From the Thibierge-Weissenbach syndrome (1910) (or CRST syndrome) to anticentromere antibodies (1980). Clinical and biological manifestations of progressive systemic sclerosis.

G. Thibierge and M.R.J. Weissenbach reported during the 1(st) July 1910 ses sion of the Hospital Medical Society the first case report of what was late r called in 1964 in the English literature a CRST syndrome. This patient ha d 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 subty pes according to the extent of cutaneous and visceral involvement. Prelimin ary classification cirteria have been edicted by the American College of Rh eumatology (ACR) in 1980. Various antinuclear autoantibodies hare been asso ciated 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 associa ted with either anti-Scl 70 (topoisomerase I) antibodies (20 to 30%) or ant i-RNA polymerase III (20%).