Sporadic Inclusion Body Myositis

Sporadic Inclusion Body Myositis

Sporadic inclusion body myositis is an inflammatory disease of the muscles. It is one of the myositis.

What is sporadic inclusion body myositis?

Sporadic inclusion body myositis ( sIBM) is an inflammatory muscle disease. Together with dermatomyositis and polymyositis, it belongs to the myositis group. In people older than 50 years, sporadic inclusion body myositis is the most common disease of this type. In Germany, about ten out of a million Germans suffer from myositis. For keratoderma overview, please visit homethodology.com.

Slowly progressive sIBM is a chronic condition that results in proximal and distal muscle weakness. Histologically, typical cytoplasmic inclusion bodies can be found within the skeletal muscles. The mean age at onset of sporadic inclusion body myositis is 55 years. However, the disease can sometimesin old ageof 30 or 80 years.

The male sex is particularly affected by the muscle disease. Around 75 percent of all patients are men. Both near and far muscles are affected by sporadic inclusion body myositis.

Causes

The causes of sporadic inclusion body myositis have not yet been fully elucidated. Pathomechanisms of a degenerative, inflammatory and cell stress-associated nature are under discussion. New studies indicate that there is a link between inflammation and degeneration. Cell stress and degeneration also play a role in the development of muscle disease.

However, the underlying mechanisms have still not been clearly elucidated. There are also assumptions that autophagic processes play a role in the development of the disease. In addition to sporadic inclusion body myositis, there is also a familial form of the muscle disease. This is passed on by recessive or autosomal dominant inheritance.

However, familial forms are much less common than the sporadic form. Typical myopathic changes occur within the muscles of people suffering from sporadic inclusion body myositis. This involves a connective tissue remodeling, fiber caliber variations and central cores.

Another characteristic feature are the inclusion bodies and the vacuoles (cell organelles) that are located in the muscle fibers. In addition, degenerative molecules such as beta-amyloid accumulate. Apart from the degenerative fibers there are also regenerating fibers.

Symptoms, Ailments & Signs

During the course of sporadic inclusion body myositis, the muscles become progressively weaker. The weaknesses often show up in an asymmetrical arrangement. The muscles of the upper arm and forearm are considered to be typical regions of manifestation. The muscles of the hand flexors, finger flexors and knee extensors are particularly affected. This is noticeable through problems when standing up or climbing stairs.

In addition, a weak grip can be felt. The heart muscle is not normally affected, so life expectancy does not suffer from the disease. Symptoms of sporadic inclusion body myositis progress slowly over months or even years.

The sIBM itself does not cause pain. However, the muscle weaknesses result in muscle tension and incorrect strain on the musculoskeletal system, which in turn creates the risk of pain. Difficulty swallowing is not uncommon. In the worst case, the patient may even be dependent on a wheelchair.

Diagnosis & course of disease

The diagnosis of sporadic inclusion body myositis requires extensive investigations, which means that identifying the muscle disease takes more time. First of all, there is a detailed discussion between the doctor and the patient, which is followed by the physical examination. The doctor pays attention to weaknesses in the knee extensors as well as in the hand and finger flexors.

Next step is a blood test. The number of special proteins, including creatine kinase (CK), is checked. The value for the sIBM is three to five times higher than in the normal state. Electromyography (EMG) is another important examination method. The doctor measures electrical voltage changes within the muscle.

This measurement takes place both when the muscle is tensed and when it is at rest. Electroneurography can also be performed to examine the nerves. Muscle biopsy is one of the central diagnostic methods. This is the removal of a tissue sample from the muscle. Typical changes can be identified during the histological examination.

There is no cure for sporadic inclusion body myositis. However, the symptoms of the muscle disease can be improved with physiotherapy treatments.

Complications

Inclusion body myositis develops over years and can cause various complications as it progresses. First, there is a noticeable weakening of the muscles. This restricts those affected in everyday life and professional life, since mostly simple tasks can no longer be carried out. People who work a lot with their hands in their job usually have to stop working. Physical activity is only possible with effort.

The result is a decrease in quality of life and the development of mental illness. In the worst case, the patient is dependent on a wheelchair. The typical swallowing disorders are less serious. In individual cases, however, these can lead to weight loss or trigger aspiration. Such aspiration pneumonia can result in pneumonia. This is accompanied by severe ear and throat pain.

The only effective antidote to inclusion body myositis is immunoglobulin—a drug that can cause a variety of side effects. The most serious complications include allergic reactions and even anaphylactic shock. Swelling and local pain may also occur with intramuscular administration. Hemolytic reactions with symptoms such as anemia, shortness of breath and tachycardia are also conceivable.

