Systemic scleroderma - Causes, symptoms and treatment. MF.

Systemic scleroderma is a disease affecting various organs, which is based on a change in connective tissue with a predominance of fibrosis and damage to the blood vessels by the type of obliterating endarteritis.

The incidence of systemic scleroderma is approximately 12 cases per 1 million people. Women are sick seven times more often than men. The disease is most common in the age group 30-50 years.

Causes of systemic scleroderma

Often the disease is preceded by factors such as infections, hypothermia, stress, tooth extraction, tonsillectomy, hormonal changes in the body of a woman( pregnancy, abortion, menopause), contact with toxic chemicals, vaccination.

The exact cause of the disease is not established. Currently, one of the main is the theory of genetic predisposition. Family cases of the disease have been established. In addition, relatives of the sick person have a higher incidence of other rheumatic diseases( rheumatoid arthritis, systemic lupus erythematosus) compared with the general population. In favor of the theory of the viral effect, reveals the changes in immunity associated with the activity of viruses( especially retroviruses and herpes viruses).But a specific virus stamp that causes systemic scleroderma has not yet been found.

Symptoms of systemic scleroderma

The main symptom of the disease is the increased function of fibroblasts. Fibroblasts are the main cells of connective tissue that synthesize collagen and elastin, whereby the connective tissue is very strong and elastic at the same time. When the function is increased, fibroblasts begin to produce collagen in large amounts, fibroblasts are increased. In the finals in various organs and tissues foci of sclerosis are formed. In addition, fibrotic changes affect the vascular wall, which thickens. Obstacles to the blood flow are created, and as a consequence, thrombi are formed. Such changes in blood vessels lead to disruption of normal blood supply to tissues and the development of ischemic processes.

Connective tissue is widely represented in the body, therefore, with systemic scleroderma almost all organs and tissues are affected. Therefore, the symptoms of the disease are very diverse.
In acute acute progression of the disease, the development of sclerotic changes in the skin and fibrosis of internal organs is characteristic for one to two years from the onset of the disease. With this variant, a constantly high body temperature, a loss of body weight, appears very quickly. The mortality rate of patients with acute fast-progressive variant is high.

For the chronic course of systemic scleroderma, the initial signs of the disease in the form of Raynaud's syndrome, lesions of the skin or joints are characteristic. These manifestations can be isolated for many years. Subsequently, symptoms of internal organs damage appear in the clinical picture.

Skin lesion is the most common symptom of systemic scleroderma and occurs in most patients. Initially, the skin of the face and hands is affected. In typical cases, sclerodermal changes go through the stages of skin tightening due to edema, followed by induration( skin tightening due to fibrosis) and partial tissue atrophy. At the same time, the skin on the face becomes dense and motionless, due to its tension, kisetoobraznye wrinkles around the mouth are formed, the face acquires a resemblance to a mask.

Face mask with systemic scleroderma

Sclerodactyly is also a characteristic feature of the disease. At the same time, skin firming of the wrists is formed with the development of deformation of the fingers( "sausage-like" fingers).

Sclerodactyly

Along with densification of the skin, trophic disorders are also manifested in the form of ulcers, suppuration, deformation of nail plates and the appearance of foci of alopecia.

Vascular disorders of are the most common initial symptom of the disease. The most common are vasospastic crises( Raynaud's syndrome).In this case, under the influence of cold, excitement or in the absence of external causes, narrowing of small vessels, usually of hands, occurs. This is accompanied by numbness, pallor, or even blue fingertips. With the progression of the disease due to tissue ischemia, long-lasting non-healing ulcers( "rat bites") form at the fingertips. In severe cases, necrosis of the last phalanges of the fingers develops.

Necrosis of terminal phalanges of fingers as a complication of Raynaud's syndrome

Joint damage to is manifested by pains in them, morning stiffness, tendency to flexion deformities due to compaction and tissue atrophy around the joint. When you feel the affected joints above them, you can determine the noise of the friction of the tendons. Systemic scleroderma is characterized by a tightening of the muscles, as well as their atrophy. Bone disease is manifested by osteolysis( destruction) of the bones of the fingers with shortening of the phalanx.

