Symptoms and treatment of a rare disease -myositis

Update: October 2018

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

  • Joints
  • A heart
  • Lungs
  • Intestines
  • Skin

Частота развития myositis: 1 случай на 2 млн населения. IN USA
registered 135 patients with myositis.

It is a systemic disease that affects the muscles in all parts of the body,
but cases are described where myositis developed only in separate
limbs (one arm or leg) or, for example, just a muscle,
moving eyeball. In such cases, use narrower
terms, for example, cervical myositis, the symptoms of which relate to
only dysfunction of the muscles in the cervical region.

In some patients, myositis occurs as short-term
a disorder that goes away after a few days or weeks. For
others is a disease that has to be fought on
lifelong.

Chronic course with improper treatment or lack thereof
can cause muscular atrophy and disability.

The reasons

Среди причин myositis выделяют:

  • Systemic autoimmune diseases (lupus, sclerodema,
    rheumatoid arthritis). Течение myositis в таких случаях затяжное и
    requires long-term treatment;
  • Infections. The most common causes are
    viral infections. Less commonly – bacteria, fungi or protozoa. Viruses and
    bacteria are able to penetrate directly into the muscles as well
    have a damaging effect by producing toxins. Viruses
    Colds, flu, and HIV are some of the many that can cause
    симптомы myositis;
  • Medicines. Many medications
    can cause temporary damage to muscle fibers. Such
    the condition is called myopathy, but in the absence of adequate
    treatment in the damaged muscle develops an inflammatory process. TO
    лекарствам, повышающим риск развития myositis, относятся статины,
    colchicine, plaquenil, alpha interferon, as well as cocaine and
    alcohol.

Myopathy may develop immediately after the onset.
medication, and after several months or years
with prolonged use. Sometimes this condition occurs in
result of improper dosing or simultaneous use
several incompatible drugs. However, myositis,
caused by the drug, rarely turns into severe
form.

  • Injury. Excessively intense exercise can lead to
    the appearance of muscle pain, edema, and weakness that lasts for
    few days after training. Since swelling and pain are
    signs of inflammation, this condition can be regarded as mild
    форму myositis, которая разрешится без последствий после короткого
    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 –
disease of older people over 50 years.

Symptoms

Основной симптом myositis – мышечная слабость. At the same time weakness
may be pronounced or detected only during
special tests. The loss of muscle strength increases gradually in
for several weeks or months. The disease affects large
muscles or muscle groups of the neck, shoulders, hips and back. When muscle myositis
symptoms usually develop symmetrically on both sides. Wherein
the patient cannot lift a heavy object, climb stairs or
even raise your hand or get dressed. Миозит

The most severe inflammation of the shoulder and pelvic muscles.
Больной с прогрессирующим течением myositis может часто падать и
have difficulty lifting from the floor or chair. Other
Symptoms characterizing myositis include:

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

Признаки myositis шеи характеризуются затруднением при повороте
or tilts of the head, as well as difficulty in swallowing.

Patients with myositis caused by a virus have symptoms.
infectious disease (fever, cough, runny nose, nausea,
diarrhea, etc.). But signs of infection may pass long before
появления симптомов myositis.

Some patients complain of muscle pain, but most
of these, this symptom does not develop due to inflammation, but as a result
injuries or other illnesses (flu, etc.).

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

Dermatomyositis is also systemic muscle disease but
в отличие от полиmyositis, начинается более остро и поражает как
adults and children alike. Symptoms заболевания схожи с описанными
above, but accompanied by skin manifestations – the appearance
rash

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

This form differs from the disease in adults in concomitant
development of vasculitis (inflammation of the blood vessels), deposition
calcium salts in tissues and impaired metabolism of fatty compounds.
In this form, skin lesions and muscle weakness develop.
at the same time, the severity and progression of each of the symptoms
each patient may vary significantly.

Fibromyositis is more common in older people and is characterized by
slow progression. There are cases when from the moment
the occurrence of the first symptoms before diagnosis was 5-6
years old. More than 20% of patients have problems with swallowing.
Muscle weakness does not always develop equally in symmetrical
muscles and may prevail in the small muscles of the gastrocnemius muscles and
forearm muscles.

Treatment

Depending on how manifest and how pronounced
myositis symptoms, treatment may include
immunosuppressants, antibiotics, antiviral drugs and
etc.

Тактика лечения myositis подбирается индивидуально в зависимости
from the cause of the disease, severity and individual
features of the patient.

For воспалительных заболеваний, вызвавших миозит, лечение
is carried out with the use of immunosuppressants:

  • Prednisolone
  • Azathioprine (Imuran)
  • Methotrexate

Специфического лечения myositis вирусной этиологии не существует.
With the development of the disease on the background of bacterial infection in the treatment
add antibacterial drugs.

For лечения myositis, возникшего после применения лекарственного
means, just cancel it. Normalization condition
comes in about 2-3 weeks.

  • TOортикостероиды

Hormonal drugs are used to reduce
inflammatory response. The most common application
prednisolone. Also prescribe cortisone, hydrocortisone,
methylprednisolone, triamcinolone, dexamethasone and betamethasone. AT
in large doses, these drugs inhibit the activity of the immune system,
which allows to reduce the manifestations of the underlying disease, but
increases the risk of developing infections. Corticosteroid dosage
calculated depending on the severity of the disease.

