Systemic scleroderma - diagnosis , prognosis, treatment


1. Causes

2. clinical picture of systemic sclerosis

3. course and prognosis of the disease

4. Diagnosis and treatment of systemic sclerosis

Systemic scleroderma - a serious chronic disease of the connective tissue and small vessels, in which the connective tissue thickens and hardens.The disease affects the skin, gastrointestinal tract, kidneys, lungs, heart.Most often it occurs in women 30-50 years old.


In our time, the exact causes of the disease are unknown.It was found that the onset of the disease is the failure of the immune system.In this case the body produces antibodies against its own tissues.Because of this disrupted the small blood vessels, dramatically increasing the production of collagen.As a result, sealing and form foci atrophy (decrease of the volume and weight of organs and tissues) lesions.

Experts note the factors that provoke the development of systemic sclerosis:

  • genetic predisposition to autoimmune diseases;
  • work, implying contact w
    ith harmful substances for a long time;
  • past infectious and acute viral disease;
  • adverse factors (vibration, cold), acting for a long time;
  • neuroendocrine disorders of the body;
  • allergic reactions.

clinical picture of systemic sclerosis

Systemic scleroderma is characterized by multiple symptoms as loss of blood vessels leading to the pathology of all organs and tissues of the body.

Raynaud's syndrome - one of the first signs of the disease, which occurs in 95% of cases.It is characterized by transient spasm of the small arteries and arterioles (small end branching arteries) fingers of the upper and lower extremities.Sometimes spasm of earlobes and the tip of the nose.During the attack, the skin becomes pale, then - cyanotic, with warming red.Pallor and blueness of the skin is accompanied by a feeling of cold and numbness and redness - pain and tingling.Sometimes Raynaud's phenomenon may be the only manifestation of the disease for several years.

Another initial symptom of systemic scleroderma is swelling of the fingers and hands.In some cases, swelling spreads to the face, forearms, feet, shins.It can be stored for several months.Sometimes swelling may be accompanied by erythema (severe inflammation of the skin as a result of the expansion of capillaries).Skin tension fingers leads to difficulties with their flexion and extension.On the fingertips, sometimes over the bony prominences can form ulcers.Develops atrophy of subcutaneous fat pads of the fingers and they are formed depressed scars.

Patients often becomes taut skin of the face, with the mouth slit is narrowed.Because of this, they have a hard brush your teeth.

Most people suffering from systemic scleroderma, there is swelling, stiffness, tenderness of the fingers and knees.In some cases, there is a symmetric polyarthritis (inflammation of the joints), muscle weakness, myositis (inflammation of muscle fibers).

Most of the patients with systemic scleroderma has signs of lesions of the esophagus - belching, burning sensation or fullness in the epigastric.These symptoms are worse when stooping and when lying down.When the disease often appears reflux esophagitis (esophageal inflammatory disease), which sometimes leads to bleeding.In addition, there are the following symptoms of the gastrointestinal tract:

  • discomfort or pain when swallowing;
  • flatulence and abdominal pain;
  • frequent diarrhea;
  • weight loss, anemia;
  • fecal incontinence, rectal prolapse.

defeat lung is the leading cause of death in systemic sclerosis.This pathology is detected in the majority of patients.Its symptoms are dry cough, shortness of breath on exertion.In severe cases, the patient developed a bacterial or viral pneumonia, pulmonary hypertension (closing the lumen of medium and small branches of the pulmonary arteries).

Systemic scleroderma, in some cases, contribute to the development of pericarditis (inflammation of the outer membrane of the heart), arrhythmia, heart failure, angina pectoris, myocardial damage.

Patients often develop kidney failure.This is especially true for patients with advanced extensive skin lesions.

In addition, systemic scleroderma often hypothyroidism (low thyroid function), trigeminal neuralgia, liver cirrhosis.

course and prognosis of the disease

Forecast systemic sclerosis depends on the nature of its flow.

In acute illness during a rapid build-up of symptoms and development of the internal organs and systems diseases.Forecast systemic scleroderma in case unfavorable.Typically, one year after the onset of the disease occurs functional disorder of internal organs.

In the case of subacute symptoms of the disease is increasing slowly but steadily.Skin lesions, joints, muscles develop quickly, while slowly formed fibrosis (replacement of normal tissue at the junction) of the internal organs.Forecast systemic scleroderma moderately favorable.It depends on the activity of the process and degree of its development.

most favorable prognosis of systemic sclerosis in chronic disease.Signs of growing slowly, sometimes for decades.In this case the internal organs and systems for a long time do not lose their functions.

Diagnosis and treatment of systemic sclerosis

diagnosis of systemic sclerosis includes the study of the clinical picture of the disease and the availability of diagnostic criteria.

diagnosis of systemic sclerosis and takes into account the presence of a large number of small features on the system, which was developed by the American Rheumatology Association.According to this system, the diagnostic features of the disease are as follows:

  • large or the main feature - scleroderma skin lesion of the trunk;
  • small or additional features - fibrotic lung, sclerodactyly (loss of fingers), Raynaud's syndrome, scarring on the fingertips.

also in the diagnosis of systemic sclerosis examine blood, urine, immune system, bones and joints, internal organs, and the heart of the patient.

Treatment of systemic sclerosis often symptomatic, it is aimed at reducing activity of the disease and to facilitate the patient's condition.

In subacute and acute course of the disease prescribe high doses of anti-inflammatory hormones.

During the chronic use immunocorrection, vitamin therapy, restorative medicine.

For the treatment of systemic sclerosis drugs used, softening the connective tissue.

Furthermore, use drugs that improve the function microcircular channel.

In the complex treatment of systemic scleroderma great importance is attached to physical therapy and physiotherapy.These treatments stimulate the metabolism of the diseased tissues, have a restorative effect.


This article is available exclusively in the educational purposes and is not research material or professional medical advice.

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