Title Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis / Renato Alberto Sinico, Loïc Guillevin, editors

Published Cham : Springer, 2020

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Description 1 online resource (334 pages) Series Rare diseases of the immune system Rare diseases of the immune system. Contents Intro; Acknowledgements; Contents; Part I: ANCA-Associated Vasculitis; 1: Introduction: Nomenclature and Classification; 1.1 AAV Pathologic Features; 1.2 Historical Background; 1.3 Vasculitis Nomenclature and Classification; 1.4 Classification and Diagnosis of AAV; 1.5 Both Serotype and Phenotype Are Useful for Classification and Diagnosis; 1.6 Concluding Remarks; References; 2: Genetics of ANCA-Associated Vasculitis; 2.1 Introduction; 2.1.1 Why Study the Genetics of AAV; 2.2 Molecular Genetic Approaches; 2.3 Genetics of GPA and MPA; 2.4 Genetics of EGPA 2.5 Contribution of Genetics to Better AAV Classification2.6 Genetics and Prognosis; 2.7 Conclusion; References; 3: Etiopathogenesis of ANCA-Associated Vasculitis; 3.1 Introduction; 3.2 Genesis of the ANCA Autoimmune Response; 3.2.1 Molecular Mimicry Theory; 3.2.2 Complementary Peptides; 3.2.3 NETosis; 3.2.4 T-Cell Dysregulation and MPO/PR3 Autoimmunity; 3.3 Evidence for the Pathogenic Role of ANCAs; 3.3.1 Clinical Support for ANCA Pathogenicity; 3.3.2 In Vitro Support for ANCA Pathogenicity; 3.3.3 Animal Model Supports ANCA Pathogenicity 3.3.4 Mechanisms of ANCA-Induced Lesions: The Role of Complement3.4 Conclusion: Pathogenic Mechanism Overview; References; 4: ANCA: Methods and Clinical Significance; 4.1 Introduction; 4.2 Detection of ANCA in Small-Vessel Vasculitis; 4.3 Revised 2017 International Consensus on ANCA Testing in Small-Vessel Vasculitis; 4.4 Clinical Usefulness of ANCA Testing; 4.4.1 Gating Policy; 4.4.2 Utility of ANCA in Managing Disease Activity and Guidance Therapy; 4.5 Proteinase 3-ANCA and Myeloperoxidase-ANCA Subtype: Useful Tool for Disease Classification in Small-Vessel Vasculitis 4.5.1 Epidemiological and Genetic/Epigenetic Features vs. ANCA Specificity4.5.2 ANCA Specificity and Clinical/Histological Features; 4.6 Conclusion; References; 5: Activity and Damage; 5.1 Introduction; 5.2 How Can Laboratory Measures Help?; 5.3 Clinical Disease Activity and Damage Measures; 5.4 Disease Activity Assessment in Vasculitis; 5.4.1 BVAS, BVAS WG, and PVAS; 5.5 Assessment of Damage in Vasculitis; 5.5.1 VDI; 5.5.2 Main Differences When Scoring VDI Versus BVAS v3; 5.5.3 Key Points About VDI; 5.6 Role of Assessment of Disease Status in AAV; 5.7 Patient-Reported Outcomes 5.8 Training5.9 Future; 5.10 Conclusions; References; 6: Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg-Strauss); 6.1 Introduction; 6.2 Epidemiology; 6.3 Triggering Factors; 6.4 Pathophysiology; 6.5 Clinical Manifestations; 6.5.1 General Symptoms; 6.5.2 Pulmonary and Ear, Nose, and Throat (ENT) Manifestations; 6.5.3 Neurological Manifestations; 6.5.4 Skin Manifestations; 6.5.5 Gastrointestinal Involvement; 6.5.6 Cardiac Manifestations; 6.5.7 Renal Manifestations; 6.5.8 Other Manifestations; 6.6 Complementary Investigations; 6.7 Diagnosis Summary 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 Bibliography Includes bibliographical references and index Notes 6.7.1 Diagnostic Criteria Online resource; title from PDF title page (SpringerLink, viewed September 23, 2019) Subject Vasculitis. Vasculitis Vasculitis Form Electronic book Author Sinico, Renato Alberto Guillevin, L. (Loïc) ISBN 9783030022396 3030022390 9783030022389 3030022382 9783030022402 3030022404

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