Symptoms and treatment of rare diseases - myositis

Myositis is a rare disease characterized by a chronic inflammatory process in the body tissues, mainly in the muscles. As the inflammatory changes increase, muscle tone decreases and the inflammation spreads to other organs, including:

  • Joints
  • Heart
  • Light
  • Intestine
  • Skin

Myositis development rate: 1 case per 2 million population. In the USA, 135 patients with myositis have been registered.

This is a systemic disease that affects the muscles in all parts of the body, but cases have been described where myositis only developed in individual limbs( one arm or leg) or, for example, only the muscle that moves the eyeball. In such cases, narrower terms are used, for example, cervical myositis, the symptoms of which concern only the violation of muscle functions in the cervical region.

In some patients, myositis occurs as a short-term disorder, which occurs after a few days or weeks. For others it is a disease that has to be fought throughout life.

Chronic course with improper

treatment or lack of it can cause muscle atrophy and disability.

Reasons for

Among the causes of myositis are:

  • Systemic autoimmune diseases( lupus, scleraema, rheumatoid arthritis).The course of myositis in such cases is protracted and requires prolonged treatment;
  • Infections. The most common causes are viral infections. Less often - bacteria, fungi or protozoa. Viruses and bacteria can penetrate directly into the muscles, and also have a damaging effect, producing toxins. The viruses of the common cold, influenza and HIV are among the many that can cause myositis symptoms;
  • Medicinal products. Many medications can cause temporary damage to muscle fibers. This condition is called myopathy, but in the absence of adequate treatment in the damaged muscle, an inflammatory process develops. The drugs that increase the risk of myositis include statins, colchicine, plaquenil, alpha-interferon, as well as cocaine and alcohol.

Myopathy can develop either immediately after the start of the drug, or after several months or years with prolonged use. Sometimes this condition occurs as a result of improper dosing or simultaneous administration of several incompatible drugs. However, myositis caused by a drug rarely changes into a severe form.

  • Injury. Excessively intense exercises can lead to muscle pain, swelling and weakness, persisting for several days after training. Since swelling and pain are signs of inflammation, this condition can be regarded as an easy form of myositis that will resolve without consequences after a short recovery period.

Risk Factors

This disease can affect both adults and children. The most common form of the disease in childhood is dermatomyositis( usually occurs at the age of 2-15 years).Polymyositis and dermatomyositis are more common in women. Fibromyositis - a disease of the elderly over 50 years.

Symptoms of

The main symptom of myositis is muscle weakness. In this case, weakness can be expressed or detected only when carrying out special tests. The loss of muscle strength increases gradually over several weeks or months. The disease affects large muscles or groups of muscles of the neck, shoulders, hips and back. With muscle myositis, the symptoms usually develop symmetrically on both sides. In this case, the patient can not lift a heavy object, climb a ladder or even raise his hand or dress.

The most severe inflammation of the shoulder and pelvic muscles. A patient with a progressive course of myositis can often fall down and experience difficulty in lifting from a floor or stool. Other symptoms that characterize myositis include:

  • Rash;
  • General fatigue;
  • Sealing or thickening of the skin of hands.

Symptoms of myositis of the neck are characterized by difficulty in turning or tilting the head, as well as difficulty in swallowing.

Patients with myositis caused by a virus, note the symptoms of an infectious disease( fever, cough, runny nose, nausea, diarrhea, etc.).But signs of infection can go away long before the onset of symptoms of myositis.

Some patients complain of muscle pain, but in most of them this symptom develops not because of inflammation, but as a result of trauma or other diseases( flu, etc.).

  • Respiratory system. Myocardial infarction can affect the respiratory muscles, as well as cause pulmonary fibrosis( scarring of normal tissue).In this case, the patient complains of cough and shortness of breath, the inability to take a deep breath.
  • Systemic inflammatory manifestations. The patient is noted fever, weight loss, general malaise and the so-called phenomenon of Raynaud( increased sensitivity to cold, especially in the fingers and toes);
  • Joint pain. Usually occurs during periods of exacerbation of the disease, while the joints do not swell, the skin over them does not change color, does not warm.

Dermatomyositis is also a systemic disease of muscle tissue, but, unlike polymyositis, begins more acutely and affects both adults and children. Symptoms of the disease are similar to those described above, but they are accompanied by skin manifestations - the appearance of a rash.

Juvenile dermatomyositis is characterized by a sudden onset, proceeds as a myositis of the legs. The child complains of difficulty walking and severe pain in the legs. High intensity pain is noted in the muscles of the lower leg. Usually the disease is preceded by a recently transferred cold or sore throat.

This form differs from adult disease in the concomitant development of vasculitis( inflammation of the blood vessels), the deposition of calcium salts in tissues and the disturbance of the metabolism of fatty compounds. With this form of skin lesion and muscle weakness develop simultaneously, but the severity and progression of each of the symptoms in each patient can vary significantly.

Fibromyositis often develops in the elderly and is characterized by slow progression. There are cases when from the time of the onset of the first symptoms to the diagnosis was 5-6 years. More than 20% of patients have problems with swallowing. Muscle weakness does not always develop the same in symmetrical muscles and can predominate in the small muscles of the calf muscles and forearm muscles.


Treatment of

Depending on how the symptoms manifesting and are expressed in the myositis, treatment may include immunosuppressants, antibiotics, antiviral drugs, etc.

The tactics of treating myositis are selected individually depending on the cause of the disease, the severity of the course and the individual characteristics of the patient.

For inflammatory diseases that caused myositis, treatment is performed using immunosuppressive agents:

  • Prednisolone
  • Azathioprine( Imuran)
  • Methotrexate

There is no specific treatment for myositis of viral etiology. When the disease develops against the background of a bacterial infection, antibacterial drugs are added to the treatment.

