Collagenoses deutsche rheuma liga bundesverband e. V

The guidebook "Collagenoses – when rheumatism gets under the skin" (2019) of the German Rheumatism League offers specialist information on clinical pictures and provides practical tips for everyday life.

The disease – what are collagenoses?

It used to be thought that collagenoses were mainly caused by a change in the connective tie. Today we know that they are systemic autoimmune diseases that can also attack the blood vessels. Typically, various internal organs, skin and joints are affected at the same time or one after the other. The diseases are chronic.

Collagenoses are rare. Women are much more likely to develop them than men. The most common collagenosis is Sjogren's syndrome. Another form, systemic lupus erythematosus (SLE), occurs primarily in women between the ages of 25. and 35. It occurs at the age of. Polymyositis and dermatomyositis are also two to three times more common in women than in men. Mixed connective tie disease (MCTD) is a special case. It combines symptoms of systemic sclerosis, lupus, or other collagenoses.

It is impossible to predict how severe the symptoms will be. There may be constant relapses. It is also possible that the disease does not manifest itself for months.

Causes – how collagenosis occurs?

Collagenoses are autoimmune diseases. In this type of disease, a person's defense system is directed against his or her own body. The immune system considers healthy body cells to be diseased or foreign and fights against them. Why it cannot distinguish friend from foe in these cases is not known.

However, scientists have been able to identify some causative agents. In all likelihood, female sex hormones play an important role in the development of systemic lupus erythematosus, for instance. Sunlight is almost certainly a trigger for systemic lupus erythematosus. Researchers also know this today. Some drugs can also cause a clinical picture similar to lupus. In other collagenoses such as systemic sclerosis. Nothing is known about the causes of Sjogren's syndrome. Hereditary factors as well as environmental factors (mineral dust or infections) are thought to play a role.

In rare cases, malignant tumors can cause collagenosis, such as in polymyositis or dermatomyositis. Polymyositis is an inflammation of the skeletal muscles. If the skin also changes in addition to the inflamed muscles, this is called dermatomyositis.

The various collagenoses manifest themselves in different ways.

Sjogren's syndrome
Sjogren's syndrome is mainly manifested by decreased tear and saliva production due to disease of the tear and salivary glands with the following symptoms:

– dry mouth – problems with swallowing and prolonged speech – problems with teeth (caries) – cracked tongue – dry eyes, lack of lacrimal fluid – dry mucous membranes

When parotid glands become inflamed and swell as a result, the cheeks become wider, similar to Goat Peter's disease.

General signs of the disease are also typical of Sjogren's syndrome, such as:

The syndrome may also spread to other organs. The pancreas may become inflamed. This can lead to digestive disorders.

Occasionally, nerve damage results in unilateral facial pain (trigeminal neuralgia).

Systemic lupus erythematosus
Systemic lupus erythematosus often initially announces itself by hypersensitivity to sun exposure. After sunbathing, the skin is redder than usual. Various joints (fingers, hands or knees) hurt and sometimes even swell up.

If the skins around the heart or lungs have become inflamed (pleurisy or pericarditis), patients feel stabbing pains in the chest that make breathing difficult. These diseases must be treated immediately.

Often the kidneys are also affected. A foaming urine may be a clue. It also happens that the nerves are affected (neuropsychiatric deficits). Blood clotting may be disturbed in a group of patients. This can result in thrombosis or miscarriage. Many patients also show the additional symptoms of Sjogren's disease. Almost all patients complain of various general symptoms. They feel tired and fatigued, they are cold, they have a fever and lose weight.

Systemic sclerosis
The typical features include Raynaud's syndrome. The blood vessels of the fingers become tense. The fingers first become white (as if dead), then blue, finally red, when the blood circulation starts again. This painful circulatory disorder can cause tie defects at the fingertips (necrosis).

Systemic sclerosis is also manifested by a more or less extensive thickening and hardening of the skin – especially on the hands, legs and face. At the beginning of the disease the fingers tend to swell, in the course they become thinner. This explains the expression "Madonna's finger". Typical for systemic sclerosis is the mask-like facial expression. The disease restricts facial expression. Patients can no longer open their mouths properly. Many feel that the skin is like a tank because it can only be moved to a limited extent.

The esophagus may also be affected. Sufferers complain of difficulty swallowing and heartburn. Especially the lungs cause problems for many patients. Shortness of breath may occur. Systemic sclerosis also includes typical general symptoms such as fatigue and exhaustion.

Polymyositis and dermatomyositis
Polymyositis as well as dermatomyositis are often first noticed by general symptoms:

– fatigue – exhaustion – weight loss – fever

In addition, those affected complain of:

– Increasing muscle weakness in the shoulder, arm and pelvic muscles – Problems climbing stairs, getting up from a chair or combing hair. – Difficulty swallowing when the muscles in the esophagus are affected.

Dermatomyositis sometimes masks a cancerous disease. Therefore, in this case, doctors should carefully look for tumors – especially in the ovaries, skin and chest.

