[Approach and challenges in primary vasculitidies]

The primary systemic vasculitidies (PSV) comprise an heterogeneous group of complex clinical entities with a common substrate: inflammation and necrosis of blood vessels. The inflammatory process involves vessels of any caliber. The location and the different size of the affected vessels, the severity of vascular damage and the different histopathological patterns which may predominate are the basic characteristics that define the different vasculitic syndromes and enable individualization. The description and progress in the knowledge of the primary vasculitidies have evolved considerably in recent decades, allowing for a better resolution of the complex problems placed by these patients. This review aims to provide a view on the practical approach to these diseases and their challenge. It focuses on ANCA associated vasculitidies (AAV) because this subgroup has the only proven biomarker and is the most common in our area. Also, multiorganic involvement is frequent. Some notes regarding other PSV are made.