Systemic scleroderma

Systemic scleroderma is a chronic disease affecting the skin and internal organs, the main sign of which will be the occurrence of sclerotic changes on the skin — scar tissue. Scleroderma is considered an autoimmune disease that appears due to a malfunction of the immune system, as it suddenly begins to destroy its own cells, and not foreign ones. As a result, this leads to an inflammatory reaction that provokes thinning of the skin, some internal organs and blood vessels.

The content of the article:
Causes of systemic scleroderma
Symptoms of systemic scleroderma
Treatment of systemic scleroderma
Systemic scleroderma
Scleroderma is considered a very serious disease, which is often accompanied by many complications. Patients are forced to follow a special diet, be careful during physical activity, monitor the degree of hydration of the skin. Often, patients with this disease experience psychological discomfort, because they are oppressed by the thought that the disease is chronic and incurable. Also, scleroderma is manifested by serious aesthetic shortcomings, due to which a person’s self-esteem suffers.

Causes of systemic scleroderma
Scleroderma is considered an acquired disease, the causes of which have not yet been fully studied. However, scientists still managed to find several factors that can provoke the disease. These factors include the following:

The genetic factor. Scleroderma is not inherited. However, it is believed that people who are related to patients have a very high risk of the disease. It is believed that the disease may occur due to gene mutation. Such mutations can occur under the influence of the following factors: ultraviolet radiation, ionizing radiation, nitrates, pesticides, food additives, viruses, bacterial antigens. At the same time, it is worth remembering that genome changes usually occur in representatives of the second generation.
Inflammatory factor. The trigger for starting the disease can be inflammation. During inflammation, the body activates the production of biologically active substances that affect tissues and cause serious changes in blood vessels and cells. As a result, the permeability of blood vessels increases significantly, and conditions are created for the activation of cells synthesizing connective fibers. Then an inflammatory reaction occurs, which triggers the disease.
Autoimmune process. It is a consequence of a serious violation of immunity. Genetic anomalies, as well as various pathogenic agents similar in structure to human proteins, can provoke its occurrence. Due to an autoimmune reaction in the human body, peripheral tissues are infiltrated by lymphocytes and macrophages.
Infectious factor. Some infectious agents can negatively affect the immune system and trigger autoimmune reactions, thereby provoking the appearance of systemic scleroderma. Antibodies can have a negative effect on the body: activate the production of fibroblasts, trigger the proliferation of vascular musculature, form a new vascular sheath.
Environmental factors. In the course of long-term studies, the presence of certain geographical zones in which the risk of the disease is especially high has been studied and proven. A detailed study of these zones has shown that there are specific environmental factors in them that can trigger the development of scleroderma. Namely, these factors include the excess of the following chemicals: silicon, mercury, vinyl chloride, benzene, trichloroethylene, paraffin, epoxy resin.
Medications. It has been proven that many medications provoke the development of the disease under certain conditions. Local lesions are often caused by drugs such as pentazocin, phytomenadion, heparin. In addition, the following medications are also dangerous: bleomycin, penicillamine, diltiazem.
Symptoms of systemic scleroderma
The signs of systemic scleroderma are different, since they mainly depend on which organ was affected the most. In addition, the symptoms of the disease often depend on its stage. Symptoms such as malaise, joint and muscle pain, and fatigue are characteristic of the initial stage of the disease. In case of damage to internal organs, heartburn and shortness of breath may appear. The symptoms of systemic scleroderma can be divided into several groups: lesions of the skin, internal organs and blood vessels.

Skin lesion
Often, the disease begins with inflammation, which is accompanied by the release of a huge amount of biologically active substances, which provokes edema. Because of this, the fingers, feet and hands increase significantly in their volume, and their swelling is also observed. Sometimes itching can even develop. This stage of the disease lasts for several weeks, after which the stage of compaction begins. Namely, the patient has a compaction of the connective tissue structure and its replacement with a pathological one.

