Polymyalgia rheumatica is an inflammatory disorder that is characterized by pain and stiffness of the neck, shoulders and hips. It may occur before or with giant cell arteritis.
Presentation
Patents with polymyalgia rheumatica generally present with pain and morning stiffness in the shoulder or hip. Patients may have difficulty getting up from a chair, turning over in bed, or raising their arms. Patients are usually in good health prior to the onset of the disease. The presentation is abrupt in 50% of patients [3].
Usually affected joints include those of [2] [3] [4]:
- Shoulders
- Hips
- Knees
- Metacarpal phalangeal joints
- Wrists
The shoulders are the most common site, occurring in 70%–95% of patients. Hip and neck pain occurs in 50%–70% of patients [2] [6].
Systemic findings [10]:
- Low-grade fever
- Weight loss
- Malaise
- Fatigue
- Difficulty completing activities of daily living
The symptoms of polymyalgia rheumatica are often vague and may occur in many other disorders [3] [4] [6].
Diagnostic criteria for polymyalgia rheumatica are [10] [11]:
- Age 50 years or older
- Erythrocyte sedimentation rate ≥40 mm/h
- Pain lasting longer than 1 month and involving at least 2 sites
- Exclusion of other diseases with similar symptoms
- Morning stiffness lasting more than 1 hour
- Rapid response to corticosteroids
Some patients exhibit a chronic-relapsing course and may need low doses of corticosteroids for many years [4].
Entire Body System
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Pain
Adjusted linear regression analysis demonstrated that high (NRS 8) pain (0.20 (95% CI 0.10-0.28)) or stiffness (0.18 (0.09-0.26)) ratings, an increasing number of sites of pain (0.18 (0.06-0.29)) or stiffness (0.19 (0.08-0.31)) and shoulder pain (0.18 [ncbi.nlm.nih.gov]
[…] can help narrow down the cause of the pain. [en.wikipedia.org]
Hip and neck pain occurs in 50%–70% of patients. [symptoma.com]
A syndrome in the elderly characterized by proximal joint and muscle pain, high erythrocyte sedimentation rate, and a self-limiting course. Pain is usually accompanied by evidence of an inflammatory reaction. [icd9data.com]
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Fever
Other symptoms include fever, weakness and weight loss. In some cases, polymyalgia rheumatica develops overnight. In others, it is gradual. [icd9data.com]
In addition to stiffness, you may have a fever, weakness, and weight loss. Polymyalgia rheumatica usually goes away within one year, but it could last several years. [niams.nih.gov]
A 56-year-old African American woman who was on triple immunosuppressive therapy (which includes tacrolimus, mycophenolate mofetil, and prednisone) for a renal transplant that she had received 10 years ago presented with malaise, low-grade fevers and [ncbi.nlm.nih.gov]
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Weight Loss
In addition to stiffness, you may have a fever, weakness, and weight loss. Polymyalgia rheumatica usually goes away within one year, but it could last several years. [niams.nih.gov]
Associated symptoms include low-grade fever, malaise, fatigue, low mood, poor appetite, and weight loss. There is no specific diagnostic test for PMR but the usual pattern is a commensurate rise in CRP and ESR. [ncbi.nlm.nih.gov]
Other symptoms include fever, weakness and weight loss. In some cases, polymyalgia rheumatica develops overnight. In others, it is gradual. [icd9data.com]
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Fatigue
Associated symptoms include low-grade fever, malaise, fatigue, low mood, poor appetite, and weight loss. There is no specific diagnostic test for PMR but the usual pattern is a commensurate rise in CRP and ESR. [ncbi.nlm.nih.gov]
Scalp tenderness, fatigue, fevers and a general sense of illness occurs in about 50% of patients. Jaw or facial soreness, especially with chewing, also occurs in about 50% of patients. [my.clevelandclinic.org]
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Anemia
Hemolytic anemia with reduction of haptoglobin and cold agglutinin was detected, and the patient had a negative Donath-Landsteiner test. [ncbi.nlm.nih.gov]
