Nuclear auto-antibodies: a useful tool for the diagnosis, the classification and the prognosis of systemic sclerosis

Purpose. - Systemic sclerosis (SSc) like other connective tissue diseases is characterized by the occurrence of antinuclear auto-antibodies (ANA). We reviewed the diagnostic value and prognostic value of ANA in SSc. Current knowledge and key points. - LeRoy proposed new criteria of limited forms of SSc which take into account the immunological profile of the patients. Anticentromere, antitopoisomerase I, antifibrillarin, anti-PM-Scl, antifibrillin or anti-RNA-polymerase I or III are considered as specific anti -autobodies. Whereas anticentromere antibodies are more associated with limited SSc and antitopoisomerase I with diffuse SSc, other auto-antibodies were associated with other organ involvement: anti-RNA-polymerase I or III and renal crisis, anti-U1-RNP and pulmonary hypertension and pulmonary fibrosis, etc. Patient with persistent antitopoisomerase I antibodies have a poor survival, those with antitopoisomerase antibodies that disappear with time have a better prognosis. Future prospects and projects. - A better identification of the patients with SSc, an individual biological profile and organ involvement may help better management of the disease and better access to clinical trials. The development of new immunological tests and their clinical application should help us in that way. (C) 2003 Elsevier SAS. Tous droits reserves.