Scleroderma and Systemic Sclerosis

Medicine 3

Head of Department:
Prof. Dr. med. univ. Georg Schett

Understanding causes


Capillary microscopy and autoantibodies help to detect the development of scleroderma at a very early stage. The present picture is of a patient who does not show any signs of scleroderma other than a Raynaud phenomenon.

Scleroderma (systemic sclerosis) is a fibrotic autoimmune disease whose cause has not yet been clarified. Fibrosis describes the hardening of connective tissue of the skin and internal organs and is often regarded as uncontrolled and excessive wound healing. In addition to fibrotic symptoms, changes also occur in small and large blood vessels and in the immune system. Even though fibrosis, vascular changes and autoimmune phenomena are closely linked, their exact interaction is largely unclear.

Facts and figures around the disease

  • Estimates of the incidence of systemic sclerosis vary widely. It can be roughly assumed that there are 10 cases per 10,000 people. However, many early and mild courses are not recognised.
  • The onset of the disease can be in childhood, but also in high adulthood. The disease most frequently occurs in the 3rd to 5th decade of life.
  • Even though no specific therapies have been available to date, the 10-year survival rate has been reduced by 30% over the past 30 years.

Typical symptoms and course of the disease

Initial stage

The first signs of the disease are Raynaud's syndrome (whitening of the hands during cold and stress), Puffy finger (swollen fingers) and autoimmune phenomenon (autoantibodies).

In the further course

The further course of the disease is very variable. The vascular changes can lead to severe Raynaud's syndrome with development of fingertip ulcerations (lesions at the end of the finger) and pulmonary hypertension. The fibrotic changes affect the skin and in severe cases can affect almost the entire body surface. This leads to significant restrictions in the mobility of the patients. In addition, almost all internal organs can be affected by the fibrotic changes, in particular the lungs, the heart or the gastrointestinal tract. Involvement of internal organs is often associated with very severe progressions.

How does the physician make the diagnosis?

It is important to diagnose early stages in order to initiate therapy in time and to contain the spread of the disease. Extensive anamnesis, physical examination, capillary microscopy and special laboratory undercuts can be groundbreaking here. If the suspicion of scleroderma is confirmed, important organ systems should be examined early for possible involvement. The diagnosis and care of scleroderma patients will usually be performed at specialized centers.

How is the disease treated?

Scleroderma treatment should be performed at a specialized center. A comprehensive therapy to cure the disease is not yet available. The therapy is carried out depending on the affected organs. Even if there is no cure for this severe disease yet, organ-specific therapies have significantly improved the care of scleroderma patients and their survival over the past 30 years.

Practical tips for everyday life

  • Important for diagnosis and treatment: it is essential to visit a specialised centre in order to assess the severity of the disease and take the right therapeutic measures.
  • Important for the initial diagnosis: the disease spectrum is very broad and ranges from patients with very mild symptoms to seriously ill patients. Some patients are therefore hardly restricted in their life expectancy and quality.
 
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