Rheumatic polymyalgia



OVERVIEW

Definition.

Rheumatic polymyalgia (PMR) is a syndrome characterized by pain and stiffness in the muscles of the neck, scapular girdle, and pelvic girdle accompanied by systemic reactions such as fever and elevated blood sedimentation [1-2].

Morbidity

The disease is prevalent in the elderly over 50 years of age, and is rare below 50 years of age. The incidence of the disease gradually increases with age, and is 2 to 2.5 times more common in women than in men [1].

The highest incidence rate is in people >65 years old, especially those aged 70-80 years [3].

The incidence of PMR is reported as (20.4-53.7)/100,000 in foreign countries, and the incidence of PMR in people over 70 years of age is as high as 112.2/100,000. Although there is no epidemiological investigation data in China, it is not uncommon in clinical practice [1].

Etiology

Causes

The etiology of rheumatic polymyalgia is unknown and may be related to genetics, intrinsic immune response, infection and other factors. There is no uniform conclusion, and studies in various countries have proposed multiple factors, but there is no clear supporting evidence [2].

High-risk factors

According to current epidemiologic data, people with the following risk factors are more likely to develop rheumatic polymyalgia.

People over 50 years of age, especially those between 70 and 80 years of age.

Family history of rheumatic polymyalgia.

People with abnormal immune function.

People with the presence of viral infections (e.g., adenovirus, respiratory syncytial virus infection) [3].

Symptoms

Main Symptoms

General symptoms

Include generalized aches and pains, malaise, malaise, lethargy, insomnia, and fever, which is predominantly low, or in a few cases, high.

The onset of the disease may be sudden or insidious, lasting for weeks or months.

Typical symptoms

Typical symptoms are stiffness and pain in neck muscles, shoulder blade and upper arm muscles, hip and thigh muscles, in severe cases, inability to get up, limited lifting of upper limbs, inability to lift lower limbs, inability to squat, difficulty in ascending and descending stairs, etc. In some cases, the pain is so severe that patients are unable to turn over and take a deep breath. Myalgia is mostly distributed symmetrically, but can also be unilateral or limited to a certain muscle group.

If the disease is not diagnosed and treated for a long time, it may cause joint and muscle movement disorders, difficulty in active or passive movement of joints, and may develop into muscle atrophy in the late stage.

Some lesions may also involve the tendon attachments of the limb girdle muscles, and some may also present with pain and edema of the wrist and interphalangeal joints, or even transient synovitis of the sternoclavicular, shoulder, knee or hip joints, which manifests as joint swelling, pain, and limitation of movement [4].

Consultation

Department of Medicine

Rheumatology

If there is no obvious reason for muscle pain, accompanied by generalized pain, malaise, fatigue, fever and other symptoms, it is recommended to consult the Department of Rheumatology and Immunology.

Preparation

Consultation: Registration, Preparation of Documents, Frequently Asked Questions

Tips

Avoid taking painkillers on your own before going to the doctor to avoid masking your condition.

If you have mobility problems, it is recommended that a family member accompany you to the clinic.

Preparation Checklist

Pay particular attention to the time of onset of symptoms, special manifestations, etc.

Are there symptoms such as pain in the neck, shoulder blade, upper arm, hip and thigh, and stiffness in these areas?

Do you have difficulty lifting your arms up, legs, squatting, or walking up and down stairs?

Are there symptoms of swelling and pain in the wrists and interphalangeal joints?

Are there any symptoms of generalized pain, discomfort, or weakness?

Are there any symptoms of fever? What is the temperature of the body during fever?

Are there any symptoms of emaciation and insomnia?

Is there a family history of rheumatic polymyalgia?

Is there a history of immune dysfunction?

Is there a history of viral infection prior to the onset of the disease (e.g., adenovirus infection, respiratory syncytial virus)?

Test results in the last 6 months, which can be brought to the doctor’s office

Laboratory tests: routine blood tests, blood sedimentation, C-reactive protein, blood biochemistry (including liver function, cardiac enzyme profile), immunological tests (including serum interleukin IL-6, rheumatoid factor, antinuclear antibodies, anti-neutrophil cytoplasmic antibodies and other autoantibodies, etc.).

Imaging tests: ultrasound, MRI.

Electromyography.

Histopathologic examination: muscle biopsy.

