Systemic Vasculitides: Current Status and Perspectives

  • Franco Dammacco
  • Domenico Ribatti
  • Angelo Vacca

Table of contents

  1. Front Matter
    Pages i-xi
  2. Biology of Blood Vessels, Experimental Models and Nomenclature of Vasculitides

    1. Front Matter
      Pages 1-1
    2. Domenico Ribatti
      Pages 3-8
    3. Domenico Ribatti, Franco Dammacco
      Pages 9-13
    4. J. Charles Jennette, Ronald J. Falk, Marco A. Alba
      Pages 15-28
    5. Joice M. F. M. Belem, Bruna Savioli, Alexandre Wagner Silva de Souza
      Pages 29-41
    6. Giuseppe A. Ramirez, Angelo A. Manfredi
      Pages 43-50
  3. Primary Systemic Vasculitides

    1. Front Matter
      Pages 51-51
    2. Enrico Tombetti, Elena Baldissera, Angelo A. Manfredi, Maria Grazia Sabbadini
      Pages 53-62
    3. Silvia Laura Bosello, Elisa Gremese, Angela Carbonella, Federico Parisi, Francesco Cianci, Gianfranco Ferraccioli
      Pages 79-96
    4. F. David Carmona, Javier Martín
      Pages 97-108
    5. Franco Dammacco, Angelo Vacca
      Pages 109-118
    6. Franco Dammacco, Sebastiano Cicco, Domenico Ribatti, Angelo Vacca
      Pages 119-128
    7. Renato Alberto Sinico, Paolo Bottero
      Pages 129-139
    8. Adrian Schreiber, Mira Choi
      Pages 141-158
    9. James Ritchie, Timothy Reynolds, Joanna C. Robson
      Pages 159-171
    10. Fernanda Falcini, Gemma Lepri
      Pages 173-187
    11. Nicolò Pipitone, Carlo Salvarani
      Pages 189-196
    12. Michele Rossini, Annamaria Di Palma, Vito Racanelli, Francesco Dammacco, Loreto Gesualdo
      Pages 197-202
    13. Roberta Fenoglio, Dario Roccatello
      Pages 203-212
    14. Maria Grazia Lazzaroni, Micaela Fredi, Sonia Zatti, Andrea Lojacono, Angela Tincani
      Pages 213-224
    15. Rosaria Talarico, Stefano Bombardieri
      Pages 225-232
    16. Robert G. Micheletti
      Pages 233-244
    17. Carlo Salvarani, Robert D. Brown Jr., Caterina Giannini, Gene G. Hunder
      Pages 245-257
    18. Alice Brambilla, Rolando Cimaz, Gabriele Simonini
      Pages 281-288
    19. Rosanna Dammacco
      Pages 289-297
    20. Shiri Shulman, Zohar Habot-Wilner
      Pages 299-309
    21. Emanuel Della Torre
      Pages 311-320
    22. Giulia De Feo, Roberta Parente, Chiara Cardamone, Massimo Triggiani
      Pages 321-329
  4. Secondary Vasculitides

    1. Front Matter
      Pages 331-331
    2. Franco Dammacco, Sabino Russi, Domenico Sansonno
      Pages 333-344
    3. Patrizia Leone, Sebastiano Cicco, Angelo Vacca, Franco Dammacco, Vito Racanelli
      Pages 345-359
    4. Masayuki Sugimoto, Kimihio Komori
      Pages 361-376
    5. Anne Claire Desbois, Patrice Cacoub
      Pages 377-383
  5. Diagnostic and Therapeutic Advances of Systemic Vasculitides

    1. Front Matter
      Pages 385-385
    2. Mazen Abusamaan, Patrick Norton, Klaus Hagspiel, Aditya Sharma
      Pages 387-405
    3. John Anthonypillai, Julian L. Ambrus Jr.
      Pages 407-423
    4. Loïc Guillevin
      Pages 425-431
  6. Back Matter
    Pages 433-438

About this book


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 agents now 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


vasculitis glomerulonephritis purpura pulmonary infiltration ANCA

Editors and affiliations

  • Franco Dammacco
    • 1
  • Domenico Ribatti
    • 2
  • Angelo Vacca
    • 3
  1. 1.University of Bari Medical SchoolBariItaly
  2. 2.University of Bari Medical SchoolBariItaly
  3. 3.University of Bari Medical SchoolBariItaly

Bibliographic information