Recommendations of the Italian Society of Rheumatology for the treatment of the primary large-vessel vasculitis with biological agents

Objective: To provide recommendations on behalf of the Italian Society for Rheumatology for the off-label use of biologic agents in the treatment of large-vessel vasculitis. Methods: A panel of experts performed a literature search and selected the evidence relevant to the topic. The following five large-vessel vasculitides were considered: giant cell arteritis, Takayasu arteritis, primary angiitis of the central nervous system, Cogan's syndrome, and Adamantiades-Behçet's disease. Results: A consensus was achieved regarding the indication of biologic agents for the treatment of large-vessel vasculitis. Levels of evidence were assigned to the papers retrieved, and the strength of the recommendations was graded according to the levels of evidence. Conclusions: These recommendations may be used for guidance in deciding which patients with large-vessel vasculitis should receive biologic therapy. Further updates of these recommendations may be published on the basis of the results of new clinical studies and of data from post-marketing surveillance.