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Scleroderma or systemic sclerosis (SCL) is an autoimmune disorder of unknown aetiology, characterised by fibrosis and microvascular injury in affected organs. The hallmark of the disease is thickening and tightness of the skin and of subcutaneous tissue. SCL may be confined to the skin (localised) or it may be generalised (systemic sclerosis). In the generalised form, involvement virtually of any organ systems can occur, most importantly the skin, blood vessels, lungs, kidneys, gastrointestinal tract, and the heart. SCL is further subdivided into limited and diffuse (depending on the extent of cutaneous involvement). A subset of patients with limited SCL have prominent calcinosis cutis, Raynaud’s phaenomenon (RP), esophageal dysmotility, sclerodactyly and telangiectasias and is called CREST syndrome. However, these features may also be seen in patients with diffuse SCL.
References and Further Reading
- Berezne A, Ranque B, Valeyre D, et al. Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol. 2008;35:1064–72.PubMedGoogle Scholar