Scleroderma: what is it and how to treat it? Causes, symptoms and signs of scleroderma

Scleroderma is very common among autoimmune diseases. What is it and how to treat such ailment? Pathology affects the skin, connective tissue, musculoskeletal system, and in some cases, internal organs. The etiology of the disease is associated with impaired immunity. Protective cells, which normally fight microbes, begin to destroy their own tissues. There is a severe damage to the skin and connective tissue. Often the patient receives a disability with scleroderma. ICD-10 refers this pathology to systemic sclerosis, the disease code is M34.

What is scleroderma?

Among autoimmune processes, scleroderma ranks second in prevalence after lupus erythematosus. The disease is chronic and progressive. Skin and connective tissue undergo scarring, which leads to disruption of tissue metabolism and circulation in small vessels. Collagen is formed in excess quantities, sclerotized and hardens. With the systemic form of the disease, serious damage to internal organs occurs.

Women often suffer from scleroderma. The disease is usually diagnosed in middle age, but often skin lesions are detected in children or adolescents. There are 2 forms of scleroderma: focal and systemic. In the first case, the ailment proceeds more easily.

Focal scleroderma

Focal scleroderma affects individual skin areas. Sometimes there are changes in the bones and muscles. Internal organs are not affected by the pathological process. There is no spasm of blood vessels, which is accompanied by numbness of the fingers( Raynaud's syndrome).The disease is often detected in childhood. More often this affliction affects the girls. Doctors give a relatively favorable forecast with this type of scleroderma. The focal form of the disease is divided into several varieties:

• Ring-shaped. On the skin appear spots that look like plaques. They have a brown color.
• Linear. On the face there are white strips of scar tissue. They have the form of a line.
• Plaque. It appears in the form of redness of skin areas due to the expansion of small blood vessels, as well as seals on the body, arms and legs. In the affected foci, secretion of secretion of the sebaceous glands ceases, the skin pattern disappears and hair falls out.
• Idiopathic atrophodermia of Pasini-Pierini. This rare form of the disease is observed in women of young age. On the back, violet spots are formed, rashes on the face are not characteristic for this type of scleroderma. With time, body formations progress.
• Parieto-Romber's face hemiatrophy. The disease begins in childhood or adolescence. On one side of the face yellow or bluish spots are formed, which creates a strong cosmetic defect. The pathological process can affect not only the skin, but also the musculoskeletal tissue.

• Scleroderma to Bushek. On the skin of the face, neck and shoulders puffiness is formed. They are dense and painful on palpation.
• Teardrop( "white spot disease").Appear white spots on the face of a child or an adult. Rashes are convex, with a pearly hue. The size of the formations is from 0.5 to 1.5 cm. This variant of the disease is often combined with the plaque form of scleroderma. Photo of spots can be seen in the photo.

Systemic scleroderma

Systemic scleroderma is characterized not only by damage to the skin, but also by internal organs. The Raynaud's syndrome is expressed. This form of the disease is divided into the following types:

• Diffuse scleroderma. First, the skin of the whole body is affected, and then the pathological process passes to the internal organs.
• Scleroderma. There are no skin manifestations, painful changes occur only inside the body.
• Juvenile scleroderma. The disease begins in children and adolescents, pathology affects the skin, bones and internal organs.
• Fasciitis eosinophilic( Schulman's syndrome).In the place of passage of veins on the skin, an "orange peel" is formed, as in cellulite. This phenomenon is accompanied by a strong swelling of the extremities.
• Limited scleroderma. On the skin of the legs and hands, small lesions are formed. With timely treatment, they disappear. In the absence of therapy, pathology passes to internal organs.
• Cross-shaped. Scleroderma is combined with other autoimmune processes - systemic lupus erythematosus or rheumatoid arthritis.

Causes of the disease

The causes of this disease are not fully understood. It is assumed that the development of pathology is influenced by genetic factors. There is a hereditary predisposition to autoimmune diseases. This does not mean that the disease manifests itself immediately after birth, pathology can develop at any age.

In people with a tendency to scleroderma to trigger the onset of the disease, the following factors can occur:

• transferred infections;
• hormonal disorders;
• hypothermia( especially frostbite of the hands and feet);
• molecular mimicry of microorganisms, due to which there is an increased activity of lymphocytes;
• hormonal disorders;
• intoxication with drugs or chemical agents;
• is a bad ecology of the place of residence;
• work associated with adverse effects( eg, chemical production).

Environment and human health are closely interrelated. Very often the development of scleroderma provokes residing in the region with a large number of industrial enterprises.especially in the case of the juvenile form of the disease, which manifests itself at an early age. Harmful effects can be provided by silica, white spirit, welding gases, ketones, solvents, trichlorethylene. Not every person whose work is associated with these substances will necessarily develop a disease. But with hereditary predisposition, the risk of scleroderma due to constant contact with chemical compounds is very high.

Skin Symptoms

Symptoms of the disease depend on its variety. Skin changes take place in several stages of development. First there is a dense edema, usually it is located on the hands and face. Then the skin thickens, and in the late stages of pathology, atrophic processes are observed.

