA. Patients with "pure" PMR lack the classic findings of GCA such as temporal artery tenderness, headache, jaw pain, visual loss, and evidence of noncranial ischemia (such as arm claudication or cerebral ischemia) [12]. In the absence of such signs or symptoms, we do NOT routinely perform a temporal artery biopsy, since a positive result is rare in this setting [12,23].

Patients with a marked inflammatory response, such as a high fever (at least 39ºC) or a lack of response to 20 mg/day of prednisone may have an abnormal temporal artery biopsy if performed. However, we do not routinely biopsy such patients since they seldom develop ischemic complications [1].

We suggest ongoing 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 [24]. (See "Treatment of polymyalgia rheumatica" and "Diagnosis of giant cell (temporal) arteritis".)

DIFFERENTIAL DIAGNOSIS — The differential diagnosis of PMR includes early seronegative rheumatoid arthritis (RA) (most common) and a large variety of other disorders that can usually be easily distinguished clinically from PMR [1,2,22]. Limited laboratory testing may be helpful depending on the specific clinical presentation.

  • Rheumatoid arthritis — Patients with RA usually 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 are different from that in PMR in which fewer joints are swollen, and the swelling subsides completely in response to low dose prednisone. (See "Clinical features of rheumatoid arthritis" and "Use of glucocorticoids in the treatment of rheumatoid arthritis".)

    There is, however, considerable overlap between PMR and seronegative RA in elderly subjects who present with a benign symmetric synovitis. The following are illustrative:

  • In a report of 159 such patients, synovitis was recurrent in 57, 20 of whom had one episode that looked like PMR and another that looked like RA [25]. All patients responded to prednisone. Twenty-four patients later developed GCA: 19 had been given the diagnosis of PMR and 5 of RA. (See "Diagnosis and differential diagnosis of rheumatoid arthritis".)
  • Another study compared 142 patients with late-onset (over the age of 60) seronegative (rheumatoid factor negative) RA, 147 with PMR, and 42 with PMR-GCA [26]. Patients were followed for a minimum of five years. Although synovitis was present at baseline in 23 percent of patients with PMR, what helped distinguish one from the other at baseline were the following: patients with PMR all had myalgia, while the presence of MCP, PIP, and/or wrist involvement was more suggestive of RA; furthermore, the synovitis generally responded quickly to treatment with glucocorticoids and did not require treatment with disease-modifying antirheumatic drugs. Acute phase reactants (ESR, CRP, IL-6) were higher in patients with PMR and responded more dramatically to treatment with glucocorticoids than in patients with RA [27].

    We initially treat those patients in whom PMR cannot be clinically distinguished from seronegative RA with glucocorticoids in the doses used for PMR, and observe the response over the first two months of therapy. Patients who respond well to such therapy are continued on this treatment regimen and glucocorticoids are tapered accordingly. Treatment for RA should be instituted instead 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 symptoms and signs of the RS3PE syndrome (remitting seronegative symmetrical synovitis with pitting edema) may be mistaken for those of PMR [28]. Patients with RS3PE typically have the sudden onset of polyarthritis, are frequently over 50 years of age, and lack rheumatoid factor. Symptoms are usually more prominent distally, unlike PMR. Some patients with this disorder respond to treatment with low-dose glucocorticoids and may be considered to have a variant of PMR with prominent edema. Others can evolve into a more specific disease such as RA or a spondyloarthropathy [29].

