The International ANCA and Vasculitis Workshop has become the cornerstone for investigations in vasculitides. Months before the meeting, I was asked by Nova Science Publishers to edit a book on primary systemic vasculitis and I immediately turned to the upcoming 16~th International ANCA and Vasculitis Workshop organized by the French Vasculitis Study Group knowing that it would have the most current and authoritative information. Yet I could not have predicted the depth and scholarly content of the meeting that I would find there, and the welcoming and ingratiating attitudes of the members. I was updated on virtually every aspect of primary systemic vasculitis and was able to recruit a majority of the present authors from the meeting's leadership and lecturers. Their task as I explained to them was to compile an authoritative volume on vasculitides.
The ensuing volume entitled, The Vasculitides, has 33 chapters organized into 5 sections including, General Considerations, Systemic Vasculitis, Nervous System Vasculitis, and Treatment. The multinational authorship is as diverse as the chapter topics they have been selected to write. Together they have crafted a volume that is expected to serve the present needs of epidemiologists, clinicians, investigators, and other health providers interested in vasculitides. Although there is an emphasis on uniform categorization according to the 2012 revised Chapel Hill Consensus Criteria, other formative topics such as childhood vasculitides and central and peripheral nervous system vasculitides are also included emphasizing their emerging importance. Recognition of the likeliest pathogenic mechanisms such as ANCA has led to more precise classification, clinical recognition, laboratory diagnosis, and successful therapeutic strategies. However the full impact of the burden of vasculitides in metrics of years of life lost (YLL), years lived with disability (YLD), and disability-adjusted life years (DALY), has yet to be applied to vasculitides, as they have in other noncommunicable disorders in the Global Burden of Disease (GBD) Study sponsored by the Institute for Health Metrics and Evaluation at the University of Washington, U.S.A.
I wish to thank each of the contributors for their participation and their tolerance for my incessant editing and rewriting that assured uniformed style and content. I also wish to express my appreciation to my colleagues at New York University School of Medicine, who expressed their enthusiasm for this volume, as well as, my family who stood silently by, while I worked diligently to finish this book. Lastly, this volume would not have been possible without the understanding and patience of Nova Science Publisher staff. David S. Younger, M.D., M.P.H June 1,2014

Foreword vii

Preface ix

List of Contributors xi

III. Nervous System Vasculitis 1

Chapter 20 The Blood-Brain Barrier 3

Chapter 21 Neuroimaging of the Vasculitides 19

Chapter 22 Adult Primary Angiitis of the Central Nervous System and Related Syndromes 47

Chapter 23 Childhood Inflammatory Brain Diseases 59

Chapter 24 Childhood Vasculitic Stroke 75

Chapter 25 Primary CNS Vasculitis: Pathophysiological Diversity 99

Chapter 26 Stroke Due to Vasculitis in Adults 109

Chapter 27 Central Nervous System Vasculitis Due to Infection 127

Chapter 28 Central Nervous System Vasculitis Due to Drug Abuse 151

Chapter 29 Peripheral Nerve Vasculitis: Classification and Disease Associations 173

Chapter 30 Paraneoplastic Vasculitis of the Peripheral Nervous System 195

IV. Treatment 205

Chapter 31 Immunosuppressant Medications Employed in Primary Systemic Vasculitides 207

Chapter 32 Overview of the Treatment of Primary Systemic Vasculitis 223

Chapter 33 Future Directions of Therapeutic Trials 243

Chapter 34 Tribute to Loic Guillevin: His Career and Achievements 259

Index 269

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