Patients receiving hormones should at least once a year.
be examined by an ophthalmologist for symptoms
cataracts. TOроме того, существует риск локального нарушения
circulation with subsequent development of bone necrosis. It is heavy
complication of hormone therapy, accompanied by severe pain
sensations. When new sources of pain appear, immediately
consult your doctor.

  • Азатиоприн (Imuran)

Azathiopin – an immunosuppressant taken orally (capsules) by
50-150 mg / day. This drug inhibits the production of red blood cells and
leukocytes, therefore during treatment it is necessary monthly
perform a blood test. TO другим побочным эффектам
include hair loss, liver damage, nausea, vomiting,
the accession of secondary infections.

  • Methotrexate (Ревматекс, Rheumatrex)

The drug is similar in mechanism of action with azathioprine. Side effects
the effects are the same.

  • Plaquenil

Plaquenil оказывает иммунодепрессивный и противовоспалительный
Effect. It is used mainly in the elderly, patients
dermatomyositis.

  • Gamma immunoglobulin

This is a drug derived from human plasma. He is already over 15
years used to treat various diseases. With myositis he
used to reduce the level of creatine phosphonase – an enzyme,
appearing in large quantities in the blood during muscle breakdown
of fibers.

Side effects of treatment are:

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

Immunoglobulin is used for myositis of viral etiology.
Usually used as a reserve drug when other methods
proved ineffective.

  • Циклофосфамид (Cytoxan)

Cyclophosphamide – an immunosuppressant with a more pronounced
activity compared with the above preparations. With another
side, there is a higher risk of side effects from
its application. TO особенностям лечения относится необходимость
strict control of the injected and excreted fluid. A drug
irritates the bladder, so the patient
Requires increased fluid intake.

Physiotherapy and exercise

Treatment при миозите не будет эффективным без физиотерапии. it
necessary to maintain muscle tone and increase
endurance. Physiotherapy provides prevention
muscle atrophy, which greatly improves the quality of life
patients.

Even slight increase in physical activity during the day
can bring tangible positive results. TO примеру, лечение
back muscles are effectively performed by swimming or even
simple work in the garden. The amount of exercise is easily adaptable for
each individual patient.

Key training recommendations include:

  • Mandatory workout. Muscles need to be warmed up for
    ensure normal blood flow improve nutrient intake
    substances and increase the contractile work of the heart;
  • Do exercises in the mode that allows you to not
    overstrain. Do not train through strength;
  • After a workout, rest, restore breathing and pulse.

A single training program does not exist. For каждого пациента
requires an individual selection. TO примеру, лечение myositis шеи
done with resistance exercises: sit up straight,
Look forward, put your hand on your forehead and tilt your head forward,
at the same time, with your hand, create the opposite resistance. Repeat the same
but with the head tilted back, then to the sides.

Consider that even this seemingly simple method requires
daily adjustment with daily performance evaluation
conducted exercises.

Additional exercise recommendations include:

  • Slow start with a gradual increase in pace;
  • TOонцентрацию на больной мышце. If you feel that too
    load the problem area, do not continue training,
    take a rest;
  • Do not lose the enthusiasm. ATо время лечения обязательно будут
    attend hard days. ATыработайте программу для хороших и
    bad days;
  • Practice with a partner.

Rehabilitation specialists for patients with muscle diseases
recommend aquatherapy. Such лечение myositis у детей особенно
effectively and often allows to restore completely in a short time
lost physical activity and avoid disability.

Prospective studies

Исследования касательно myositis и других аутоиммунных миопатий
continue continuously. Usually new methods of diagnosis and treatment
myositis внедряются в результате открытий в смежных областях
(rheumatoid arthritis, lupus).

Promising areas of treatment that will be available in
future are:

  • Rituximab (Rituximab, Rituxan) – a monoclonal drug
    antibodies aimed at suppressing the activity of B-cells that respond
    for the development of an inflammatory reaction in myositis;
  • TNF blockers (tumor necrosis factor). Suppress the production of TNF
    – protein compounds involved in the launch mechanism
    inflammatory response. AT лечении myositis применяются: Ремикад
    (Remicad) for intravenous administration; Enbrel and Humira for
    subcutaneous administration;
  • Orencia (Orencia, Abatatsept): used to treat
    rheumatoid arthritis. Evidence based on effectiveness
    with myositis yet;
  • Interferon-alpha blockers. A drugы, блокирующие активность
    interferon-alpha is currently in its initial stages
    tests in patients with autoimmune diseases.
  • CellCept is an immunosuppressant used in
    transplantology.

Forecast

With proper treatment, most patients
polymyositis and dermatomyositis eventually fully or partially
restore muscle tone. With fibromyositis, treatment is usually not
has a positive effect, but allows significantly
slow the progression of symptoms. Wherein пациентам требуются
walking aids or strollers for
movement.

Forecast ухудшается при присоединении сопутствующих заболеваний
(pneumonia, cancer).

При своевременно начатом лечении инфекционного myositis мышц с
using antibiotics or immunomodulators, has
positive outlook. The symptoms of the disease are becoming much
less pronounced immediately after recovery from the infection. Treatment
usually effective, except in cases of trichinosis. Complicated
the course of infection, sepsis significantly worsen the prognosis.

Автор: TOоробов Никита Александрович врач-анестезиолог

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