Systemic Sclerosis – Actual perspectives
Keywords:
Systemic sclerosis, scleroderma, classification, diagnosis, treatment, prognosisAbstract
ystemic sclerosis is a relatively rare disease, characterized by variable degrees of fibrosis within the skin and internal organs. There are two major subsets, limited and diffuse systemic sclerosis, which are differentiated primarily by the extent of skin involvement, and are characterized by different disease evolution and prognosis. Concepts regarding this disease have changed over the years. Uniform concepts are extremely important, as they will allow comparison of groups of patients from different centres, and therefore facilitate a better knowledge of this disease.
Although systemic sclerosis was first described in the 18th century and is recognized as a multi-systemic disease since the 19th century (before, cutaneous sclerosis – scleroderma – was considered as one disease and other diseases, such as gastrointestinal, pulmonary, etc., were considered to be associated with it), its’ aetiology remains unknown and its’ pathogenesis not entirely understood. This has resulted in the non-existence of a specific and therefore curative treatment. Despite this, advances have been made regarding the treatment of several of the disease manifestations. The discovery that angiotensin-converting enzyme (ACE) inhibitors, for instance are effective in the treatment of sclero-derma renal crisis, the most fatal complication of systemic sclerosis up till then. Such new advances have allowed improvement in the quality of life and the rate of survival of these patients. In this article, the new perspectives of treatment of this disease and its’ different aspects are discussed.
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