A mild anemia is often is often present. One blood test, called an erythrocyte sedimentation rate, is very high, much more so than in most other diseases. [medical-dictionary.thefreedictionary.com]
[…] with giant cell arteritis: 10–20% of patients with polymyalgia rheumatica also have GCA References:[4][1][3] Clinical features Systemic symptoms Constitutional symptoms: fever, weight loss, night sweats Fatigue and malaise Depressed mood Symptoms of anemia [amboss.com]
Others are anemia, depression and flu-like symptoms including appetite loss and resulting weight loss, fever and weakness. [wellstar.org]
Gastrointestinal
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Loss of Appetite
BACKGROUND: A 73-year-old, previously well woman was admitted to an emergency department because of a 3-month history of severe proximal girdle pain and stiffness with loss of appetite and weight. [ncbi.nlm.nih.gov]
Patients also experience fatigue, loss of appetite, weight loss or a flu-like feeling. GCA can damage eyesight by impacting the blood supply to the eye. This can be treated. [nebraskamed.com]
Possible Symptoms of PMR Impaired Range of mMotion Low fever Pain in shoulders (often first symptom), neck, hip or thigh Fatigue (tiredness, weakness, low energy) Loss of appetite Unintended weight loss Possible Intervention Physical therapy Healthy diet [geriatricpain.org]
Some people with polymyalgia rheumatica have additional symptoms, including: feeling very tired loss of appetite weight loss depression When to get medical advice See your GP if you have symptoms of pain and stiffness that last longer than a week. [nhs.uk]
Cardiovascular
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Acrocyanosis
A 60-year-old male who had been treated for polymyalgia rheumatica (PMR) with Raynaud's phenomenon was admitted to our hospital with acrocyanosis and rapid progressive anemia. [ncbi.nlm.nih.gov]
Skin
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Night Sweats
Fever, night sweats, and weight loss were not discriminating features and were equally distributed among the RA, PMR, and GCA patients. [dovepress.com]
Other clues are fatigue, weight loss, a slight temperature, night sweats and feeling unwell. Giant cell arteritis (GCA) is a rare condition linked to PMR, caused by inflammation of medium and large arteries, usually in the head and neck. [saga.co.uk]
human leukocyte antigen HLA-DR4) Association with giant cell arteritis: 10–20% of patients with polymyalgia rheumatica also have GCA References:[4][1][3] Clinical features Systemic symptoms Constitutional symptoms: fever, weight loss, night sweats [amboss.com]
The patient can present with slow, subacute or chronic symptoms of malaise, fever, weight loss, night sweats, and anorexia. [visualdx.com]
Musculoskeletal
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Arthritis
Report of a Ten‐Year Study in Shelby County, Tennessee, Arthritis & Rheumatism, 26, 10, (1214-1219), (2005). D. Pongratz and W. [doi.org]
We report here cases of rheumatoid arthritis (RA) and polymyalgia rheumatica (PMR) occurring after ICI treatment. [ncbi.nlm.nih.gov]
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Myalgia
The headache and myalgia were improving with corticosteroid treatment; however, a trans-sphenoidal surgery was performed due to visual field loss. [ncbi.nlm.nih.gov]
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Muscle Weakness
Polymyalgia rheumatica (PMR) is classified as diffuse soft tissue rheumatism with characteristic pain and stiffness of proximal extremities/muscles without muscle weakness or atrophy. [youtube.com]
There is no true muscle weakness nor evidence of muscle disease on electromyography (EMG) or on biopsy. [healthcentral.com]
It does not lead to muscle weakness, but causes intense pain. It can make it difficult to get out of bed, stand up from a chair, exit a car, comb your hair, take a bath, and get dressed. [mountsinai.org]
Snapshot A 60-year-old woman presents to her physician's office for headache and muscle weakness. She reports that she usually never has headaches but recently started experiencing headaches in the morning about 2 weeks ago. [step2.medbullets.com]
