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 Behçets 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. 

Franco Dammacco is Emeritus Professor of Internal Medicine at the University of Bari Medical School and has been President of the Italian Society of Internal Medicine. His research and clinical interests include HCV-related cryoglobulinemic vasculitis, onco-hematology, connective tissue diseases and immunodeficiency syndromes. He is author or co-author of more than 470 papers and Editor-in-Chief of the journalClinical and Experimental Medicine.

Domenico Ribatti is full Professor of Human Anatomy at the University of Bari Medical School. His main research topics are angiogenesis and anti-angiogenesis, with special emphasis for hematological malignancies, tumor microcirculation, and immunological competence.  He is author of over 650 papers  and 8 monographs.

Angelo Vacca is full Professor of Internal Medicine at the University of Bari Medical School and President of the Italian Society of Immunology, Clinical Immunology and Allergology. He isactively involved in research areas such as angiogenesis and tumor progression, multiple myeloma and related malignancies, biological markers of tumor invasion and metastasis, and controlled chemotherapy studies. On these topics he has published over 300 papers.

 

Preface.- Introductory remarks.- Giant Cell Arteritis.- Takayasu Arteritis.- Polyarteritis Nodosa.- Mechanisms of ANCA-Associated Vasculitides.- Granulomatosis with Polyangiitis (Wegeners) and  Similar AAV with Probable Etiology.- Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss).- Anti-Glomerular Basement Membrane Disease.- IgA Vasculitis.- HCV-Related Cryoglobulinemic Vasculitis.- Vasculitis and Pulmonary Hypertension: New Therapeutic Approaches.- Vasculitis in SLE, RA and Other Connective Tissue Diseases: Diagnosis and Treatment.- Central Nervous System Vasculitis.- Uveitis.- IgG4 Syndrome.- Behçet Syndrome.- Urticarioid Vasculitis.- Guidelines for the Diagnosis and Treatment of Vasculitides.- Index.