For the treatment of myositis that has arisen after the use of the drug, it is sufficient to cancel its reception. Normalization of the condition occurs approximately in 2-3 weeks.

  • Corticosteroids

Hormonal drugs are used to reduce the inflammatory response. The most common use of prednisolone. Also appoint cortisone, hydrocortisone, methylprednisolone, triamcinolone, dexamethasone and betamethasone. In large doses, these drugs inhibit the activity of the immune system, which reduces the manifestations of the underlying disease, but increases the risk of infection. Dosage of corticosteroids is calculated depending on the severity of the course of the disease.

Patients receiving hormones should be examined at least once a year by an ophthalmologist for symptoms of cataract. In addition, there is a risk of local circulatory disorders with subsequent development of bone necrosis. This is a serious complication of hormone therapy, accompanied by severe pain. When new sources of soreness appear, contact your doctor immediately.

  • Azathioprine( Imuran)

Azathiopine is an immunosuppressant taken internally( capsules) at 50-150 mg / day. This drug depresses the production of erythrocytes and leukocytes, so during the treatment it is necessary to conduct a monthly blood test. Other side effects include hair loss, liver damage, nausea, vomiting, attachment of secondary infections.

  • Methotrexate( Revmatex, Rheumatrex)

The drug is similar in mechanism of action to azathioprine. The side effects are the same.

  • Plaquenyl

Plaquenyl has an immunosuppressive and anti-inflammatory effect. It is used mainly in elderly people with dermatomyositis.

  • Gamma-immunoglobulin

This is a drug derived from human plasma. It has been used for more than 15 years to treat various diseases. In myositis, it is used to reduce the level of creatine phosphokinase, an enzyme that appears in large quantities in the blood with the destruction of muscle fibers.

Side effects of treatment are:

  • Headache;
  • Aseptic meningitis( irritation of cerebral membranes);
  • Fever;
  • Digestive disorders;
  • General weakness.

Immunoglobulin is used in the myositis of viral etiology. It is usually used as a reserve preparation, when other methods proved ineffective.

  • Cyclophosphamide( Cytoxan)

Cyclophosphamide is an immunosuppressant that has a more pronounced activity than the drugs described above. On the other hand, there is a higher risk of side effects from its use. The peculiarities of the treatment include the need for strict control of the injected and isolated liquid. The drug has an irritant effect on the bladder, so the patient needs an increased volume of fluid consumed.

Physiotherapy and training

Treatment with myositis will not be effective without physiotherapy. This is necessary to maintain muscle tone and increase stamina. With the help of physiotherapy, muscular atrophy is prevented, which significantly improves the quality of life of patients.

Even a slight increase in physical activity during the day can bring a tangible positive result. For example, the treatment of back muscles is effectively carried out using swimming or even simple work in the garden. The volume of exercises is easily adapted for each individual patient.

The main recommendations for training include:

  • Mandatory workout. Muscles need to be warmed up to ensure normal blood flow improving the intake of nutrients and increase the contractile work of the heart;
  • Do the exercises in a mode that allows you not to overexert. Do not exercise through strength;
  • After a workout, rest, regain respiration and pulse.

There is no single training program. Each patient needs an individual selection. For example, the treatment of myositis of the neck is carried out using resistance exercises: sit up straight, look forward, put your hand on the forehead and tilt your head forward, while hand create reverse resistance. Repeat the same, but with the head tilted back, then sideways.

Please note that even such a seemingly simple method requires a daily adjustment with a daily evaluation of the effectiveness of the exercises.

Additional recommendations for exercises include:

  • Slow start with a gradual increase in tempo;
  • Concentration on a diseased muscle. If you feel that you are overloading the problem area, do not continue training, rest;
  • Do not lose enthusiasm. During the treatment will necessarily be present hard days. Develop a program for good and bad days;
  • Train with your partner.

Rehabilitation specialists for patients with muscle diseases recommend aquatherapy. Such treatment of myositis in children is especially effective and often allows in a short time to completely restore the lost physical activity and avoid disability.

Prospective Studies of

Studies on myositis and other autoimmune myopathies continue uninterruptedly. Usually new methods of diagnosis and treatment of myositis are introduced as a result of discoveries in related areas( rheumatoid arthritis, lupus).

The promising treatment options that will be available in the future are:

  • Rituximab( Rituximab, Rituxan) is a drug of monoclonal antibodies aimed at suppressing the activity of B cells responsible for the development of the inflammatory reaction in myositis;
  • Blockers of TNF( tumor necrosis factor).Suppress the production of TNF - protein compounds involved in the mechanism of triggering an inflammatory response. In the treatment of myositis, Remicad is used for intravenous administration, Enbrel and Humira for subcutaneous administration;
  • Orensia( Orencia, Abatacept): used to treat rheumatoid arthritis. There is as yet no evidence of efficacy in myositis;
  • Interferon-alpha blockers. Drugs that block the activity of interferon-alpha are currently at the initial stage of testing in patients with autoimmune diseases.
  • CellCept is an immunosuppressant used in transplantology.

Forecast

With the right treatment, the majority of patients with polymyositis and dermatomyositis eventually, in whole or in part, restore muscle tone. With fibromyositis, treatment usually does not have a positive effect, but it can significantly slow the progression of symptoms. In this case, patients require auxiliary devices for walking or stroller to move.

Prognosis worsens when associated diseases( pneumonia, cancer) join.

With the timely begun treatment of infectious muscle myositis with the use of antibiotics or immunomodulators, has a positive prognosis. Symptoms of the disease become much less pronounced immediately after recovery from infection. Treatment is usually effective, except in cases of trichinosis. Complicated course of infection, sepsis significantly worsen the prognosis.