Systemic sclerosis

At the same time the small blood vessels change. As a result, the skin hardens. Internal organs such as the gastrointestinal tract, the lungs, the kidneys or the heart can also be affected. The disease is not considered curable. Nevertheless, you can do a few things to alleviate the symptoms.

Overview: What is Systemic Sclerosis?

Sclerosis or scleroderma refers to a group of diseases that occur when the skin or organs harden due to an increase in connective tissue. Sclerosis is derived from the Greek word “skleros” = “hard”. Systemic sclerosis is the most severe disease in this group. Systemic means “affecting the whole organism”. In this case, not only the skin but also the internal organs can be affected and severe organ damage can occur in the course of the disease. Scleroderma can affect a wide variety of organs. However, in addition to the skin, the gastrointestinal tract and the lungs, but also the kidneys, heart or joints are particularly frequently affected. Systemic sclerosis is a rheumatic autoimmune disease. This means that the immune system attacks the collagenous connective tissue and triggers an inflammatory reaction, causing it to harden. Collagen is a protein and an important component of skin, bones, tendons, cartilage, ligaments and teeth. In parallel, there are changes in the small vessels. Systemic sclerosis must be distinguished from localised scleroderma (e.g. morphea). This involves the formation of single or multiple circumscribed indurations on the skin without involving the internal organs.

Systemic sclerosis – frequency and age

Systemic sclerosis is a very rare rheumatic disease. It occurs in about 1 in 10,000 people worldwide. It is most common between the ages of 30 and 50, but can affect people of any age. Women are affected three to seven times more often than men.

Systemic sclerosis: causes and risk factors

The exact causes of systemic sclerosis are still unclear. However, it is known that the regulation of certain connective tissue cells is disturbed in this disease. These so-called fibroblasts play a decisive role in the formation of connective tissue. In the case of scleroderma, they are strongly activated by inflammatory cells, among other things, and they produce too much collagen. This in turn results in fibrosis or sclerosis – i.e. a hardening or scarring of the skin – and a narrowing of the blood vessels.

Symptoms: Systemic sclerosis

Typically, fibrosis of the skin develops first on the fingers and hands and on the face. Numerous symptoms can occur with systemic sclerosis:

• The first typical sign of the disease is Raynaud’s syndrome – named after the French doctor Maurice Raynaud. In this case, the blood vessels in the fingers contract suddenly, usually in response to a cold stimulus. Due to the resulting circulatory disorder, the fingers look white and then turn blue as a result of the lack of oxygen. These attacks only last a short time (minutes).
• At the beginning of the disease, there are persistent swellings, especially on the fingers – so-called puffy fingers. The fingers can then stiffen due to the hardening of the connective tissue.
• As fibrosis progresses in the face, a mask face (hypomimia) can develop. People with fibrosis have a smaller mouth and/or wrinkles around the mouth. Permanently dilated small blood vessels may also form on the face and other parts of the skin. Sometimes there is also increased or decreased skin pigmentation. In some patients, the skin fibrosis progresses from the fingers and hands to other areas of the body.

Systemic sclerosis: prevention, early detection, prognosis

The causes of systemic sclerosis are still unknown. Therefore, there is also no possibility to prevent the disease. However, there are numerous measures that you, as the affected person, can take to alleviate the symptoms and new therapy procedures that can slow down the progression of the disease. In addition, various medications have been developed in recent years that can slow down the progression of the disease in certain organs. Your doctor will advise you on which medications might be suitable for you based on the results of the regular SSc assessments. Measures that you can carry out independently:

• Avoiding cold and damp
• Avoid nicotine
• Connective tissue massages

Course and prognosis of systemic sclerosis

The course of the disease depends on whether and to what extent internal organs are affected. Regular screening of the internal organs is therefore important, as it enables an individualized prognosis to be made.

Self-help groups

Systemic sclerosis: Treatment

Currently, systemic sclerosis is not considered curable. Therefore, we mainly focus our treatment on alleviating the symptoms. An important therapeutic goal is also to maintain the mobility of the joints.