Systemic Vasculitides: Current Status and Perspectives

The systemic vasculitides,includinglarge, medium, small, and variable vessel vasculitis,have been the focus of intensive basic and clinical investigations over the last twodecades. Among theimportant advances stemming from these efforts are new definitions, classifications, and diagnostic criteria for the different classes of vasculitis; the addition of anti-neutrophil cytoplasmic autoantibodies as a new criterion for classifying vasculitis; the recognition of  the viral etiology of conditions such as cryoglobulinemic vasculitis and polyarteritis nodosa; an appreciation of thebroad spectrum of clinical manifestations and potentially devastating complicationsassociated with vasculitis; the many features and remarkable clinical heterogeneity of IgG4-related, immune-mediated diseases; and the proposal of intriguing pathogenetic hypotheses for certain chronic, relapsing vasculitides.

This improved understanding of the systemic vasculitides has been accompanied by a trend away from the use of eponyms for these conditions; thus, established terms such as Wegener’s granulomatosis and Churg-Strauss syndrome have been replacedby the more descriptive definitions “granulomatosis with polyangiitis” and “eosinophilic granulomatosis with polyangiitis,” respectively.

Additional clinical laboratory tests,rapidly developing imaging techniques that can assess inflammation, especially in large-vessel vasculitis, and artificial neural network approaches will no doubt bring a wealth of informationthat ultimately leads to the identification of novel disease biomarkers. Expected applications include the identification of individuals at increased risk ofrelapsewho would benefit from patient-tailored therapy. Although the conventional combination of glucocorticoids and immunosuppressive drugs is effective in the treatment of a large proportion of vasculitic disorders, safer medications, with fewer side effects, are being developed, includingseveral biological agentsnow being closely evaluated in multi-center studies.

This volume brings togethercomprehensive and up-to-date reviews written by experiencedscientists and clinicians from many countries. Its aim is toprovide readers with state-of-the-art knowledge of the major vasculitides and cutting-edge insights into their multi-faceted features. It is our hope that this book serves as a valuable and stimulating resourcefor basic and clinical researchers, specialists in related disciplines, as well as practicing physicians and advanced medical students interested in this fascinating branch of pathology.



Franco Dammacco, Domenico Ribatti, Angelo Vacca