Bücher der Reihe Rare Diseases of the Immune System

This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels.

This volume on Rarer Arthropathies, part of the series "e;Rare Diseases of the Immune System"e; aims to fill a gap in the literature by informing and updating clinicians on the unusual and uncommon forms of arthritis and related musculoskeletal conditions. It will be a valuable go-to resource for clinicians to support them in differential diagnosis, in particular when facing unusual presentations. Nineteen chapters written by experts have covered a wide range of these conditions, providing first rate information on topics yet lacking adequate coverage; from Palindromic Rheumatism to SAPHO Syndrome and other chronic immune-mediated conditions, while also including arthritis in leprosy, tuberculosis and chikungunya.Smaller, crisp paragraphs and apt subheadings will help the readers in easily understanding the up to date information provided in the chapters. Line diagrams, original full-color clinical photographs and informative tables also support easy assimilation of information, turning this book in a useful ready reckoner on the desks and smartphones. All specialists, including rheumatologists, orthopedic surgeons, pediatricians, physicians practicing internal medicine and rehabilitation medicine will find this volume invaluable in   their daily clinical work. 

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.¿

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 in both pediatric and adult patients.

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 in both pediatric and adult patients.

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.

This book provides detailed information on the nosology, pathology, pathogenesis, clinical presentation, diagnosis and treatment of fibro-inflammatory disorders, rare diseases that often display systemic organ involvement.

In this volume devoted to systemic lupus erythematosus (SLE) and Sjoegren syndrome, leading specialists from different disciplines present the latest research findings on many aspects of the diseases and describe the most recent trends in treatment, such as the "treat to target" approach.

This book presents detailed state of the art knowledge on the humoral primary immunodeficiencies (PIDs), i.e., disorders arising from impaired antibody production due to defects intrinsic to B cells or defective interaction between B and T cells.

This book, part of the series Rare Diseases of the Immune System, offers comprehensive, up-to-date coverage of the pathophysiology and management of the antiphospholipid syndrome (APS). Specific chapters describe the treatment of the different symptoms, therapies of value in avoiding recurrences, and innovative treatment approaches.

This book provides detailed information on the nosology, pathology, pathogenesis, clinical presentation, diagnosis and treatment of fibro-inflammatory disorders, rare diseases that often display systemic organ involvement.

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.

This book, written by very well known opinion leaders in the field, covers all aspects of familial Mediterranean fever, the most common monogenic autoinflammatory disease.

This book provides an updated overview of agammaglobulinemia, a rare form of primary immunodeficiency which is considered the prototype of the congenital humoral defects, and which is characterized by the absence of peripheral B cells and very low serum immunoglobulin levels.

In this volume devoted to systemic lupus erythematosus (SLE) and Sjoegren syndrome, leading specialists from different disciplines present the latest research findings on many aspects of the diseases and describe the most recent trends in treatment, such as the "treat to target" approach.

This book, written by very well known opinion leaders in the field, covers all aspects of familial Mediterranean fever, the most common monogenic autoinflammatory disease.

This book, part of the series Rare Diseases of the Immune System, offers comprehensive, up-to-date coverage of the pathophysiology and management of the antiphospholipid syndrome (APS). Specific chapters describe the treatment of the different symptoms, therapies of value in avoiding recurrences, and innovative treatment approaches.

The main aim of this volume is to provide an updated overview of the genetic basis of a group of complex disorders that are considered rare individually, but show a relatively high prevalence on the whole in developed countries: the rare autoimmune diseases. These are chronic disorders that involve long and expensive treatments with considerable side effects that may dramatically reduce the quality of life of affected people. Therefore, shedding light into their pathologic mechanisms is a major concern given their growing social and economic awareness and impact.General sections include different chapters on single entities as systemic lupus and sclerosis, Behçet's disease, Sjogren's syndrome and polymiositis, and sections devoted to autoimmune vasculitis, hepatic autoimmune conditions, as well as those affecting the nervous system, and a concluding chapter in which the shared predisposition amongst all diseases is discussed. All the chapters are given a homogeneous scheme in which the authors explain the clinical singularities of every disease and report the recent breakthrough discoveries related to them, giving a critical interpretation and suggesting future perspectives. All contributors are renowned researchers with high expertise in each disease. Due to the exponential increase in our understanding of the genetic causes of these complex diseases, a major advance in biomedical discovery is taking place. However, most of the achievements in this field are very recent and there is a lack of bibliography gathering them together. This book is thus filling a gap and will prove a useful companion to clinicians in the first place (internists, rheumatologists, clinical immunologists, and hematologists) but also to basic and applied researchers.