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What is polymyalgia rheumatica?

by Josephine Andrews
Published: Last Updated on 246 views

Polymyalgia rheumatica is an inflammatory rheumatic disease that primarily affects older women. Common symptoms are pain in the shoulder and/or hip area as well as general symptoms such as fatigue and fever. Early treatment with cortisone can help many sufferers. Read more about the causes and symptoms of polymyalgia rheumatica and the associated diagnostics, treatment and prognosis here.

ICD codes for this disease:

ICD codes are internationally valid codes for medical diagnoses. They can be found, for example, in doctor’s letters or on certificates of incapacity for work.

M31 M35

quick overview

  • What is Polymyalgia Rheumatica (PMR)? Inflammatory rheumatic autoimmune disease (immune system attacks endogenous tissue); colloquially called inflammatory soft tissue rheumatism or muscular rheumatism
  • Causes : Unknown. Genetic factors and external influences (eg infections) may be involved in the onset of the disease.
  • Symptoms : muscle pain (especially in the shoulder and/or pelvic girdle area), stiff muscles and joints in the morning, general symptoms (e.g. fatigue, fever, night sweats , weight loss, depressed mood)
  • Diagnosis : Doctor-patient interview, physical examination, blood tests, ultrasound (ACR-EULAR classification of polymyalgia rheumatica)
  • Therapy : medication (cortisone, possibly also methotrexate ); if necessary, further support measures (e.g. physiotherapy , ergotherapy ).
  • Prognosis : If detected early, polymyalgia rheumatica is usually easily treatable. If cortisone therapy is too short (< 1 year), the risk of a relapse increases.

Polymyalgia rheumatica: definition

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease. The term polymyalgia is also popular. Because the immune system attacks the body’s own tissue, it is an autoimmune disease.

The attack of the misdirected immune system in PMR triggers inflammatory changes in the shoulder, upper arm, pelvic and thigh muscles and the adjacent soft tissues (such as bursa). Therefore, the disease is colloquially called inflammatory soft tissue rheumatism or muscular rheumatism .

Soft tissue rheumatism and muscular rheumatism are not official disease names! Rather, the terms describe the painful symptoms in the respective area.

When they say “soft tissue rheumatism” or “soft tissue rheumatism” some people mean fibromyalgia syndrome! However, this is only a specific form of soft-tissue rheumatism (generalized soft-tissue rheumatism).

Associated with giant cell arteritis

Polymyalgia rheumatica is closely related to another autoimmune disease – giant cell arteritis, GCA for short (formerly also called temporal arteritis , cranial artery, capitis artery or Horton’s disease). In both cases, autoimmune-related vascular inflammation occurs in medium-sized and large arteries:

  • Polymyalgia rheumatica : The area of ​​the shoulders, neck and upper arms is mainly affected, usually later also the pelvic girdle and thighs. Inflammatory changes occur in the artery under the collarbone (subclavian artery). This inflammation spreads to adjacent joints, bursae, and tendon sheaths. The vascular inflammation itself tends to fade into the background (subclinical vasculitis ).
  • Giant cell arteritis : Inflammation of the arteries is more prominent and mainly affects arteries of the skull, usually the temporal artery (A. temporalis). Inflammatory cells ( leukocytes ) migrate into the arterial wall. As a result, giant cells form – the wall becomes thicker and firmer, and blood circulation is disrupted.

Experts disagree as to whether polymaylgia rheumatica and giant cell arteritis are actually two different diseases or whether PMR is actually a milder form of GCA. However, some people with polymyalgia rheumatica also develop giant cell arteritis (about 20 percent). Conversely, 40 to 60 percent of patients with GCA also have PMR. In principle, both diseases can occur simultaneously or follow each other (first PMR, then GCA or vice versa).

Polymyalgia rheumatica: who is affected?

Polymyalgia rheumatica is the second most common inflammatory rheumatic disease in old age (after rheumatoid arthritis ). It is most common in people between the ages of 70 and 80. Illness before the age of 50 is very rare.