Osteolysis of distal phalanges of fingers

The most vulnerable digestive system organs in systemic sclerosis are the esophagus and intestine .In the esophagus due to the condensation of its wall, sclerotic deformity is formed with a violation of the normal passage of food. Patients complain of a feeling of coma behind the sternum, nausea, heartburn, and the urge to vomit. If there is considerable deformity, surgery may be required to expand the lumen of the esophagus. The intestine is less often affected, but the symptoms of its disease significantly reduce the quality of life of patients. In the clinical picture, pain, diarrhea, and weight loss prevail. Constipation is common for the defeat of the colon.

Lesion of the lungs is currently coming to the forefront among the causes of mortality of patients with systemic scleroderma. There are two types of lung lesions: interstitial disease-fibrosing alveolitis and diffuse pneumosclerosis, as well as pulmonary hypertension. External manifestations of interstitial lesions are nonspecific and include shortness of breath, dry cough, general weakness, rapid fatigue. Pulmonary hypertension is manifested by progressive dyspnea, the formation of blood stagnation in the lungs and heart failure. Often, pulmonary thrombosis and acute right ventricular failure cause death of patients.

Scleroderma is characterized by the defeat of all layers of the heart. With myocardial fibrosis, the heart increases in size, stagnation of blood in the cavities is formed with the development of heart failure. Very often, due to a violation of the innervation of the enlarged heart, patients develop arrhythmias. Arrhythmias are the main cause of sudden death in patients with scleroderma. When sclerosis of the heart valves formed by the type of stenotic. And with pericardial fibrosis, adhesive pericarditis develops.

is based on lesions of the kidneys with sclerosis of small blood vessels with the development of ischemia and death of kidney cells. With a progressive version of scleroderma, the kidney crisis often develops, which is characterized by a sudden onset, rapid development of renal insufficiency and malignant hypertension. For a chronic variant of scleroderma there is a moderately pronounced change in the kidneys, which for a long time remains asymptomatic.

Diagnosis of systemic scleroderma

The diagnosis of systemic scleroderma is reliable if there is one "large" or two "small" criteria( American College of Rheumatology).

• "Big" criterion:
- proximal scleroderma: symmetrical thickening of the skin in the area of ​​the fingers, with proximal extension from the metacarpophalangeal and metatarsophalangeal joints. Skin changes can be observed on the face, neck, chest, abdomen.
• "Small" criteria:
- Sclerodactyly: the above skin changes are limited to the fingers.
- Digital scars-areas of skin twitches on the distal phalanges of the fingers or the loss of the substance of the fingertips.
- bilateral basal pneumofibrosis;mesh or line-nodal shadows, most pronounced in the lower lungs with a standard X-ray examination;there may be manifestations of the "honeycomb" type.

In Russia, the following signs of systemic scleroderma were suggested.

Diagnostic signs of systemic scleroderma( NG Guseva, 1975)

Treatment of systemic scleroderma

Patients with scleroderma are advised to follow a certain regimen: avoid psychoemotional shocks, prolonged exposure to cold and vibration. It is necessary to wear warm clothes to reduce the incidence and severity of vasospasm attacks. It is recommended to quit smoking, give up caffeine-containing drinks, as well as from drugs that cause vasoconstriction: sympathomimetics( ephedrine), beta-adrenoblockers( metoprolol).

The main areas of treatment for scleroderma are:

• Vascular therapy for the treatment of Raynaud's syndrome with signs of tissue ischemia, pulmonary hypertension and nephrogenic hypertension. Angiotensin converting enzyme( enalapril) inhibitors, calcium channel blockers( verapamil) and prostaglandin E are used. In addition, antiplatelet agents( quarantil) are used to prevent thrombus formation.

• Anti-inflammatory drugs should be prescribed early in the course of the disease. Non-steroidal anti-inflammatory drugs( ibuprofen), hormonal drugs( prednisolone) and cytostatics( cyclophosphamide) are recommended according to a certain scheme.

• Penicillamine is used to suppress excess fibroblasts.

Surgical treatment of systemic scleroderma consists in the elimination of skin defects by plastic surgery, as well as the elimination of narrowing of the esophagus, amputation of dead fingers.

Complications of systemic scleroderma and

prognosis With a rapidly progressive form of scleroderma, the forecast is unfavorable, the disease ends in a lethal outcome 1-2 years after the manifestation, even with the timely commencement of treatment. In chronic form with timely and comprehensive treatment, the five-year survival rate is up to 70%.

Doctor therapist Sirotkina EV