This contributed volume provides a complete overview of Neuro-Behçet’s disease (NBD), one of the most serious manifestations of Behçet’s disease. It serves as a comprehensive and critical review of the current scientific literature regarding NBD, covering the epidemiology, pathology, prognosis, and treatment of the disease. This book is an essential resource for both researchers and physicians working on neurology, rheumatology, and internal medicine fields.

It is with great pleasure that I write this Foreword to the Proceedings of the International Conference on Behçet’s Disease which was held in Berlin in June 2002. This was the first International Conference held under the auspices of the International Society for Behçet’s Disease which was founded in 2000 in Seoul. First, I congratulate our colleagues in Berlin, led by Professor Christos Zouboulis of the Department of Dermatology at the Free University of Berlin, for having organised a most successful conference and for having compiled these proceedings so rapidly. It will be realised immediately on scanning the contents of this book that the conference was truly international with 210 participants from 26 countries, as Professor Zouboulis has noted in his preface. These included basic scientists, epidemiologists, pathologists, clinicians and, importantly, representatives from patient organisations. The latter held their own conference alongside the scientific-medical conference to mutual benefit. The combined session of patients and doctors (abstracts on pp 601 – 626) gave the opportunity for an exchange of information and fruitful discussion. The wide ranging scope of the communications is evident from the index and it was most encouraging to see their origin – from all parts of the world, from senior and junior colleagues and, from many different disciplines. Many communications may be regarded as preliminary reports of research in progress and we look forward to seeing the definitive publications in appropriate journals in due course.

Behçet’s Syndrome has seen great strides over the last two decades in the availability of new treatments and the understanding of underlying pathogenesis. Only 30 years ago the majority of particularly young men with Behçet’s lost total eye sight, now only a minority do. This book covers the most recent developments in the basic and clinical aspects of Behçet’s Syndrome. International authorities have collaborated to offer their diverse expert knowledge on the multiple affected organs and systems, including the skin, the eye, the brain, the lungs and not the least the gastrointestinal and the locomotor systems. A special chapter is devoted to juvenile disease. The definitive resource on Behçet’s Syndrome, this book is well suited for rheumatologists, dermatologists, ophthalmologists, neurologists, and health professionals caring for Behçet’s patients.

Behçet's disease is a chronic relapsing inflammatory disease of unknown etiology involving multiple organs. Along with blindness, other bodily functions are greatly affected by this disease; conditions of the vascular, intestinal and central nervous systems are usually life threatening and require aggressive therapy. Recently, significant progress has been made in several areas of this disease. The introduction of biological agents (such as anti-TNF) have had a positive impact on the effectiveness of treatments. However, there are still a number of unmet needs in various fields of this disease. Despite this, there remains a number of unaddressed issues concerning the treatments of this disease. Several genes have been shown to be associated with this disease, in addition to HLA-B51. Abnormalities in several subsets of T helper cells have been implicated in the pathogenesis of Behçet's disease. Recent advances in the field of innate immunity have shed light on the new aspects of Behçet's disease; that is, this disease has both autoimmunity and autoinflammatory aspects. On the other hand, there are still controversies as to the diagnosis and treatment of recalcitrant manifestations of the disease. This publication highlights the most recent understanding of Behçet's disease and raises several unanswered questions in both basic to clinical treatment of this ailment. The contents of this book are primarily based on the most up-to-date results of research programs sponsored by the Japanese government as well as findings from Korean researchers. In particular, novel classification for neurological involvement (which has been discovered in Japan) led to the establishment of diagnostic criteria and treatment recommendation. Thus, this publication brings forth useful information for a variety of specialists who are involved in the management of this intractable disease.

Behcet's disease is a multisystem inflammatory disease with unknown etiology and has a unique geographic distribution. It is characterized by recurrent oral aphthous lesions, recurrent uveitis, skin lesions, and genital ulcerations. However, it may involve the eye, joints, and cardiovascular, gastrointestinal, and neurological systems at varying degrees as well as the skin and mucous membranes. The disease may manifest itself in a wide spectrum of symptoms ranging from mild symptoms to life-threatening symptoms or severe symptoms that may create permanent sequelae. Knowing the nature of the disease will make it easier to diagnose and manage the disease. This book was written by expert authors, and detailed epidemiology, etiopathogenesis, mucocutaneous findings, and systemic involvement of Behcet's disease are presented to readers.