Systemic scleroderma - Causes, Symptoms and Treatment .MF .

August 12, 2017 17:52 | Diseases Of The Heart And Blood Vessels

Systemic scleroderma - a disease that affects various organs, which is based on the change of the connective tissue with a predominance of fibrosis and loss of blood vessels by type of obliterating endarteritis.

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

Causes of systemic sclerosis

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

exact cause of the disease has not been established.Currently, one of the main theory is a genetic predisposition.Established family history of the disease.In addition, sick relatives revealed a higher incidence of other rheumatic diseases (rheumatoid arthritis, systemic lupus erythematosus), compared with the general population.In favor of the theory of the impac

t of viral immunity says identifying the changes related to the activity of viruses (especially retroviruses and herpes viruses).But the specific stamp of the virus that causes systemic sclerosis, is not found.

Symptoms of systemic sclerosis

The main feature of the disease is to strengthen the function of fibroblasts.Fibroblasts - a core of connective tissue cells that synthesize collagen and elastin, whereby the connective tissue characterized by great strength and at the same time elasticity.With an increase in the fibroblasts begin to function to produce large amounts of collagen, increased fibroobrazovanie.In the final, in different organs and tissues of MS lesions are formed.Furthermore, fibrosis and affect vascular wall which thickens.It creates obstacles to blood flow, and as a result, form clots.These vascular changes result in a disruption of the normal blood supply to the tissue and the development of ischemic processes.

Connective tissue is widely represented in the body, so in systemic sclerosis affects almost all organs and tissues.Therefore, the symptoms are very diverse.
Acute quickly progressing form of the disease characterized by the development of sclerotic skin changes and fibrosis of internal organs in one - two years from the onset of the disease.Under this option, quickly appears constantly high body temperature, body weight loss.The mortality of patients with acute quickly progressing variant is high.

to chronic course of systemic sclerosis is characterized by the initial signs of the disease in the form of Raynaud's syndrome, skin lesions or joints.These manifestations may be isolated for many years.Subsequently, clinical symptoms of internal organ involvement.

skin lesion is the most characteristic feature of systemic sclerosis and occurs in most patients.Initially the affected skin of the face and hands.In typical cases, scleroderma stage changes are skin tightening due to swelling, then there is induration (thickening of the skin due to fibrosis) and partial atrophy of tissue.At the same time the skin on your face becomes tight and immobile due to its tension generated kisetoobraznye wrinkles around the mouth, a person acquires similarity to the mask.

masklike face with systemic scleroderma

sclerodactyly is also a characteristic feature of the disease.This forms a skin seal brushes with the development of deformities of the fingers ( "sosiskoobraznye" fingers).


Along with skin seal also revealed trophic disorders in the form of ulceration, suppuration, the deformation of the nail plate and the emergence of foci of alopecia.

Vascular disorders are the most common initial symptom of the disease.The most common are crises vasospastic (Raynaud's syndrome).Thus under the influence of cold, in the absence of agitation or external causes narrowing of small vessels occurs usually hands.This is accompanied by numbness, blanching or even bluish fingertips.With the progression of the disease due to tissue ischemia at their fingertips formed nonhealing ulcers ( "rat bites").In severe cases, develop necrosis last phalanges.

Necrosis end phalanges as a complication of Raynaud's syndrome

defeat joint pain manifested in them, morning stiffness, a tendency to flexion deformations due to compression and atrophy of the tissues around the joint.When the feeling of the affected joints over them may determine the noise tendon friction.For systemic sclerosis is characterized by the seal of muscles and their atrophy.The disease is manifested bone osteolysis (destruction) of bone fingers with shortening phalanx.

Osteolysis of the distal phalanges

most vulnerable organs of the digestive system when the system is sklerodemii esophagus and intestines .In the esophagus, due to its sealing wall is formed sclerotic deformation in violation of the normal passage of food.Patients complain of a feeling of lump in the chest, nausea, heartburn, retching.When large deformation may need surgery to expand the lumen of the esophagus.The intestine is affected less often, but the symptoms of the illness significantly reduces the quality of life of patients.The clinical picture is dominated by pain, diarrhea, weight loss.For lesions of the colon characterized by constipation.