Medication use in the last 3 months, carry the box or package with you to the doctor if available

Glucocorticoids: prednisone, methylprednisolone, etc.

Non-steroidal anti-inflammatory drugs: diclofenac, meloxicam, celecoxib, etc.

Immunosuppressants: methotrexate, azathioprine, cyclophosphamide, etc.

Diagnosis

Diagnosis is based on

Medical history

The following is not necessary for the diagnosis of the disease, but a history of the following may provide some reference for the diagnosis of the disease.

Age 50 years or older.

Family history of rheumatic polymyalgia.

People with immunologic abnormalities.

People with viral infections (e.g., adenovirus, respiratory syncytial virus).

Clinical manifestations

Rheumatic polymyalgia is mainly characterized by stiffness and pain in the neck, scapula, upper arm, hip and thigh muscles, as well as systemic symptoms such as generalized pain, fatigue, lethargy, insomnia, fever, etc. The symptoms may include active or passive movement of the joints.

There may be active or passive joint movement disorder, wrist and interphalangeal joint edema, and muscle atrophy in the advanced stage.

Laboratory Tests

Routine blood tests may be performed to see if the patient is anemic.

Patients with rheumatic polymyalgia may have mild to moderate anemia. If the routine blood test suggests anemia, it has some significance in the diagnosis of the disease.

Knowing whether the blood sedimentation rate is increased can help in the diagnosis.

Patients with rheumatic polymyalgia generally have a significantly increased sedimentation rate. If the examination of the patient’s blood sedimentation ≥ 40 mm / h, suggesting active inflammation, can be used as one of the diagnostic criteria.

It can help to know whether inflammation is present in the patient and assist in the diagnosis.

C-reactive protein is increased in patients with rheumatic polymyalgia and parallels the activity of the disease. If elevated C-reactive protein is present, it suggests severity of activity and aids in the diagnosis of the disease.

Include liver function, renal function, and cardiac enzyme profile.

It will help to know whether the patient has liver damage, muscle damage, kidney damage, etc. The presence or absence of transaminases and serum creatine kinase tests can be clarified by liver function and cardiac enzyme profile. If the values of blood creatinine and urea nitrogen are increased, it can indicate the presence of kidney injury.

Patients with rheumatic polymyalgia may have mildly elevated transaminases and normal serum creatine kinase. If liver function suggests elevated aminotransferases and cardiac enzyme profile suggests elevated serum creatine kinase, this may assist in the diagnosis and differential diagnosis.

The primary determination of the presence of immune system abnormalities assists in the diagnosis and differential diagnosis.

It mainly includes serum interleukin (IL-6), rheumatoid factor, anti-nuclear antibody, anti-neutrophil cytoplasmic antibody and other autoantibodies.

Patients with rheumatic polymyalgia have elevated serum interleukin (IL)-6 levels; antinuclear and other autoantibodies and rheumatoid factor are generally negative. Abnormalities in serum interleukin (IL-6), rheumatoid factor, antinuclear antibodies, and other autoantibodies are helpful in the diagnosis of the disease.

Imaging

Imaging may be used to visualize the presence of synovitis at the shoulder, knee, or hip joints.

If ultrasound examination reveals the presence of synovial membrane, bursa, and joint cavity effusion at the shoulder, knee, or hip joint, it may indicate non-specific inflammation. It may suggest a non-specific inflammatory response and help in the diagnosis of the disease.

MRI examination can be used to understand the shoulder, knee or hip joint imaging changes to assist in diagnosis.

Shoulder imaging MRI manifestations include subdeltoid/subacromial bursitis and biceps tenosynovitis. Bilateral subdeltoid/subacromial bursitis is an imaging hallmark of rheumatic polymyalgia, an abnormality found in 66% of patients with rheumatic polymyalgia [5].

If MRI shows synovitis of the shoulder, knee or hip, it helps in the disease diagnosis of rheumatic polymyalgia.

Electromyography

Identification of the presence of inflammatory myopathy by electromyography can assist in the differential diagnosis.

Patients with rheumatic polymyalgia do not show inflammatory myopathy on EMG. If EMG is abnormal, it can help in the differential diagnosis of the disease.

Pathohistologic examination

Muscle biopsy for pathohistologic examination to rule out inflammatory myopathies may assist in the differential diagnosis.

