In spite of their relatively low prevalence systemic vasculitides have been the object of
intensive basic and clinical investigations over the last few years. As a consequence
important advancements have been achieved: from updated diagnostic and classification criteria
and a more rational nomenclature to the recognition of an expanding spectrum of clinical
manifestations and potentially devastating complications from the recognition of the viral
etiology of conditions such as HCV-related cryoglobulinemic vasculitis and HBV-associated
polyarteritis nodosa to newly named variable vessel vasculitis exemplified by Behçet's disease
from single-organ vasculitis such as central nervous system vasculitis to the more recently
emerging features of the IgG4-related immune-mediated diseases that are showing remarkable
clinical heterogeneity. In addition intriguing pathogenetic hypotheses are being reported for
certain chronic relapsing vasculitides that are improving our understanding of their biology
and basic pathophysiology. New avenues are being explored that will hopefully allow a deeper
comprehension of the relationships between certain virus-driven vasculitides and
lymphoproliferation and possibly lead to the identification of novel biomarkers that may be
used to single out patients at an increased risk of relapse. This explosion of knowledge is
obviously resulting in state-of-the-art personalized treatments of systemic vasculitides. This
book is a collection of reviews on the major vasculitides written by scientists and clinicians
with a multi-year experience in this field. We hope it will provide the reader with a
stimulating container of new advances in scientific knowledge and more rational therapeutic
approaches to this fascinating chapter of pathology.
