Scleroderma system – Scleroderma

Scleroderma (Systemic Sclerosis)

What is scleroderma?

Scleroderma is a rare connective tissue disease. It leads to inflammation of the small vessels, and,ultimately to a change in the tissue structure of the skin, joints and internal organs. There are three main forms of scleroderma:

• Localized (Alopecia) scleroderma (linear and morphea-“morfea” (in the form of plaques)) – usually it affects only the skin on some parts of the body. This form of the disease is less severe;
• Systemic scleroderma – It affects extensive areas of the skin and / or internal organs, most commonly affects the lungs. This form of scleroderma are the most dangerous, and can lead to death;
• Perekresny syndrome (overlap-синдром) – may include scleroderma together other autoimmune syndromes.

Scleroderma – causes

Overproduction of collagen and other connective tissue proteins – key features of scleroderma. It is not clear, what causes this overproduction. Dysfunction of the immune system may contribute to the development of excess collagen.

Risk factors for scleroderma

Scleroderma is more common in women. Localized scleroderma usually strikes people 20-40 years. Linear scleroderma is common in children. Systemic scleroderma usually strikes people between the ages 30-50 years.

Other factors, that may increase the likelihood of developing scleroderma include:

• The presence of family members with autoimmune disorders;
• Exposure to chemicals, such as silicon dioxide, ketones, or welding fumes.

Scleroderma – symptoms

Localized scleroderma

Symptoms of localized scleroderma, usually, limited to skin, and does not affect internal organs. Skin lesions may go through several months or several years. In some cases, scleroderma leads to permanent disability. Symptoms of localized scleroderma include:

• Hard patches on the skin, mostly on the face and trunk (Morphea);
• Thickening of the skin in strips, which may extend to underlying muscles and bones (or linear scleroderma Morphea).

Systemic scleroderma

This form of the disease, usually, It refers to the diffuse disease. Many cases of systemic scleroderma starts with Raynaud's syndrome, that includes the edema, pricking, numbness, Leather blue and white, and pain in the fingers and toes. The disease can progress over the years.

Diffuse scleroderma begins suddenly, and can affect not only the skin, but internal organs. Other symptoms of diffuse scleroderma may include:

• Thickening and hardening of the skin;
• Joint and muscle pain, stiffness and swelling;
• Breathing problems, ingestion and digestion of food due to thickening and rigidity of the lung tissue, esophagus, bowel;
• Inflammation and sealing large and small blood vessels.

Complications of diffuse scleroderma can affect virtually every system of the body. Frequent complications include:

• Bleeding;
• Pulmonary hypertension;
• Irregular heart rhythm;
• Heart failure;
• Renal failure;
• High blood pressure.

Diagnosis of scleroderma

The doctor will ask about your symptoms and medical history, and perform a physical examination. Diagnosis is based on changes in the skin.

• Tests may include:
• Blood tests;
• The study esophageal motility;
• Biopsies of skin and other tissues.

For the diagnosis may need pictures of internal organs and structures. For this purpose, applied:

• Roentgen;
• MRT;
• CT scan.

Scleroderma – treatment

Currently, there is no way a complete cure of scleroderma. There are methods, helping to reduce the symptoms of the disease. Methods of treatment of the symptoms of scleroderma include:

Joints and muscles

• Aspirin or nonsteroidal anti-inflammatory agents (NSAIDs);
• Corticosteroids.

The esophagus and digestive tract

• Medicines, limiting the production of acid in the stomach;
• Frequent meals in small portions;
• Being in bed with your head elevated, to avoid heartburn.

The skin and viscera

• Corticosteroids;
• Immunosuppressive;
• Phototherapy.

Kidney problems and / or high blood pressure

• ACE inhibitors or other antihypertensive drugs;
• Antineoplastic;
• Vasodilators.

Raynaud's syndrome

• Calcium channel blockers;
• Being in the heat and wearing warm clothing, to avoid hypothermia;
• We need to quit smoking.

Restricted mobility

Physical therapy and exercise are important for maintaining blood circulation, joint flexibility and muscle strength.

Prevention of scleroderma

At the moment, there are no methods to prevent or scleroderma.