Patients with rheumatic polymyalgia do not show inflammatory myopathy on pathohistologic examination. Muscle biopsy is instructive in the diagnosis of the disease.

Diagnostic criteria

The diagnosis can be made by fulfilling the following 3 criteria.

Age of onset ≥50 years.

Morning stiffness of cervical, scapular and upper arm or pelvic and thigh myalgia on both sides.

Hematologic sedimentation rate (ESR) ≥40 mm/h or low-dose glucocorticoids are effective.

Satisfaction of the first 2, if ESR is normal, a rapid response to treatment with low-dose glucocorticoids (prednisone 10-15 mg/d) may be substituted for criterion #3 [1].

Differential diagnosis

Giant cell arteritis

Similarities: there are systemic symptoms such as malaise, weight loss, and fever.

Differences: Giant cell arteritis may be characterized by headache, visual abnormalities, temporal artery rages, increased or decreased pulsations with tenderness, and poor response to low-dose glucocorticoid therapy; temporal arteriography, ultrasound, and biopsy can assist in the differential diagnosis.

Rheumatoid arthritis (RA)

Similarities: clinical manifestations of joint swelling and pain.

Differences: RA is characterized by symmetric synovitis of small joints with destructive joint lesions and rheumatoid nodules, and is often positive for rheumatoid factor and anti-cyclic citrullinated polypeptide (CCP) antibodies [6].

Polymyositis

Similarities: there are clinical manifestations of muscle weakness and myalgia.

Differences: polymyositis is characterized by progressive proximal extremity, cervical, and pharyngeal muscle weakness, elevated serum muscle enzymes, abnormal electromyography, muscle biopsy showing lymphocytic infiltration, and myofibrillar atrophy, whereas patients with rheumatic polymyalgia have normal muscle enzymes, electromyography, and muscle biopsy, and myalgia is more pronounced than myalgia [6].

Fibromyalgia syndrome (FMS)

Similarities: there are clinical manifestations of myalgia.

Differences: FMS has fixed symmetrical pressure points, normal muscle strength and joints, sleep disorders, tension headaches, irritable bowel, irritable cystitis, generally normal blood sedimentation and C-reactive protein, and ineffective on glucocorticoid therapy.

Treatment

Aim of treatment: to alleviate the symptoms as soon as possible through drug treatment, to reduce recurrent attacks and delayed recovery, and to achieve the goal of cure.

Treatment principle: rational application of drug therapy to prevent recurrence of the disease; drug therapy is based on small-dose glucocorticoid.

General treatment

Eliminate worries about the disease and actively cooperate with the treatment.

Follow the doctor’s instructions and use medication reasonably to prevent recurrence of the disease.

Exercise appropriately to prevent muscle atrophy.

Medication

Glucocorticoid

Small-dose glucocorticoid therapy is the drug of choice and is the cornerstone of the treatment of rheumatic polymyalgia [7]. It provides anti-inflammatory and symptomatic control of joint and muscle pain.

Commonly used drugs are prednisone and methylprednisolone.

Prednisone should be applied in small doses, the symptoms can be rapidly improved within 1 week, after 2 to 4 weeks prednisone is gradually reduced, as the symptoms improve and the blood sedimentation is close to normal, a maintenance dose can be applied to maintain the treatment.

More serious disease, fever, myalgia, activity is obviously limited by the starting dose of prednisone can be slightly higher, with the improvement of symptoms, gradually reduce the amount of maintenance therapy, general maintenance drug 1 to 2 years.

Reduce the dose too early, too fast or stop the drug too early can lead to recurrence of the disease, most patients can stop using glucocorticosteroids within 2 years, a small number of patients need to be maintained in small quantities for many years.

Long-term use of glucocorticosteroids in the elderly should be particularly aware of their adverse effects and complications (e.g., hypertension, diabetes mellitus, cataracts, osteoporosis), and appropriate treatment should be given in a timely manner [1,8].

Non-steroidal anti-inflammatory drugs (NSAIDs)

Non-steroidal anti-inflammatory drugs (NSAIDs) can be used in first-onset or milder cases, and about 10% to 20% of patients with rheumatic polymyalgia have symptom control with NSAIDs alone [1].

Commonly used drugs such as diclofenac sodium, meloxicam, celecoxib and so on.