Most patients are female: Polymyalgia rheumatica is two to three times more common in women than in men.

There are also differences in the geographical distribution of the disease: within Europe, polymyalgia rheumatica occurs more frequently in the north than in the south. Overall, the disease is more common in Europeans than in Asians, African Americans, and Latinos.

Polymyalgia rheumatica: cure & prognosis

Polymyalgia rheumatica is not quickly overcome. The course generally extends over two to four years. However, some patients suffer from it for ten years or more. It is therefore important to detect and treat the disease as early as possible.

The right drug treatment (cortisone) can suppress polymyalgia rheumatica so that the patient no longer has any symptoms and the laboratory values ​​are normal. In the best-case scenario, it stays that way even after you stop taking the medication. Doctors then speak of “drug-free remission”.

About 50 to 70 percent of all patients with polymyalgia rheumatica are symptom-free after completion of cortisone therapy. The following applies: The chances of a lasting freedom from symptoms can be increased by a sufficiently long cortisone therapy. On the other hand, those who have taken the cortisone for less than twelve months are more prone to a relapse.

Polymyalgia rheumatica: causes

The cause of polymyalgia rheumatica is not yet known. Experts suspect that there is a genetic predisposition to the disease. The disease occasionally runs in families. However, it is not a classic hereditary disease!

In addition to genes, external factors could also be involved in the onset of polymyalgia rheumatica. For example, infections such as the parvovirus B19 are discussed. They could trigger the dysregulation of the immune system that leads to the inflammatory changes described.

Polymyalgia rheumatica: symptoms

The part of the name “polymyalgia” comes from the Greek and refers to a typical symptom of the disease – lots of muscle pain :

Within a few days to two weeks, those affected develop severe and usually bilateral (symmetrical) pain in the shoulders, neck and upper arms . The symptoms are usually based on bursitis, more rarely on inflammation of the biceps tendon or the synovial membrane (synovitis). The hip, thigh, and lower back areas can also hurt.

The pain is present around the clock – both at rest and during movement and stress. Often they intensify at night in the second half of the night and in the morning. After some time, the pain may appear in waves and shift.

A pronounced morning stiffness is also typical of polymyalgia rheumatica : joints and muscles feel stiff for more than 45 minutes in the morning. This, along with the pain, makes it difficult for patients to get out of bed and get dressed.

In addition to restricted mobility, some patients also suffer from swollen joints . Joints away from the shoulder or pelvis can also be affected, usually the hands and knees. If the synovial membrane of the wrists becomes inflamed, some patients also develop carpal tunnel syndrome .

In addition, general symptoms can occur with polymyalgia rheumatica, such as:

  • exhaustion, tiredness
  • lack of drive
  • Fever
  • Loss of appetite with weight loss, nausea
  • Increased sweating , especially pronounced night sweats
  • Depressive mood

If patients have giant cell arteritis in addition to polymyalgia rheumatica, there are additional symptoms such as headaches (often unilateral and in the temple area), chewing pain and visual disturbances . For more symptoms of giant cell arteritis click here .

The suspicion of giant cell arteritis represents an emergency, especially in the case of visual disturbances! Inflammation of the eye vessels may have developed. Without rapid medical treatment, there is a risk of blindness!

Polymyalgia rheumatica: investigations and diagnosis

In the case of unclear symptoms such as severe shoulder and neck pain with fatigue and fever, the family doctor is usually the first point of contact. If he suspects a rheumatic cause, he will refer you to a specialist. This is usually a specialist in internal medicine and rheumatology, or rheumatologist for short.