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Shoulder Pain
Typical symptoms include acute or subacute bilateral shoulder pain with severe stiffness and often neck and bilateral hip pain. [mayoclinic.pure.elsevier.com]
The diagnosis of PMR is based on clinical features such as new-onset bilateral shoulder pain, including subdeltoid bursitis, muscle or joint stiffness, and functional impairment. [ncbi.nlm.nih.gov]
After stabilization for bleeding he was evaluated for shoulder pain. He reported increasing pain and stiffness in his shoulder and pelvic girdle over 1 month. [jrheum.org]
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Myopathy
METHODS: We studied 60 patients who had undergone 18 F-FDG PET/CT scans for workup of suspected PMR, arthritis, enthesitis, or myopathy. Final diagnoses were made by board-certified rheumatologists. [ncbi.nlm.nih.gov]
On the other hand, patients with statin-associated myopathy may have normal serum CK levels which make it sometimes difficult to distinguish PMR from myopathic syndromes [33]. [journals.plos.org]
Creatine kinase: This test is done to check for muscle tissue damage (myopathy), which can cause weakness and pain of the shoulder and hip muscles. If the level of creatine kinase in the blood is elevated, muscle damage is likely. [merckmanuals.com]
Polymyalgia rheumatica is differentiated from inflammatory myopathy ( DM and PM ) by the absence of frank muscle weakness or characteristic skin and other nonmusculoskeletal findings. [rheumaknowledgy.com]
Neurologic
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Headache
The headache and myalgia were improving with corticosteroid treatment; however, a trans-sphenoidal surgery was performed due to visual field loss. [ncbi.nlm.nih.gov]
Snapshot A 60-year-old woman presents to her physician's office for headache and muscle weakness. She reports that she usually never has headaches but recently started experiencing headaches in the morning about 2 weeks ago. [step2.medbullets.com]
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Asthenia
However, at that time, she started to complain of asthenia, abdominal cramping and pain on the left side, weight loss and bloody diarrhoea. A colonoscopy confirmed a diagnosis of left-sided UC. [ncbi.nlm.nih.gov]
Muscle tenderness, peripheral synovitis, arthritis, carpal tunnel syndrome or distal tenosynovitis, as well as non-specific symptoms, such as fatigue, asthenia, malaise, low-grade fever, anorexia and weight loss, may be associated. [orpha.net]
[…] these symptoms are usually bilateral, and affect the upper arms, neck, pelvis girdle, hips, and thighs in 50–90% of cases The onset of symptoms is often rapid, generally over a few days Constitutional manifestations such as low-grade fever, fatigue, asthenia [thelancet.com]
Workup
There is no definitive diagnostic test for polymyalgia rheumatica [3]. A rapid response to corticosteroids is considered diagnostic. Lack of response may indicate an incorrect diagnosis [9] [10].
Laboratory studies [12]
- Erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- Complete blood count (CBC) with differential
- Liver function tests
- Creatine kinase level
- Serum creatinine and urinalysis
- Thyroid function test
- Serum calcium
- Blood glucose
White blood cell count in polymyalgia rheumatica is normal or mildly elevated. Platelet count is increased indicating systemic inflammation [3] [12].
Erythrocyte sedimentation rate (ESR) is the test most sensitive for polymyalgia rheumatica, but is not specific. It may be elevated in any inflammatory disease and may not always be elevated in polymyalgia rheumatica [3] [4] [10].
Serum interleukin 6 (IL-6) levels are elevated and rise and fall with disease activity. This test is not readily available in all laboratories [10].
Antinuclear antibodies, rheumatoid factor, and anticyclic citrullinated peptide (anti-CCP) levels are usually normal [10].
Other procedures
Synovial fluid aspiration in patients with synovitis show signs of inflammation, white blood cell counts of 1,300-11,000 cells/µL, with 34% polymorphonuclear leukocytes [12].