The following external signs of the disease can be distinguished:

• Reynaud syndrome. Numbness, burning and tingling of the fingers and toes. Such phenomena are observed even with a slight freezing. The skin of the limbs becomes pale and cyanotic. This symptom is associated with spasm of the vessels of the hands and feet.
• The skin on the fingers becomes rough. Appears scar tissue. Lesions of the skin of the hands are accompanied by thickening and deformation of the fingers. Because of this, the disease is often mistaken for rheumatoid arthritis.
• Around the mouth you can see kisetoobraznye folds.
• Skin pigmentation.
• Feeling of constant chilliness, the patient often freezes and quickly.
• With a drop-shaped form, white spots on the face of a child or adult become the first sign of the disease.
• The expression disappears. The face becomes smooth and maskoobraznym, nasolabial fold is almost imperceptible.

In addition, a person strongly loses weight, quickly becomes tired and complains of a headache. On the body appear rashes. Their color can vary from brown to red. The nature of the rash depends on the form of scleroderma. Photos of the spots can be seen in the photo above.

Manifestations from internal organs

The systemic form of scleroderma affects the entire body. The patient may complain of negative manifestations on the part of different systems. The most common changes are in the kidneys, lungs and heart. Often there are changes in the digestive organs, which is manifested by the following symptoms:

• heartburn;
• flatulence;
• diarrhea;
• ulceration on the walls of the stomach;
• if swallowed;
• disorders of peristalsis;
• deterioration of food processing.

If the pathological process affected the kidneys, the following symptoms may occur:

• rare urination;
• protein and erythrocytes in urine analysis;
• arterial hypertension;
• kidney failure;
• is a disorder of consciousness.

Heart attack may exhibit the following symptoms:

• disorders of left ventricular function;
• myocardial compaction;
• inflammation of the serous and muscular membrane of the heart;
• shortness of breath;
• tachycardia;
• pain in the heart;
• heart failure( rare).

If the lungs are involved in the pathological process, the following manifestations are observed:

• cough without phlegm;
• dyspnoea at physical exertion;
• inflammation of the pleura and lungs with the formation of scars and seals;
• increased risk of lung cancer.

In addition, peripheral nerve lesions may occur. There are pains in the limbs and on the face along the trigeminal nerve. The patient complains of numbness or a violation of the sensitivity of the hands and feet. In severe cases, paresis is possible( paralysis).On the part of the endocrine glands, a decrease in thyroid function( hypothyroidism) is possible.

Prognosis of the disease

Patients are often confused when they hear the diagnosis of "scleroderma."What is it and how to treat this ailment? This question worries the sick. It can be said that to date, this disease is not completely cured. Suspend the increased formation of collagen is impossible. Modern therapy can only slow the development of pathology.

The prognosis for the focal form of scleroderma is more favorable than for the systemic one. The average survival rate for this disease is about 68% for 5 years. The effectiveness of treatment depends on the following factors:

• The sex of the patient. Disease in men has a more unfavorable prognosis.
• Age of the patient. Scleroderma is more difficult to treat in people over 45 years of age.
• Degree of involvement of internal organs. If in the first 3 years of the disease lungs and kidneys were involved in the pathological process, the prognosis of the course of the disease worsens.
• A decrease in hemoglobin in the blood, an increase in ESR and the presence of protein in the urine indicates a severe form of the disease.
• An unfavorable factor is the early onset of the disease before the age of 20 years.

Is scleroderma dangerous? Without treatment, restraining the development of the disease, this pathology can lead to very serious consequences. Autoimmune disease can cause life-threatening complications, such as renal and heart failure, pulmonary hypertension, finger necrosis due to circulatory disorders.

Diagnosis of the disease

To which specialist should I go if the patient has noticed manifestations similar to the symptoms of scleroderma? The rheumatologist is engaged in the treatment of connective tissue diseases of autoimmune origin. After the history, external examination of the patient, listening to the heart and lungs with the help of a phonendoscope, the following examinations will be assigned:

• clinical analysis of blood and urine;
• blood test for antibodies to scleroderma;
• capillaroscopy;
• biopsy of affected tissues;
• X-ray of extremities and internal organs;
• echocardiography;
• study of respiratory function;
• CT to identify changes in tissues.

A rheumatologist will give answers to the patient on all questions about scleroderma. What is it and how to treat the disease will become known after the results of the diagnosis, when the form and nature of the pathology will be revealed. Probably, additional consultation of experts is required: the urologist, the cardiologist, the lung specialist, the dermatologist.

Scleroderma treatment

Scleroderma therapy uses drugs to reduce collagen production, non-steroidal anti-inflammatory drugs, as well as drugs to suppress the work of the immune system.

Of antifibroznyh funds most often appointed "Kuprenil."The drug helps to remove copper from the body. The use of the drug leads to a decrease in scleroderma activity. Copper reduces the production of an enzyme that processes collagen. Therefore, the removal of this substance from the body improves the condition of patients and slows the pathological process in the connective tissue.