Defeat Lung now coming to the fore among the causes of mortality in patients with systemic sclerosis.Characterized by two types of lung lesions: interstitial disease -fibroziruyuschy diffuse alveolitis and pulmonary fibrosis and pulmonary hypertension.External symptoms of interstitial lesions are nonspecific and include shortness of breath, dry cough, general weakness, fatigue.Pulmonary hypertension is manifested by progressive shortness of breath, formation of blood stagnation in the lungs and heart failure.Often, pulmonary vascular thrombosis and acute right ventricular failure causes the death of patients.

For Scleroderma is characterized by loss of all layers of the heart.When fibrosis of the myocardium heart increases in size, is formed stagnation of blood in the cavities with the development of heart failure.Very often, due to violation of the innervation of an enlarged heart in patients having arrhythmia.Arrhythmias are the main cause of sudden death in patients with scleroderma.When sclerosis heart valve defects are formed on the stenotic type.A pericardial fibrosis develops adhesive pericarditis.

Underlying defeat renal sclerosis is the small blood vessels with the development of ischemia and death of kidney cells.With progressive variant of scleroderma often develop renal crisis, which is characterized by sudden onset, rapid development of renal failure and malignant hypertension.For chronic variant of scleroderma tend to moderate severity, changes in kidney, which remains asymptomatic for a long time.

diagnosis of systemic scleroderma

diagnosis of systemic scleroderma is valid if one "big" or two "small" criteria (American College of Rheumatology).

• «Big» criterion:
- proximal scleroderma: symmetric thickening of skin in the fingers spread proximally from the metacarpophalangeal and metatarsophalangeal joints.Skin changes can occur on the face, neck, chest, abdomen.
• «Small» criteria:
- sclerodactyly: the above-mentioned skin changes restricted his fingers.
- Digital scars, areas of the skin depressions on the distal phalanges, or loss of substance fingertips.
- bilateral basal pulmonary fibrosis;mesh or linear-nodular shadows, most pronounced in the lower regions of the lungs with a standard X-ray examination;may be manifestations on "lung cell" type.

following signs of systemic sclerosis have been proposed in Russia.

Diagnostic signs of systemic sclerosis (N.G.Guseva 1975)

Treatment of systemic sclerosis

scleroderma patients are advised to observe a certain mode: avoid psycho-emotional shocks, prolonged exposure to cold and vibration.You must wear clothes that retains heat, to reduce the frequency and severity of attacks of vasospasm.It is recommended to quit, to give up caffeinated drinks, as well as drugs that cause vasoconstriction: sympathomimetics (ephedrine), beta-blockers (metoprolol).

The main areas of treatment of scleroderma are:

• vascular therapy for the treatment of Raynaud's syndrome with signs of tissue ischemia, pulmonary hypertension and of renal hypertension.Used ACE inhibitors (enalapril), calcium channel blockers (verapamil), and prostaglandin E. In addition, antiplatelet agents are used to prevent blood clots (Curantylum).

• anti-inflammatory drugs appropriate to prescribe in the early stages of disease development.Recommended nonsteroidal anti-inflammatory drugs (ibuprofen), hormones (prednisolone) and cytotoxic drugs (cyclophosphamide) in a specific pattern.

• To suppress excessive fibroobrazovaniya apply penicillamine.

Surgical treatment of systemic sclerosis is to eliminate skin imperfections through plastic surgery, as well as the elimination of esophageal narrowing, amputation of fingers of dead areas.

complications of systemic sclerosis and

forecast When rapidly progressing form of scleroderma poor prognosis, disease are fatal within 1-2 years after the demonstrations, even with timely beginning of treatment.In the chronic form with timely and comprehensive treatment of the five-year survival rate is 70%.

therapists Sirotkin EV