This volume, written by well-known experts in the field, covers all aspects of Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis (AAV). The expression refers to a group of diseases, characterized by destruction and inflammation of small vessels. The clinical signs vary and affect several organs, such as the kidney, lung, skin, nervous system and others. The opening chapters give some historical hints, explain the genetic basis of the disease and provide insights into the pathogenesis derived from recent experimental studies and guides the reader through classification and nomenclature. A large part of the book is then devoted to a detailed description of the specific related diseases and their clinical presentations, the disease course, and potential complications. The advice regarding treatment is based on the best currently available evidence in this constantly evolving area.

The book is part of Springer's series Rare Diseases of the Immune System, which presents recently acquired knowledge on pathogenesis, diagnosis, and therapy with the aim of promoting a more holistic approach to these conditions. AAVs are systemic autoimmune diseases of unknown cause that affect small (to medium) sized blood vessels. They include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome).

This volume will be an invaluable source of up-to-date information for all practitioners involved in the care of patients with these diseases.

Autorentext

Renato Alberto Sinico is a Professor of Nephrology and Director of the Postgraduate School of Nephrology at Università degli Studi di Milano Bicocca. From 2005 to 2015 he was responsible for the Clinical Immunology Unit at Ospedale San Carlo Borromeo of Milan, where he has worked since 1980. He has been president of Forum Interdisciplinare delle Malattie Autoimmuni (FIRMA) and secretary of Immunopathology Study Group of the Italian Society of Nephrology. With a special interest in kidney failure, glomerulonephritis, vasculitis, lupus, cryoglobulinemia, rare autoimmune diseases, autoantibodies such as ANCA and anti-DNA, Prof. Sinico works with numerous international journals, and is the author of more than 160 scientific publications.

Loïc Guillevin is a specialist in internal medicine, involved for many years in the management and care of patients affected by systemic vasculitides, like eosinophilic granulomatosis with polyangitis syndrome, but also granulomatous polyangitis granulomatosis, classical or hepatitis B virus (HBV)-related polyarteritis nodosa, microscopic polyangiitis or mixed essential cryoglobulinemia. Founder of the French Vasculitis Study Group (FVSG) counting over 600 French physicians and with strong international contacts. More than 20 prospective therapeutic trials have been conducted so far by the FVSG, with subsequent publications in renowned international medical journals, with major practical findings: the most severe forms of the disease, identified as those having specific renal, cardiac, gastrointestinal and/or central nervous system involvement, should receive a combination of corticosteroids and immunosuppressant, mostly monthly cyclophosphamide pulses. Pr Loïc Guillevin and his main collaborators are currently based in the Department of Internal Medicine, Hôpital Cochin, Université Paris 5, Paris, France. In 2003 the FVSG created its web homepage,primarily dedicated to continuing medical education, but the development of a patients' homepage, with the help of patients and patients' associations, is planned for the very near future.

Inhalt

Preface.- 1 Introduction: Nomenclature and Classification.- 2 Epidemiology.- 3 Genetics.- 4 Pathogenesis B lymphocytes T lymphocytes.- 5 ANCA: methods and clinical significance.- 6 Activity and Damage.- 7 Eosinophilic Granulomatosis with Polyangiitis (EGPA).- 8 Granulomatosis with Polyangiitis (GPA).- 9 Microscopic Polyangiitis (MPA).- 10 Vasculitis /organs involvement.- 11 ENT involvement.- 12 Lung involvement .- 13 Kidney involvement.- 14 Nervous system involvement. 15 Peripheral Nervous System.- 16 Central Nervous System.- 17 Skin involvement.- 18 Miscellaneous organ/system involvement (heart, gastrointestinal, articular.).- 19 Prognosis and outcome.- 20 Therapy.- Subject Index.