What is Scleroderma?

Scleroderma means ‘hard skin’ but is a complex and perplexing disease with features of vascular damage, organ scarring and immune system dysfunction.

There are an estimated 2.5 million individuals worldwide who suffer from scleroderma, with the majority being women of childbearing age. The cause is unknown and effective overall treatments are lacking, although recent progress has been considerable.

Not all patients with scleroderma (systemic sclerosis) will suffer serious complications, but in its more aggressive form it is the most fatal of all the rheumatologic disorders.

Collaborative clinical and basic research is active on many fronts. Effective treatments have been developed for some of the complications, including kidney involvement and some forms of lung involvement. Critically important research is underway in several areas including the biology of scar tissue formation, the mechanisms of blood vessel damage and attempting to better understand the central role of the immune system in triggering tissue damage. Progress is hampered by the extraordinary clinical diversity of the disease. No two patients are truly alike.

Scleroderma is important in its own right but is appropriately seen as a linchpin disorder, the understanding of which would prove fundamental to many human diseases.

A breakthrough in understanding scleroderma would doubtless have a strong impact on many other diseases involving abnormal scarring and blood vessel injury. This might include conditions as diverse as interstitial lung disease, intestinal hypomotility disorders and many illnesses with blood vessel injury as their basis.

From the perspective of the patients, the burden of disease is high and quality of life is affected for nearly all of them. Universal symptoms of pain, fatigue and malaise have a major influence on quality of life, particularly social life and interaction. Research and treatment addresses the burden of disease while we seek more fundamental answers. Although scleroderma is a relatively rare disease, its chronicity and suffering impose a large burden of both reduced quality of life and considerable health care costs.

Despite the challenges of scleroderma, a spontaneous international collaboration has developed in recent years. The World Scleroderma Foundation is dedicated to initiating and supporting research in scleroderma in all its aspects across all regions of our planet. Our commitment is to facilitate patient-oriented research independently, but also in collaboration with other existing organisations and learned societies working in this field. Support is given in the form of project grants, expert advisory panels and knowledge dissemination. Patient groups as well as allied health professionals form an integral part of the foundation structure. Our association will liaise with society, industry and government in an open and dynamic fashion, whilst remaining independent of regional, political and industrial pressures. The World Scleroderma Foundation is committed to improving the quality of life for scleroderma sufferers and their families.

• Sine Sklerodermie

  This form does not involve the skin, it is mainly characterized by the involvement of internal organs, especially with associated pulmonary hypertension and involvement of the gastrointestinal tract.

• Zirkumskripte Sklerodermie

  (morphea or local inflammatory scleroderma)
  This form of scleroderma is a fibrosis, usually benign, that is limited to the skin and subcutaneous tissue. If the skin around the joints is affected, movement restrictions may occur. However, internal organs are not affected by this form of scleroderma. Since this form belongs to the non-systemic forms, it is not the subject of our research network.

  Further information on localized scleroderma can be found on the Website Sklerodermie Selbsthilfe e.V. under “Zirkumskripte Sklerodermie”.

• CREST

  This form belongs to the already mentioned limited form. The symptoms include Calcification of the skin (calcinosis cutis), restricted movement of the esophagus (esophageal mitility disorder), exposure to cold affects the blood circulation in the fingers, these become discolored and you can feel tingling up to pain (Raynaud), hardening of the fingers (sclerodactyly ) or widening of the blood vessels (telangiectasia).

• Overlap Syndrom

  Scleroderma occurs together with other rheumatic diseases, e.g. B. rheumatoid arthritis.