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.
The creation of the WSF was inspired by years of dedicated commitment from people such as Dame Carol Black, Daniel Furst, James Seibold, Marco Matucci Cerinic, Maureen Mayes, Virginia Steen, Thomas Medsger and many others. This has resulted in the formation of the World Scleroderma Foundation, legally constituted in Basel, Switzerland, on November 12, 2009. We regret that some of the early pioneers and dear friends such as Gerald Rodnan, E. Carwile LeRoy and Joe Korn are no longer with us to share our excitement.
Any person or organisation involved with scleroderma may be a member of the foundation on approval of the Board.
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