Polymyalgia Rheumatica

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].

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].

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].

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].

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].

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].

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.

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.

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].

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.

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