[From Thibierge-Weissenbach syndrome (1910) to anti-centromere antibodies (1980). Clinical and biological features of scleroderma].
G. Thibierge and M.R.J. Weissenbach reported during the 1st July 1910 session of the Hospital Medical Society the first case report of what was later called in 1964 in the English literature a CRST syndrome. This patient had progressive systemic sclerosis (PSS) with Raynaud's phenomenon, recurrent cutaneous calcinosis and face and trunk telangiectasiae. Since this first description, progressive systemic sclerosis has been split in various subtypes according to the extent of cutaneous and visceral involvement. Preliminary classification criteria have been edicted by the American College of Rheumatology (ACR) in 1980. Various antinuclear autoantibodies have been associated with the prognosis of the different subtypes of PSS: anticentromere antibodies are detected in 50% of patients with CREST syndrome which has a better vital prognosis than diffuse scleroderma. This later form is associated with either anti-Scl 70 (topoisomerase I) antibodies (20 to 30%) or anti-RNA polymerase III (20%).
Meyer
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Service de Rhumatologie, Hôpital Bichat, Paris.
D000974:Antibodies, Antinuclear; D017675:CREST Syndrome / Q000145:classification / Q000266:history / Q000276:immunology*; D002503:Centromere / Q000276:immunology*; D049672:History, 19th Century; D049673:History, 20th Century; D006801:Humans
D000974:Antibodies, Antinuclear