The systemic vasculitides have been the focus of rapidly increasing research activity, which has over the past decade yielded significant advances in our understanding of their aetiopathogenesis and clinical management In 2013, the 16th International Vasculitis and ANCA Workshop was held in Paris, organized by Professor Loic Guillevin. The present volume has arisen out of this meeting and reflects the diverse range of topics that were discussed, with individual chapters written in main by participants in the meeting.
It is becoming increasingly apparent that the global occurrence of many types of vasculitis varies between populations of different ethnic and geographical background, and that this reflects genetic differences perhaps more than environmental factors.
The classification of the vasculitides has been a controversial topic for many years, but the second Chapel Hill Consensus Conference held in Chapel Hill in 2012 has clarified the situation, and the revised nomenclature and definitions adopted there have been used throughout this volume. In the ANCA-associated vasculitides there has been increasing recognition, as a result of epidemiological, genetic and clinical studies, that classification should in future be based on ANCA specificity rather than conventional clinical phenotypes.
The pathogenicity of ANCA is increasingly supported by experiments using cell culture systems and in vivo animal models. Several novel target antigens in ANCA-associated vasculitis have been identified, but some of these remain controversial. There have been important advances in our understanding of inflammatory mechanisms in vasculitis, involving both the innate and adaptive immune systems.
The recent major developments in therapy are reflected in the therapeutic section of the volume, with the introduction of targeted therapies for ANCA vasculitis and giant cell arteritis. The advent of B cell depletion using rituximab for ANCA-associated vasculitis has been one of the striking advances over the past decade.
The authors are to be congratulated on providing cutting-edge reviews on important advances in systemic vasculitis, of relevance to the multi-disciplinary teams looking after these patients. The publication of this volume provides an appetizer to the forthcoming 17th International Vasculitis and ANCA Workshop to be held in London in April 2015. Richard A. Watts, M.D., M.P.H., Norwich, UK Charles Pusey, M.D., London, UK July 13,2014

Foreword vii

Preface ix

List of Contributors xi

I. General Considerations 1

Chapter 1 History and Background of Vasculitis 3

Chapter 2 Overview of Primary and Secondary Vasculitides 15

Chapter 3 Epidemiology of Primary Systemic Vasculitis 65

Chapter 4 Experimental Autoimmune Vasculitis Insights into Human Vasculitis Using Animal Models 85

Chapter 5 Genetic Aspects of Vasculitis 95

Chapter 6 Nomenclature and Pathologic Features of Vasculitides 121

Chapter 7 Detection Techniques and Clinical Relevance of ANCA Testing in ANCA-Associated Vasculitis 153

Chapter 8 Classification of Pediatric Vasculitides 154

Chapter 9 The Clinical Approach to Patients with Vasculitis 161

II. Systemic Vasculitis 175

Chapter 10 Classification and Pathogenicity of ANCA-Associated Vasculitis 177

Chapter 11 Polyarteritis Nodosa 199

Chapter 12 Cryoglobulinemic Vasculitis 227

Chapter 13 Systemic Vasculitis and the Lung 241

Chapter 14 Systemic Vasculitis and the Kidney: ANCA Vasculitis and Glomerulonephritis 261

Chapter 15 Systemic Vasculitis of the Gastrointestinal Tract 277

Chapter 16 Rheumatoid Arthritis Vasculitis 297

Chapter 17 Giant Cell Arteritis 311

Chapter 18 Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 337

Chapter 19 Dermatologic Aspects of Systemic Vasculitis 369

Index 399

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