The main adverse effects of NSAIDs are intestinal irritation symptoms, which may include epigastric discomfort, vague pain, nausea, vomiting, fullness, belching, loss of appetite and other dyspeptic symptoms, and prolonged oral administration of NSAIDs may increase the risk of peptic ulcers, liver damage and bleeding.

NSAIDs are contraindicated in people with active peptic ulcers or bleeding.

Immunosuppressants

Immunosuppressants may be used in combination in patients who have contraindications to the use of glucocorticoids, or who are ineffective, or who have difficulty tapering, or who have severe adverse effects [8].

Commonly used immunosuppressants include methotrexate, azathioprine, and cyclophosphamide.

Common adverse reactions of methotrexate include nausea, vomiting, stomatitis, diarrhea, increased transaminases, and can also lead to reversible bone marrow suppression, pneumonia, alopecia, teratology, etc., and occasionally lead to hepatic fibrosis and cirrhosis. During the use of the drug need to regularly check the blood routine and liver and kidney function.

The main adverse reactions of azathioprine are general malaise, dizziness, nausea, vomiting, diarrhea, fever, chills, rash, vasculitis, myalgia, arthralgia, hypotension, hepatic and renal dysfunction and cholestasis, etc.; it should be used with caution in the elderly.

Common adverse reactions to cyclophosphamide are myelosuppression, loss of appetite, nausea and vomiting, and hemorrhagic cystitis.

Other treatments

There are emerging studies showing the efficacy of biologic agents such as monoclonal antibody-based TNF-α antagonists (infliximab, tolizumab, etc.), but data are limited.

Based on these single, small sample size results, the risk of serious complications associated with the use of biologics and their high price, biologics are not used clinically for the treatment of rheumatic polymyalgia [3].

Further clinical studies are needed for the treatment of rheumatic polymyalgia with biologics [9].

Prognosis

Cure

Rheumatic polymyalgia has a good prognosis after reasonable treatment, and the condition can be rapidly relieved or cured; some patients may also have recurrent episodes of the disease, which will not be cured [10-11].

If the disease progresses without treatment, the patient may have impaired mobility, muscle atrophy and other conditions.

Prognostic factors

Patients with poor medication adherence can affect prognosis.

Patients with contraindications to glucocorticoid use have a poor prognosis.

Hazards

Patients with rheumatic polymyalgia have quality of life affected by arthralgia, generalized pain, and mobility problems.

Patients take medication for a long time, which is easy to cause psychological burden.

Some patients with recurrent and prolonged illness can develop psychological disorders such as anxiety, irritability and depression.

Daily

Daily Management

Dietary management

Daily diet should be nutritious and increase the intake of iron-containing foods, such as animal liver, soybeans, meat, eggs and black sesame.

Patients taking glucocorticosteroids or non-steroidal anti-inflammatory drugs for a long period of time should try to avoid stimulating foods such as coffee, chili peppers, acidic foods, etc. to minimize gastrointestinal reactions.

Exercise management

Exercise should be done according to the strength of the body and the intensity of exercise should be increased gradually.

Patients should try to choose exercises that increase muscle strength, such as lifting heavy objects, walking with sandbags tied to the legs, and other exercise programs according to their own disease recovery to prevent myasthenia gravis.

Psychological support

Maintain optimism in daily life, face the disease positively and establish confidence in overcoming the disease.

Family members should care more about the patient, sympathize with the patient’s mood, give encouragement and support. If anxiety, depression and other psychological disorders occur, the patient should go to a professional institution as early as possible for intervention or application of medication.

Follow-up

Regular follow-up can understand the recovery of the disease, and at the same time can observe the progress of the disease, treatment effect, adverse drug reactions, and whether there is a relapse. Any abnormalities can be handled in a timely manner.

If the condition is stable, the relevant indicators can be rechecked every 3 to 6 months, and any changes in the condition should be followed up promptly.

The review may need to do blood routine, C-reactive protein, blood sedimentation, liver function, immunological examination, ultrasound, MRI and other examination items.

Prevention

The cause of rheumatic polymyalgia is unknown, and it cannot be prevented effectively, but the risk of developing the disease can be reduced in the following ways

Preventing infections, keeping warm, maintaining balanced nutrition and getting enough sleep on a daily basis.

People with a family history of rheumatic polymyalgia and aged 50 or above should have regular medical checkups for early detection and treatment.

People with abnormal immune function should actively treat the primary disease and control the progression of the disease.

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