Behçet's Syndrome: From Pathogenesis to Treatment (Rare Diseases of the Immune System)

Behçet's syndrome can reasonably be considered a unique entity among diseases of the immune system for several reasons: It has specific features and, uniquely among the immune system pathologies, represents a link between autoimmune diseases, systemic vasculitis, and autoinflammatory diseases. In addition, it is of interest to a variety of specialists, including immunologists, rheumatologists, dermatologists, and ophthalmologists, and requires a complex multidisciplinary approach. Many aspects need to be considered in a syndrome that presents a wide spectrum of symptoms and for which the therapeutic armamentarium is expanding significantly, with the development of new treatments, not least the so-called biologics. This book offers comprehensive coverage of the disease by some of the world’s leading experts in Behçet's syndrome from all the relevant specialties. Epidemiology, genetics, pathogenesis, organ system involvement, differential diagnosis, novel treatments, surgical management, and prognosis are just some of the topics addressed. Behçet's Syndrome: From Pathogenesis to Treatment will be an invaluable reference for a range of practitioners, researchers, and undergraduates or postgraduates interested in immuno-rheumatology, dermatology, and rare diseases.​ Front Matter....Pages i-x The Numbers of Behçet: A Rare Disease?....Pages 1-4 From Hippocrates to Hulusi Behçet: What History....Pages 5-15 Epidemiology of Behçet Syndrome....Pages 17-24 Behçet’s Syndrome According to Classical and Population Genetics....Pages 25-37 Infections, Autoimmunity, and Behçet’s Syndrome: What Liaison?....Pages 39-51 Pathogenesis of Behçet Syndrome....Pages 53-66 Mucocutaneous Involvement in Behçet’s Syndrome....Pages 67-81 Neurological and Neuropsychological Manifestation in Behçet’s Syndrome....Pages 83-96 Ocular Involvement and Behçet Disease....Pages 97-115 Articular and Muscular Manifestations in Behçet’s Disease....Pages 117-123 Cardiovascular Issues: Aneurysms and Pseudoaneurysms, Thrombosis, Atherosclerosis, and Cardiac Involvement....Pages 125-135 Intestinal Behçet Disease....Pages 137-149 Audio Vestibular Involvement in Behçet’s Disease....Pages 151-153 Behçet’s Syndrome and Gynecological Manifestation in Reproductive Age and Pregnancy....Pages 155-164 Pediatric Onset of Behçet Syndrome....Pages 165-176 Behçet’s Disease. Differential Diagnosis....Pages 177-188 Classification and Diagnosis Criteria for Behçet’s Disease....Pages 189-198 Prognosis and Disease Activity....Pages 199-205 Old and New Treatment for Behçet’s Disease....Pages 207-216 Surgical Treatment of Angio-Behçet....Pages 217-225 Back Matter....Pages 227-229 Behcet's syndrome can reasonably be considered a unique entity among diseases of the immune system for several reasons: It has specific features and, uniquely among the immune system pathologies, represents a link between autoimmune diseases, systemic vasculitis, and autoinflammatory diseases. Lorenzo Emmi, Editor. Includes Bibliographical References And Index. Mode Of Access: World Wide Web.