The rheumatologist interviews the patient and examines the body first of all for the typical symptoms of the disease. It uses laboratory, ultrasound and X-ray examinations. It checks the affected organs with the help of functional tests. And it prompts microscopic examination of organ tie – for example, kidney tie – taken by biopsy. The symptoms of the different collagenoses can vary greatly.

In systemic lupus erythematosus, many patients suffer from kidney. Inflammation can be detected with a urine test. The first visible signs that something is wrong are swollen ankles due to water retention. If the urine foams, this is an indication of a high protein content. Then the patient should contact a physician.

If the patient suffers from muscle weakness, the cause may be polymyositis or dermatomyositis.

Typical signs are elevated muscle enzyme levels in a laboratory test. With ultrasound examinations the doctor gets on the track of the diseased muscles. Magnetic resonance imaging (MRI) can also provide early evidence of affected muscles. Electromyography is used to check whether a muscle shows unusual electrical activity. A tie sample from the muscle (biopsy) can prove that inflammatory processes are taking place within the muscle.

Today, certain autoantibodies can confirm the diagnosis so clearly that a muscle biopsy is not always necessary. Antibodies always provide indications of collagenosis. The so-called antinuclear antibodies (ANA) are typically detectable in the blood in collagenoses. The exact type of antibodies gives information about the different collagenoses.

In active systemic lupus erythematosus, antibodies against the genetic material DNA can be detected in the blood. In patients with Sjogren's syndrome, on the other hand, other subgroups of ANA (anti-Ro and anti-La antibodies) can be found.

Collagenosis cannot be cured, but there are now many medications that are effective. Two things are important for successful therapy:

– an experienced doctor who specializes in collagenosis – a patient who can assess his condition well

1. Drug collagenosis therapy

The treatment of collagenoses depends on whether or not there is a disease flare-up at the moment. It is still dependent on the organ affected. The basis of most therapies is a combination of cortisone and other drugs that suppress the immune system (immunosuppressants). cortisone inhibits inflammation in the body. For the treatment of collagenosis itself doctors use other preparations. They have a stronger effect and fewer side effects than cortisone.

Cortisone
Cortisone is used in low doses over the long term – or in high doses during flare-ups, but then only for a very short time. In the treatment of systemic sclerosis, high doses can even be dangerous. Cortisone treatment can have various side effects:

– Increased risk of infection – Osteoporosis – Diabetes – Weight gain – Shape changes

However, these side effects can be well controlled today. So-called non-steroidal anti-rheumatic drugs (NSAIDs) inhibit pain. inflammation within hours. However, they cannot stop the disease. If NSAID treatment is combined with cortisone, gastric ulcers may occur. Cardiovascular or kidney diseases can also worsen when taking NSAIDs.

Anti-malarial drug
The anti-malarial drug hydroxychloroquine is used in almost all patients with systemic lupus erythematosus. It is a "multi-talent" that also has many other good effects on lupus patients. Because eye damage can occur at high doses (especially with chloroquine, which was used in the past), regular checks by an ophthalmologist are important.

Cyclophosphamide
The drug cyclophosphamide prevents cells from multiplying. It originates from cancer therapy, is very effective, but has strong side effects, so that it is only used for severe forms of systemic lupus erythematosus.

Biologics
A great hope are biotechnologically produced drugs that can specifically combat inflammation in the body. The antibody belimumab is approved for the treatment of lupus erythematosus, which intercepts a messenger substance and thus interrupts an amplification loop of the autoimmune disease. Rituximab is another antibody approved for the treatment of rheumatoid arthritis and vascular inflammation.

Immunosuppressants
The active substance azathioprine suppresses the misdirected immune system. As a rule, it is well tolerated. Doctors prescribe methotrexate (MTX) in order to be able to manage with lower doses of cortisone. It inhibits inflammation very effectively. Can be taken for decades. However, the drug is not suitable for patients with kidney dysfunction.

2. Non-drug collagenosis therapy

Physiotherapy is very important, especially in the case of polymyositis or dermatomyositis. It helps build muscle strength, and it prevents muscles from stiffening. In the case of systemic sclerosis, in addition to medication, massage, physiotherapy, occupational therapy, respiratory gymnastics and exercise therapy are used. It improves blood circulation and prevents skin and joints from becoming stiff.

The patient must cooperate constantly. He must take care of his skin. Do exercise. It is also important that it protects the skin and joints from the cold. It should also not smoke or be exposed to cigarette smoke.

A healthy diet can have a positive effect on the course of collagenosis. Mediterranean food, for example, is recommended – especially when prepared with high-quality vegetable oils. Sea fish is also advisable. It is rich in omega-3 unsaturated fatty acids, which lower cholesterol levels. Meat should not be on the menu more than twice a week. It contains an acid that promotes inflammation.

Especially patients taking cortisone should be careful with sweets. Cortisone increases blood glucose levels. Carbohydrates rich in fiber, such as those found in whole grain products, legumes or potatoes, are healthier.

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