Radiographic studies show normal joints or mild osteoarthritis. More extensive arthritis indicates other disorders such as rheumatoid arthritis.
Magnetic resonance imaging (MRI) is not necessary for diagnosis, but reveals bursitis and synovitis [12] [13].
Ultrasonography may be helpful for differential diagnosis. In polymyalgia rheumatica shoulder, bursa effusions may be seen [13].
There are no reliable tests for predicting the possible recurrence in polymyalgia rheumatica. Erythrocyte sedimentation rate (ESR) has been used for this, but results are inconsistent [9]. C-reactive protein and interleukin-6 may be more sensitive predictors [9].
Serum
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Thrombocytosis
Most people with polymyalgia rheumatica have an unusually high number of these cells (thrombocytosis). On the other hand, many people with polymyalgia rheumatica have a lower number of red blood cells than normal and are often anemic. [web.archive.org]
Anemia of chronic disease, leukocytosis, reactive thrombocytosis, and decreased albumin are also common. Alkaline phosphatase and hepatic enzymes may be elevated. [rheumaknowledgy.com]
Such abnormalities can include a mild normocytic anemia due to chronic inflammation, leukocytosis, and thrombocytosis. Inflammatory markers (erythrocyte sedimentation rate [ESR] and C-reactive protein [CRP]) frequently are elevated. [practicalpainmanagement.com]
Hayreh 2003 points out that although essential thrombocytosis has increased thrombotic morbidity, the thrombocytosis in GCA is reactive and thus an antithrombotic agent is likely to have little effect. [doi.org]
Other laboratory signs of inflammation may include: thrombocytosis, leukocytosis, anemia, hypoalbuminemia, polyclonal hypergammaglobulinemia, and an elevated ferritin. [rheumatologyadvisor.com]
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Elevated Sedimentation Rate
Although lab tests alone cannot definitively diagnose PMR, most people with the disease have an elevated sedimentation rate (ESR). This is determined by a blood test for certain types of inflammation. [wellstar.org]
New York: McGraw-Hill, 1997. pol·y·my·al·gi·a rheu·ma·ti·ca a syndrome within the group of collagen diseases different from spondylarthritis or from humeral scapular periarthritis by the presence of an elevated sedimentation rate; much commoner in women [medical-dictionary.thefreedictionary.com]
Diagnosis The diagnosis of polymyalgia rheumatica may be confirmed by a thorough clinical examination including a detailed patient history and specialized blood testing that demonstrates an abnormally elevated sedimentation rate. [rarediseases.org]
Elevated sedimentation rate and elevated acute phase reactions are hallmarks of this disease. It is primarily a disorder of elderly Caucasians of northern European ancestry. [reliasmedia.com]
Treatment
The diagnosis of polymyalgia rheumatica is often made after an extensive workup to exclude other diseases. This delays treatment and relief of symptoms [6].
The goal of treatment is to control pain, reduce stiffness, prevent systemic components, and prevent the effect of the disease on daily life [6] [11].
Corticosteroids are the treatment of choice, as they result in complete or near-complete symptom relief and normalization of erythrocyte sedimentation rate [3] [4]. Starting doses of prednisone of 10 to 15 mg/day are recommended [6]. Expect relief of symptoms within 24-72 hours. If symptoms are not well controlled within 1 week, steroid doses should be increased until they are [6] [14]. Once symptoms are controlled and erythrocyte sedimentation rates have dropped, slow tapering by 1 mg/month is recommended [14].
Inflammatory markers document the progression of the disease, but should not be used to decrease or stop treatment. Patient-reported complaints of pain, morning stiffness, and physical and mental function are the best measures of disease activity and response to treatment [3] [4] [6] [14].
Long-term high cumulative doses of corticosteroids have been associated with more adverse effects [6] [14]. If not contraindicated, non-steroidal anti-inflammatory medications may be used for supplemental pain relief [6] [14].