Another popular antifibrotic agent is Lidase. This is an enzyme that is obtained from the spermatic glands of cattle. The drug has the property of splitting hyaluronic acid, which is involved in the formation of collagen. The same medicine has the same properties as Longylid.

Often together with "Lidase" antibiotics of group of penicillins are appointed. The exact mechanism of action of these drugs in scleroderma is unknown. However, their use leads to the necessary therapeutic effect. It is assumed that penicillins have antifibrotic and anti-inflammatory effects.

Nonsteroidal anti-inflammatory drugs are prescribed with low disease activity and complaints of pain in the joints. These drugs are Orthofen, Indomethacin, Naize, Ibuprofen, Diclofenac, Piroxicam. In addition to tablets, they can prescribe local anesthetic ointments for application to affected areas.

To suppress immunity apply hormonal drugs and cytotoxic drugs. Glucocorticoids are usually prescribed for high disease activity. Their dosage is carefully calculated, since these drugs can cause side effects such as Itenko-Cushing syndrome( obesity, red moon face, increased blood pressure, disruption of the gonads).To hormonal drugs are:

• "Metipred";
• "Prednisolone".

Cytostatic drugs reduce the production of immune cells: lymphocytes T and B, which are autoaggressive. These medicines include:

• "Azathioprine."
• "Plakvenil".
• "Methotrexate".
• "Cyclophosphane".
• "Cyclosporine."

With high activity scleroderma, pulse therapy is prescribed: intravenous drip injection of large doses of cytostatics and hormones. Conduct symptomatic treatment of scleroderma. Prescribe drugs to improve blood microcirculation "Dipiridamol", "Pentoxifylline", "Iloprost", "Alprostadil", "Warfarin."Often, vasodilators are prescribed: "Amlodipine", "Verapamil", "Nifedipine".

When the digestive tract is affected, antibiotics are indicated: "Erythromycin", "Amoxicillin", "Metronidazole" and the prokinetics of "Metoclopramide".As additional methods of treatment, the doctor can prescribe hemosorption, plasmapheresis, physiotherapy, exercise therapy.

It is important for all patients with scleroderma to remember that in this disease it is very harmful to be in direct sunlight. From sunburn and visit the solarium is better to refuse. Excessive exposure to ultraviolet radiation can nullify the effect of therapy.

Alternative medicine

Often patients are interested in: is it possible to treat scleroderma with folk remedies? Of course, it is impossible to completely heal from such a complex disease with only home remedies. The use of folk remedies is permissible only after consultation with the doctor, as an addition to the main therapy. The following recipes are used:

• Aloe juice is applied as compresses to the lesion sites. You can use ichthyol ointment.
• Baked onions are ground and mixed with one part honey and two parts kefir. This mixture is used for compresses.
• Make homemade ointment from melted pork fat and dry herb wormwood or celandine.
• For the restoration of connective tissue, ingested decoctions of horsetail, litterworms, knotweed.
• With complications on the heart will help decoction of adonis and cyanosis, and with damage to the kidneys are useful compounds with bearberry and a leaf of cranberries.

New developments in the treatment of scleroderma

Medical scientists have long been studying the nature of scleroderma: what is it and how to treat this complex and serious disease. After all, traditional methods of therapy do not lead to the complete elimination of the disease. The search for new methods of treatment is under way, preparations are being developed that can more effectively influence pathology.

A new genetically engineered medicine "Tocilizumab"( "Actemra") was created. This drug is an antibody to the interleukin-6 receptor. It is this anti-inflammatory cytokine that is the provoking factor of scleroderma and rheumatoid arthritis. Clinical studies have shown the effectiveness of the drug.

A new complex method of therapy with the enzyme "Lidase" is proposed in combination with vitamin preparations "Pantogam", "Dexpanthenol" and "Pikamylon".This method is effective in the focal form of the disease. Biologically active substances help improve metabolism in the affected tissues.

Currently, studies of drugs that could block the signaling pathway for the development of scleroderma are being conducted. Investigated molecule inhibitors that can affect the pathogenesis of the disease. This could help prevent fibrosis - proliferation of connective tissue. New in the treatment of scleroderma is also associated with the use of stem cells. They are capable of replacing diseased cells. This leads to a reduction in fibrosis and the formation of new blood vessels in place of those that have been damaged by pathology.

Prevention of

Disease Because of the uncertainty of the exact causes of the disease, specific prevention of scleroderma has not been developed. People with a genetic predisposition to autoimmune pathologies to reduce the likelihood of the disease will help comply with the following rules:

• It is necessary to treat infectious diseases in a timely manner.
• Avoid overcooling and exposure to harmful chemical factors.
• Maintain an active lifestyle to maintain tonus of the musculoskeletal system and connective tissue.

Patients who have scleroderma revealed need to stand on a regular basis and regularly visit a rheumatologist. It is necessary to continuously take prescribed medications, in many cases lifelong maintenance therapy is required.