There is no single, specific test to detect polymyalgia rheumatica. The doctor makes the diagnosis on the basis of the anamnesis interview, the blood and ultrasound tests. He excludes other diseases with similar symptoms (such as rheumatoid arthritis , polymyositis ).

anamnese

The first step in clarifying your symptoms is a detailed discussion of your medical history ( anamnesis ). The doctor will ask you exactly what symptoms you have, how long they have existed and how they manifest themselves in everyday life (e.g. stiff joints in the morning, severe shoulder and neck pain especially at night). It is also important for the doctor to know whether there are previous or underlying diseases and whether you are already taking medication.

Physical examination

The interview is followed by a general physical examination. It gives the doctor information about your general state of health and helps to rule out other diseases as the cause of the symptoms.

During the physical examination, the doctor pays particular attention to your joints. If the synovial membrane has become inflamed (synovitis) due to polymyalgia, the joints (such as hands or knees) will be slightly swollen. The doctor will also test your mobility: some PMR patients have difficulty raising their arms to the side more than 90 degrees.

blood tests

As with almost all inflammatory diseases, conspicuous inflammatory values ​​can also be determined in the blood of polymyalgia rheumatica : As a rule, the blood sedimentation rate and/or the C-reactive protein ( CRP ) have increased in the case of PMR . The number of white blood cells (leukocytes) may also be increased. In addition, doctors sometimes find an increased number of platelets ( thrombocytes ) and a shift in blood proteins.

Autoantibodies such as rheumatoid factor , which is mostly present in the blood in rheumatoid arthritis and some other rheumatic diseases, are typically not detectable in polymyalgia rheumatica.

Ultrasonic

Doctors can use ultrasound to detect the inflammatory changes in the shoulder area (e.g. bursitis) that typically occur with polymyalgia rheumatica. X-rays, on the other hand, are unremarkable. At most, they show age-typical changes such as joint wear and tear, but no signs of inflammation.

ACR-EULAR classification of polymyalgia rheumatica

In 2012, the experts from the European League Against Rheumatism (EULAR) and the American College of Rheumatology developed a scoring system to support the diagnosis of polymyalgia rheumatica. Doctors can use this score when patients

  • are older than 50 years,
  • have new shoulder pain on both sides and
  • the CRP and/or blood sedimentation are increased.

The criteria themselves relate primarily to the symptoms of polymyalgia rheumatica, various blood values ​​and an ultrasound examination. Depending on the test results, the person concerned collects points, which indicates the presence of polymyalgia rheumatica from a corresponding number of points.

Further investigations if necessary

If the doctor additionally or alternatively suspects giant cell arteritis, a tissue sample ( biopsy ) from arteries can be revealing: A piece is usually removed from the temporal artery and examined under a microscope for inflammatory changes. In the case of polymyalgia rheumatica, on the other hand, the vascular biopsy delivers an inconspicuous result.

Sometimes further investigations are necessary to rule out other diseases. An example: If the cause of the symptoms is polymyositis (an autoimmune disease of the muscles) in addition to polymyalgia rheumatica, a tissue sample from the painful muscles can help. In PMR, such a muscle biopsy is unremarkable. In polymyositis, on the other hand, typical changes are found in the muscle sample.

In contrast to polymyositis, an electromyography (EMG), which measures muscle activity, and the blood level of creatine kinase are usually normal.

Once the diagnosis of polymyalgia rheumatica has been made, further examinations for therapy planning can be useful. This can be a bone density measurement , for example . The cortisone therapy required for polymyalgia rheumatica can promote or intensify bone loss ( osteoporosis ).

Polymyalgia rheumatica: therapy

Doctors treat polymyalgia rheumatica with medication. In addition, further therapeutic measures can be useful as support in individual cases.

medication

The basis of the treatment of polymyalgia rheumatica is always the administration of glucocorticoids (“cortisone”) such as prednisone . It should be started immediately after the diagnosis and in sufficient dosage in order to get the disease and the symptoms under control as quickly as possible.

cortisone

Glucocorticoids such as prednisone inhibit the immune system and thereby counteract inflammation. Patients with polymyalgia rheumatica take a cortisone tablet once a day, in the morning. The dosage should be as high as necessary, but as low as possible :

The doctor usually starts the therapy with 15 to 25 milligrams of prednisone per day (the exact dose is individually adjusted). He then regularly monitors the effect of the treatment and disease activity. After a few weeks, the cortisone dose can usually be gradually reduced. If the polymyalgia rheumatica comes back (recurrence), the doctor increases the dosage again.