Corticosteroid-sparing agents in patients with polymyalgia rheumatica may reduce corticosteroid adverse effects. It is of particular benefit in diabetic patients or those with osteonecrosis [3] [5].
- Methotrexate at 7.5 mg/week along with prednisone showed decreased complications [15].
- Tumor necrosis factor alpha (TNF-α), Etanercept, reduces the cumulative dose of prednisone needed [15]. Results have been variable, but indicate the drug to be safe [10].
Relapses are common in polymyalgia rheumatica. Close, frequent follow-up is necessary to monitor for relapse and long-term complications of corticosteroid use. Medications for treatment of osteoporosis and gastrointestinal ulcer prophylaxis should be instituted as necessary [3] [4].
Diet
Calcium and vitamin D supplements should be prescribed for all patients with polymyalgia rheumatica on starting corticosteroid therapy [3] [4].
Activity
No activity restriction are recommended. Physical therapy for those with difficulty with mobility and activities of daily living may be needed.
Follow-up
Patients on corticosteroids should be followed monthly to track disease progress. After tapering of the steroid dosage, quarterly follow-up should be sufficient to identify possible relapses [5] [15].
Relapses occur in approximately 50% of patients, most often within 1 year of corticosteroid withdrawal. All patients should be monitored for symptom of recurrence during tapering of corticosteroids and for 12 months after therapy [5]. If symptoms recur prednisone is still the drug of choice [5].
Prognosis
Polymyalgia rheumatica is a chronic, recurring disorder. It may last as long as 2 years or more. Recurrences occur in 40-50% of patients [8]. Prognosis is generally good.
Adverse complications of corticosteroid use, osteoporotic fractures and diabetes mellitus, are common. One study reported 65% of patients had at least one serious corticosteroid complication [9].
Polymyalgia rheumatica has been associated with atherosclerosis and a higher risk of cardiovascular and cerebrovascular disease [2]. The use of corticosteroids may actually decrease this risk by controlling inflammation [2].
Quality of life, both physical and mental, may be affected by polymyalgia rheumatica. Pain and morning stiffness may reduce physical quality of life, while disability and decreased function may cause depression [7].
Etiology
The symptoms of polymyalgia rheumatica are due to inflammation [3]. The exact cause of the inflammation is unknown. An autoimmune process may be involved in polymyalgia rheumatica because it is associated with a high level of Interleukin 6 (IL-6) [5].
Epidemiology
The mean incidence of polymyalgia rheumatica is 52.5 cases per 100,000 persons 50 years and older, with a prevalence of 0.5-0.7% [4].
Polymyalgia rheumatica occurs most frequently in Caucasians, only occasionally occurring in Africans and Hispanics [4] [5] [6].
Polymyalgia rheumatica develops almost always in individuals over the age of 50 with the mean age of 72 years. The incidence of polymyalgia rheumatica increases with age [6] [7].
Women are affected two to three times more often than men [5] [6].
Pathophysiology
The exact cause is not known. Polymyalgia rheumatica presents as inflammation of the shoulders, hips, knees, and wrists. Inflammation is thought to start in response to an unknown antigen [3]. This disease is often associated with significantly elevated indicators of inflammation such as erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) [6]. Rapid response to corticosteroids is the only consistent diagnostic finding [6]. The lack of response to corticosteroids indicates the need to question the diagnosis [3].
A genetic component predisposing some individuals to the disorder is suspected because of the higher incidence among Northern Europeans and in family members [3] [5]. This hypothesis postulates that in genetically predisposed patients an environmental factor, possibly a virus, causes white blood cell activation with the production of cytokines [2].
An autoimmune process may also occur in polymyalgia rheumatica. Patients usually have an elevated Interleukin 6 level which is likely responsible for the systemic inflammatory response [2] [3] [5].
Polymyalgia rheumatica may be associated with the aging process as it occurs almost entirely in those over 50 years of age and the incidence increases with age.