The final stage of this process is atrophy: soon the skin becomes thin, loses its natural shade, easily cracks. Patients have lesions of the skin of the abdomen, chest, thighs, feet and shins. In rare cases, the lesion may affect the limbs and trunk. Depending on the type of foci on the skin, the following forms of scleroderma are distinguished: plaque, linear, bullous, limited, white spot disease. Patients with systemic scleroderma also have signs such as ulcers on the fingers, atrophy of the muscles of the hand and muscles of the lower extremities, thickening of the skin of the neck and the skin around the mouth.

Defeat of internal organs
Systemic scleroderma has a detrimental effect on many internal organs (especially the liver, heart, lungs, kidneys, genitourinary system, gastrointestinal tract, bones, muscles). The most common sign of the disease is a violation of the gastrointestinal tract. In this case , the patient usually complains of the following symptoms of the disease:

problems with swallowing;
heartburn;
narrowing of the esophagus;
bloating;
diarrhea;
stomach pain;
intestinal colic;
vomiting blood;
rectal prolapse;
intestinal bleeding.
The disease can have a detrimental effect on the functioning of the liver, as well as lead to the appearance of cirrhosis. One of the most common causes of death in patients with scleroderma is considered to be lung dysfunction. Patients often have such serious pathologies as pulmonary hypertension and interstitial lung disease. Often, patients with scleroderma complain of heart problems: shortness of breath during physical exertion, pain behind the sternum, dizziness, cardiac arrhythmia, swelling of the jugular veins, palpitations.

The disease provokes a violation of the functioning of the kidneys and even the development of renal failure. Because of this, the amount of filtered blood is significantly reduced, decay products accumulate in the body, thereby causing very adverse consequences. It is believed that the very first signs of the disease in patients are associated with pathologies of the musculoskeletal system. Often the lesion covers the joints of the hands and fingers. Disruption of the genitourinary system can be accompanied by symptoms such as erectile dysfunction, pain during sexual intercourse, decreased sexual desire, premature menopause.

Vascular damage
In most patients with scleroderma, Raynaud’s syndrome is detected — this is a strong spasm of the peripheral arteries, which is usually localized in the area of the hands. At the same time, the patient has a three-phase change in the skin tone of the fingers.

At first, the patient’s fingers turn pale — at the same time, the border between the brush of a normal shade and a pathological one is clearly visible.
Then the fingers begin to turn blue due to poor supply of oxygen and essential nutrients, as a result of which hemoglobin accumulates in them.
Then comes the redness of the fingers after the elimination of vascular spasm and reactive fullness.
In addition to changing the color of the fingers, patients may experience numbness or tingling. If the disease is not treated for a long time, it can result in the occurrence of symptoms such as dry gangrene of the fingers, ulcers that heal poorly on them and even their death.

Treatment of systemic scleroderma
To diagnose systemic scleroderma, laboratory tests are prescribed (general and biochemical blood tests, determination of the level of NT-proBNP and CXCL4, general urine analysis), radiological examination methods, electrocardiography, ultrasound, gastrointestinal endoscopy, histological examination.

The choice of tactics and methods of treatment depends on the type of disease, as well as the degree of damage to internal organs. The following medications are prescribed for the treatment of systemic scleroderma:

enzyme preparations — reduce the rate of formation of sclerotic foci, restore skin elasticity, reduce the hardness of the skin integuments;
vasodilators — eliminate spasm of blood vessels, which in turn has a good effect on blood circulation in internal organs;
immune suppressing drugs;
anti—inflammatory drugs – reduce inflammation and accompanying symptoms such as pain, swelling and joint damage.
During the treatment of patients, it is important to follow a special diet. Namely, you should limit yourself to solid food in case of problems with the digestive tract. It is desirable that the patient’s diet contains food that is rich in plant fibers, vitamins and minerals. If the patient has kidney damage, it is necessary to significantly reduce the amount of fluid intake. Also, patients should avoid excessive doses of vitamin C.

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