Synonyms
Scleroderma, systemic sclerosis
Definition
Progressive systematic sclerosis is a rare systemic disease of the connective tissue in which there is an increase in connective tissue in the skin, vessels and internal organs. It belongs to the group of collagenoses. Women are three times more frequently affected by progressive systematic sclerosis than men, and the disease often manifests itself between the ages of 30 and 50. There are 6-25 cases per 100,000 inhabitants per year.
Origin
The origin of progressive systemic sclerosis is unknown. It is believed that an inflammation in the vessels is the trigger for the increased formation of connective tissue in the body. There are 2 forms of progressive systemic sclerosis: In systemic scleroderma the symptoms can be very variable: In cutaneous scleroderma, the so-called “morphea” occurs, which means that the skin is changed similar to a scar. Other organs of the body can also be affected. It comes about:
- Edema (water retention) of the skin
- The skin is hardened and taut, especially on the fingers
- In the course of the disease the skin becomes thinner
- Raynaud syndrome of the fingers
- Rhythm disturbance of the heart
- Arthritis
- Renal failure
- Chesty cough
Diagnosis
With the help of capillaroscopy (microscopic examination of the smallest vessels of the nail bed) and the detection of certain antibodies in the blood, the diagnosis of progressive systematic sclerosis can be made.
Therapy
Both drugs that limit the inflammation and symptomatic measures such as keeping fingers warm in Raynaud’s syndrome are used. Lymph drainage and physiotherapy are also used.
Prognosis
The cutaneous form has a good prognosis, while renal involvement and changes in lung tissue (pulmonary fibrosis) are limitations.