Prevention
Polymyalgia rheumatica cannot be prevented at this time. The pain, disability, and the effects on quality of life can be prevented with early diagnosis and prompt treatment.
The use of steroid-sparing agents, such as methotrexate may reduces the serious corticosteroid-related complications [9]. The prevention of and early treatment of osteoporosis and uncontrolled diabetes will potentially prevent the many, serious complications of these disorders.
Summary
Polymyalgia rheumatica is a common disorder of the elderly, individuals 50+ years [1] [2]. It is a chronic inflammatory disease of unknown etiology characterized by pain in the hip and shoulder girdles with morning stiffness that lasts for more than 1 hour [2].
Polymyalgia rheumatica affects the proximal muscles and joints, and may cause disability in older adults [3] [4]. It is diagnosed by the presenting symptoms and the exclusion of other diagnoses. A rapid response to corticosteroids, the treatment of choice, is considered diagnostic [1].
Polymyalgia rheumatica has an excellent prognosis [5]. However, recurrences are common [1] [2]. More than 50% of patients remain on steroids for 2 years or more [2].
Although the etiology of polymyalgia rheumatica is unknown, infectious or environmental agents may be triggers [6]. A genetic component is also suspected, as the disorder occurs more often in individuals of Northern European descent and in family members.
Polymyalgia rheumatica is often a co-morbid disorder with giant cell arteritis. Approximately 15% of patients with polymyalgia rheumatica develop giant cell arteritis and 40-50% of patients with giant cell arteritis have a history of polymyalgia rheumatica [1] [2].
Patient Information
What is polymyalgia rheumatica?
Polymyalgia rheumatica is a relatively common inflammatory disease of older adults. Peak incidence is after 50 years of age. It causes pain and morning stiffness of the shoulders, hips, neck, and wrists. It is a self-limited condition which resolves in months to several years with no residual effects. However, relapses occur in 40 to 50 % of patients.
What are the symptoms of polymyalgia rheumatica?
Symptoms of polymyalgia rheumatica include:
- Pain in the joints of the shoulders, hips, neck, and wrists
- Morning stiffness lasting longer than 1 hour
- Difficulty preforming activities of daily living
- Low-grade fever
- Weight loss
- Malaise
- Fatigue
What causes polymyalgia rheumatica?
The cause of polymyalgia rheumatica is unknown. There are several theories related to its occurrence. It is felt that polymyalgia rheumatica may be an autoimmune (the body’s immune system attacking itself) inflammatory disease triggered by an infection or environmental agent. Another theory purposes a genetic factor that predisposes certain individuals to acquiring the disease.
Who gets polymyalgia rheumatica?
Polymyalgia rheumatica occurs almost exclusively in individuals older than 50 years of age with a mean age of 72 years.
Polymyalgia rheumatica is twice as common in females as males.
It is primarily a disease of Caucasians, only occurring rarely in African Americans and Hispanics. The incidence of polymyalgia rheumatica is highest in those of Northern European descent.
How is polymyalgia rheumatica diagnosed?
There is no specific test for polymyalgia rheumatica. It is usually diagnosed by its presenting symptoms and the fact that they respond quickly to corticosteroid medications. Some blood tests that indicate inflammation will also be used to diagnose the disease and response to treatment.
How is polymyalgia rheumatica treated?
Polymyalgia rheumatica is treated almost exclusively with corticosteroids like prednisone. Other medications may be added to reduce the dose of prednisone needed.
What are the complications of polymyalgia rheumatica?
The complications of polymyalgia rheumatica include short-term disability associated with pain and stiffness and decreased quality of life.
Other complications are not a result of the disease itself, but to long-term corticosteroids use. These complications include severe osteoporosis and uncontrolled diabetes mellitus.
References
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- Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, Miranda-Filloy JA, Gonzalez-Juanatey C, Martin J, et al. Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum. 2009;61(10):1454-61.
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