Vascularites systémiques : critères de classification, scores d’activité et scores pronostiques

Abstract

Systemic vasculitides are classified according to their clinical and biological presentations and main histological features. Several classification criteria have been proposed, including those of the American College of Rheumatology in 1990, and those of the Chapel Hill Consensus Conference in 1993, which are the most used. There are currently no validated and universally accepted diagnostic criteria. Several scores assessing the activity of vasculitis have been devised, including the Birmingham vasculitis activity score (BVAS), whose latest version is named BVAS 2003. However, these activity scores are of limited relevance in everyday practice, mainly because of their relative complexity and, more importantly, because the therapeutic goal in systemic vasculitis is to achieve complete and sustained remission, i.e. a score of zero for activity, and not merely the reduction by a certain percentage of these scores. The five-factor score (FFS) is a prognostic score, developed in 1996 by the French Vasculitis Study Group, which can help in choosing the most appropriate therapy for patients with polyarteritis nodosa, Churg–Strauss syndrome, or microscopic polyangiitis. Finally, there are scores evaluating cumulative damage related to vasculitis and its treatment, and their use has now become almost systematic in clinical studies. Many efforts are currently under way at the international level to develop, update and validate all these criteria and scores.