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Systemic and nervous system vasculitides are a heterogeneous group of related disorders, each characterized by vascular inflammation such that they have the potential to cause serious morbidity and mortality if unrecognized and therefore untreated. Systemic vasculitis affect all populations and every nationality and walk-of-life, from childhood to older age. The first edition of The Vasculitides, published in 2014, met the urgent need for a clear, concise, and reliable textbook regarding the epidemiology, pathogenesis, clinical presentation, laboratory evaluation and management of these disorders, assembling participants of the 16th International Vasculitis and ANCA Workshop in Paris, France. Five years later, two subsequent meetings have taken place in London and Tokyo. Participants of the 19th International Vasculitis and ANCA Workshop will gather in April 2019 at the University of Pennsylvania for an exceptional venue to share translational scientific discoveries, data from clinical trials, and advances in the clinical assessment, pathophysiology, genetic biomarkers, standard-of-care, and novel therapies of vasculitis. The Workshop participants regathered to update the The Vasculitides, producing an even more cutting-edge two-volume set of books. This is the first book in the set, covering general considerations and systemic vasculitis.