How long the cortisone has to be taken in total varies from person to person. As a rule, experts recommend taking it for at least a year. Basically, the doctor prescribes cortisone intake for as long as necessary, but as short as possible . The reason: Long-term use of cortisone can cause some side effects such as bone loss (osteoporosis). That is why the doctor makes sure that there is an adequate supply of vitamin D and calcium (both important for strong bones) during the treatment . If necessary, he will prescribe appropriate preparations.

In the case of (additional) giant cell arteritis, cortisone therapy is given immediately and in higher doses, otherwise there is a risk of blindness!

methotrexate

If a relapse is very likely in patients with polymyalgia rheumatica and/or high-dose and long cortisone therapy is necessary, the doctor often prescribes methotrexate as well. As a result, the cortisone dose can usually be reduced and its side effects prevented.

Combined therapy with cortisone and methotrexate can also be considered if relapses occur repeatedly in polymyalgia rheumatica. The same applies if comorbidities (e.g. osteoporosis, diabetes) already exist or cortisone is not effective enough.

Other medications for polymyalgia

In order to reduce the dose of cortisone therapy and thus possible side effects, scientists have examined the monoclonal antibody tocilizumab in polymyalgia rheumatica (and giant cell arteritis) in various studies. The results so far indicate a benefit in PMR therapy. Tocilizumab could therefore primarily help patients who cannot receive methotrexate or who continue to have symptoms under combination therapy.

Due to the limited study situation, there is no official recommendation for the use of tocilizumab in the currently valid guidelines!

TNF-alpha blockers, which are regularly used in rheumatoid arthritis, for example, have proven in several studies to be ineffective in polymyalgia rheumatica.

Other measures

Regular check -ups are very important during the treatment of polymyalgia rheumatica . In the first year, they are scheduled every four to eight weeks. In the second year they are recommended every eight to twelve weeks. In this way, the doctor treating you can react quickly to changes.

Consult your doctor promptly if your symptoms increase or side effects of the treatment occur.

If necessary, patients with polymyalgia rheumatica can receive psychosomatic or psychotherapeutic support – for example, if they suffer from severe depression as a result of the disease.

The attending doctor prescribes accompanying physiotherapy and occupational therapy , especially for older and frail people . This is to prevent patients from permanently losing mobility over the course of the painful illness.

Because patients are more susceptible to infection, adequate vaccination protection is important. The increased risk of infection results on the one hand from the inflammatory rheumatic disease itself. On the other hand, the cortisone treatment suppresses the immune system. You should therefore catch up on missing vaccinations, preferably before the start of therapy. Some may not be administered during high-dose cortisone treatment (live vaccines such as against measles or rubella ). On the other hand, the administration of inactivated vaccines (e.g. against influenza or pneumococci ) is not a problem.

Tips for polymyalgia rheumatica

  • Join a self-help group – talking to other sufferers can help you cope better with the painful condition.
  • Avoid being overweight or obese ( obesity ).
  • If you have polymyalgia rheumatica, make sure you eat a varied and balanced diet. We recommend, for example, the Mediterranean diet (Mediterranean diet), which is rich in vegetables, fruit and valuable vegetable fats, while meat is only served in moderation.
  • Make sure your body is getting enough calcium and vitamin D.
  • Do not smoke if you have polymyalgia rheumatica and you should only consume alcohol in moderation.
  • Do endurance sports regularly. For example, go running, cycling or swimming for half an hour three times a week.
  • Attend regular check-ups.

With these tips you can improve your quality of life despite polymyalgia rheumatica and support the success of the drug treatment.

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