A new nomenclature of systemic vasculitides (SV) and current approaches to their treatment have necessitated the discussion of some debatable questions on this condition. The paper gives the data of examining 325 patients with different forms of SV, followed up in the Interregional Consulting Center for SV patients, and the results of testing the American College of Rheumatology classification criteria for SV and the authors’ criteria, by taking into account the International Chapel Hill Consensus Conference, USA (1994 and 2011) guidelines for CV nomenclature. It discusses the etiological factors and pathogenetic components of SV, morphological aspects, and relationships between the local and systemic forms of SV. The findings were compared with the data available in the literature. It is concluded that differentially diagnostic criteria for CV should be elaborated to specify the stage of the disease, the activity and use of adapted therapy regimens.