    Some patients with RS3PE have a paraneoplastic disorder that is associated with solid tumors and hematologic disorders [30]. The possibility of malignancy may be increased in those who do not have a favorable response to low-dose glucocorticoid therapy.
  • Bursitis/tendinitis — The symptoms and findings in the shoulders in PMR may be similar to subdeltoid bursitis without PMR or rotator cuff tendinitis. However, tenderness is minimal in most cases of PMR, while patients with bursitis or tendinitis alone lack constitutional symptoms, an elevated erythrocyte sedimentation rate, anemia, and bilateral shoulder involvement. Despite these general differences, a form of subacromial and subdeltoid bursitis as well as shoulder synovitis may underlie the shoulder girdle stiffness in PMR [19]. (See 'MRI and ultrasound' above.)
  • Spondyloarthropathy — Proximal symptoms similar to PMR can be seen in patients with late onset spondyloarthropathy (SpA) [31]. 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 [32]. SpA and PMR can be distinguished in these patients by the presence of other findings typical of SpA, but not PMR, such as enthesitis, dactylitis, anterior uveitis, sacroiliitis on imaging, and the greater prevalence of HLA-B27. (See "Clinical manifestations, diagnosis, and management of undifferentiated spondyloarthritis and related spondyloarthritides".)
  • CPPD disease — Patients with polyarticular calcium pyrophosphate deposition disease (CPPD) infrequently present with symptoms suggestive of PMR [33]. The diagnosis of CPPD disease is made by the identification of characteristic crystals on examination of the joint fluid. Radiographic findings, such as chondrocalcinosis, may also be present. (See "Clinical manifestations and diagnosis of calcium pyrophosphate crystal deposition 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".)
  • Fibromyalgia — Patients with fibromyalgia syndrome (FMS) experience widespread musculoskeletal pain, stiffness, aching, and fatigue. Although such patients are often younger than 50 years, FMS can be seen in older patients. No obvious abnormalities on physical examination other than characteristic tender points may be present in patients with FMS, and unlike most patients with PMR, those with FMS have a normal erythrocyte sedimentation rate, C-reactive protein, and hematocrit. (See "Clinical manifestations and diagnosis of fibromyalgia in adults".)
  • Malignancy — There is no association between the occurrence of PMR alone and malignancy [34]. However, occasional patients with malignancy have a paraneoplastic syndrome with diffuse muscle and joint pains that can mimic PMR [2,35]. These patients do not respond to prednisone but do respond to removal of the tumor [35]. (See "Malignancy and rheumatic disorders", section on 'Malignant diseases: Musculoskeletal manifestations'.)

    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".)
  • Infective endocarditis — Endocarditis and other infectious diseases may occasionally mimic PMR. Persistent fever may suggest the possible diagnosis of infective endocarditis. 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 "Diagnostic approach to infective endocarditis".)
  • 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, and evidence of myositis on muscle biopsy establish the diagnosis of an inflammatory myopathy. (See "Clinical manifestations and diagnosis of adult dermatomyositis and polymyositis".)
  • Vasculitis — Vasculitic disorders other than GCA can present with arthralgia 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 [36]. 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, and neuropathy and positive ANCA. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis".)
  • Other — Other conditions in which the ESR is normal may occasionally be mistaken for PMR [22]. These 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 of Parkinson disease".)
  • Hyperparathyroidism — Hyperparathyroidism may present with proximal stiffness and aching, often in older patients, but with elevated parathyroid hormone and often with hypercalcemia. (See "Diagnosis and differential diagnosis of primary hyperparathyroidism".)
  • Drug-induced myalgias or myositis — Symptoms of myalgias and aching may be due to the use of statins, but such patients usually do not exhibit signs of inflammation, and symptoms may be associated with elevated creatine kinase levels. (See "Muscle injury associated with lipid lowering drugs".)
  • Depression — Depression may be associated with somatic symptoms and weight loss, and can occur in some patients with PMR. Patients with depression do not have typical proximal aching and morning stiffness. (See "Clinical manifestations and diagnosis of depression".)

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SUMMARY AND RECOMMENDATIONS

  • Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized clinically by aching and morning stiffness in the shoulders, hip girdle, neck, and torso. It can be associated with giant cell (temporal) arteritis (GCA). Polymyalgia rheumatica (PMR) is almost exclusively a disease of adults over the age of 50 and 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 range of motion of the shoulders, neck, and hips. Muscle tenderness is not prominent, and muscle strength is usually normal when carefully tested. (See 'Symptoms and signs' above.)
  • The characteristic laboratory finding is an elevation in the erythrocyte sedimentation rate (ESR) that can exceed 100 mm/h, although values below 40 mm/h are seen in a minority of patients. Elevated serum CRP may be more sensitive than a high ESR for the diagnosis of and/or monitoring relapses. (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 one month, 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
  • Erythrocyte sedimentation rate (ESR) increased to 40 mm/h or more, or an increased C reactive protein in patients with a normal ESR

Some experts add a prompt response of symptoms to glucocorticoids as an additional criterion. (See 'Diagnosis of PMR' above.)

  • 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.)
  • The differential diagnosis of PMR includes early seronegative rheumatoid arthritis and a variety of other disorders that can usually be distinguished clinically from PMR. (See 'Differential diagnosis' above.)

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