When should you go to the doctor?

A doctor is needed as soon as there are abnormalities and peculiarities of the muscular system. A decrease in usual physical capacity and endurance is a concern and needs to be evaluated and treated. If existing muscles decrease in size in a visible form, there is a need for action. Changes in muscles in the upper or lower arm in particular should be discussed with a doctor. If there are restrictions on joint activity, this is also considered a warning signal from the organism and a doctor’s visit is necessary.

If the person has difficulty climbing stairs or getting up from a chair, a doctor should be consulted. Further investigations are necessary so that a diagnosis can be made. Disturbances in cardiac activity, shallow breathing or tachycardia should also be presented to a doctor. If the person concerned suffers from swallowing disorders, pain or a feeling of tightness in the throat, this should be reported to a doctor.

Since the disease leads to the need for a wheelchair in severe cases, a doctor should be consulted as soon as the first irregularities in locomotion or limitations in the usual possibilities of movement occur. The characteristic feature of sporadic inclusion body myositis is the slow increase in symptoms. Patients usually report that the existing irregularities increase in intensity and extent over months or years.

Treatment & Therapy

So far, no effective therapy concept has been developed that would allow sporadic inclusion body myositis to be cured. In contrast to polymyositis and dermatomyositis, treatment with cortisone has no effect whatsoever. Tests with immunosuppressants such as cyclosporine A, methotrexate or azathioprine were also unsatisfactory.

There is hope of improving the course of the disease by administering immunoglobulins. However, further studies are required on this therapeutic concept. Physiotherapy exercises are considered to be effective in improving the symptoms of the disease. Aerobic endurance training and light muscle training are particularly effective. The strength values ​​can be increased in this way.

One of the most important tasks of physiotherapy is to enable the patient to walk safely. Walking aids can also be used. Occupational therapy is also important to strengthen hand functions. In the case of swallowing disorders, logopedic treatment must be carried out. Swallowing can be made easier by adopting special postures.

In order to maintain the patient’s physical abilities as long as possible, a combination of physiotherapy, occupational therapy and speech therapy is recommended.

Prevention

There are no preventive measures against sporadic inclusion body myositis. The causes of the muscle disease are still unknown.

Aftercare

Sporadic inclusion body myositis is chronic and cannot be cured causally. However, symptomatic follow-up care can be provided, which reduces or alleviates the various complaints. The disease affects and weakens the muscles. This leads to various limitations in everyday life and in severe cases a walker or wheelchair may be necessary.

Therefore, physiotherapeutic treatment is advisable. The special physiotherapy tries to maintain maximum mobility and reduces pain caused by muscle tension and incorrect strain. Occupational therapy also helps to strengthen hand functions.

The decrease in quality of life caused by sporadic inclusion body myositis can lead to mental problems and, in some cases, depression. To improve this condition, continuous consultations with the psychologist should be introduced. Drug treatment with immunoglobulin is possible, but unfortunately causes a variety of side effects such as allergic reactions, swelling, shortness of breath, tachycardia and, in the worst case, anaphylactic shock.

Therefore, you should consult your family doctor. Regular follow-up visits are also important to monitor the progression of the disease. In the case of disease with sporadic inclusion body myositis, life expectancy is not negatively affected. However, the disease progresses steadily, so the symptoms can increase over the years.

You can do that yourself

Sporadic inclusion body myositis is an incurable chronic disease. It is therefore important for those affected to deal with the clinical picture. They receive help primarily in self-help groups and internet forums for people with chronic muscle diseases.

Most of those affected can no longer go about their everyday lives in the usual way. A professional reorientation or retirement is often necessary. The patients depend on the support of relatives. In addition, it is usually necessary to convert the apartment of the person concerned to make it suitable for the disabled.

The therapeutic measures for the treatment of sporadic inclusion body myositis can be supported by a number of self-help measures. In order to maintain the mobility of the affected person for as long as possible, light physical activity can be helpful in addition to physiotherapy. When sporadic inclusion body myositis begins, regular, short walks or swimming can have a positive effect on the course of the disease.

In the case of advanced symptoms, however, it is necessary for the patient to become familiar with the necessary supports such as a walking frame or a wheelchair at an early stage. But even in advanced cases, it is advisable to maintain and promote the remaining physical mobility. Those affected should try to take a few steps with the help of relatives. Ideally, short distances in your own home are ideal for this.

Sporadic Inclusion Body Myositis