According to the Autoimmune Diseases Coordinating Committee (ADCC), between 14.7 and 23.5 million people in the USA – up to eight percent of the population are affected by autoimmune disease. Autoimmune diseases are a family of more than 100 chronic, and often disabling, illnesses that develop when underlying defects in the immune system lead the body to attack its own organs, tissues, and cells. In Handbook of Autoimmune Disease, the editors have gathered in a comprehensive handbook a critical review, by renowned experts, of more than 100 autoimmune diseases, divided into two main groups, namely systemic and organ-specific autoimmune diseases. A contemporary overview of these conditions with special emphasis on diagnosis is presented. Each chapter contains the essential information required by attending physicians as well as bench scientists to understand the definition of a specific autoimmune disease, the diagnostic criteria, and the treatment.

According to the Autoimmune Diseases Coordinating Committee (ADCC), between 14.7 and 23.5 million people in the USA – up to eight percent of the population are affected by autoimmune disease. Autoimmune diseases are a family of more than 100 chronic, and often disabling, illnesses that develop when underlying defects in the immune system lead the body to attack its own organs, tissues, and cells. In Handbook of Autoimmune Disease, the editors have gathered in a comprehensive handbook a critical review, by renowned experts, of more than 100 autoimmune diseases, divided into two main groups, namely systemic and organ-specific autoimmune diseases. A contemporary overview of these conditions with special emphasis on diagnosis is presented. Each chapter contains the essential information required by attending physicians as well as bench scientists to understand the definition of a specific autoimmune disease, the diagnostic criteria, and the treatment.

Autoimmune disorders of the skin remain an enigma for many clinicians and scientists not familiar with these mostly severe and chronic diseases. The book provides an overview and the latest information on the broad spectrum of cutaneous autoimmune disorders for clinicians, scientists and practitioners in dermatology, medicine, rheumatology, ENT, pediatrics and ophthalmology. The book is unique since it presents the state-of-the-art knowledge on pathophysiology, clinical diagnosis and management of these disorders provided by the world experts in the field. The primary intention is to broaden the understanding of the pathophysiology of cutaneous autoimmune disorders and to provide a practical guide to how to identify and handle these conditions. The book is illustrated with many tables, illustrative figures and clinical color photographs. The third edition has been thoroughly updated and extended by chapters on paraneoplastic cutaneous syndromes, atopic dermatitis and autoimmunity and Skin manifestations of rheumatic diseases.

This book aims to raise awareness of IgG4-related sclerosing cholangitis among practicing physicians and to equip readers with a sound understanding of the principles of diagnosis and treatment. Clinical, serological, and histopathological features are clearly described and imaging appearances on all relevant modalities are illustrated, covering the bile duct and other involved organs. Differential diagnosis from other diseases, including primary sclerosing cholangitis and cholangiocarcinoma, is precisely explained. Information is then presented on all significant current and emerging therapeutic strategies, including steroids, immunosuppressive drugs, and rituximab. Finally, attention is drawn to significant prognostic features. While IgG4-related sclerosing cholangitis is now a widely acknowledged condition, most practitioners are still liable to misdiagnose it owing to a lack of familiarity with its presenting features. This book should help to rectify the situation and will be an asset for all who may encounter the disease in clinical practice.

This book offers a detailed review of the pathological and imaging features, diagnosis and treatment of IgG4-Related Kidney Disease (IgG4-RKD). IgG4-related disease (IgG4-RD), which is characterized by an elevated serum IgG4 level and infiltration of systemic organs by IgG4 positive plasma cells, is a newly recognized systemic disease. Diverse renal manifestations including specific tubulointerstitial nephritis, membranous nephropathy, and tumor-like urological diseases extending to the pelvis and urinary tract in IgG4-RD have been reported and are recently attracting attention as IgG4-RKD. In this book, leading researchers in the field present the latest insights into the broad spectrum of IgG4-RKD characteristics. In addition, they provide a detailed explanation of the pathology of IgG4-RKD including comparisons between the kidney and other affected organs, such as the lacrimal, salivary glands and pancreas in the histopathological section. IgG4-Related Kidney Disease will have a major impact on future immunological and nephrological studies and offers a useful resource not only for nephrologists but also general physicians and investigators in related fields.

This volume details our current understanding of the architecture and signaling capabilities of the B cell antigen receptor (BCR) in health and disease. The first chapters review new insights into the assembly of BCR components and their organization on the cell surface. Subsequent contributions focus on the molecular interactions that connect the BCR with major intracellular signaling pathways such as Ca2+ mobilization, membrane phospholipid metabolism, nuclear translocation of NF-kB or the activation of Bruton’s Tyrosine Kinase and MAP kinases. These elements orchestrate cytoplasmic and nuclear responses as well as cytoskeleton dynamics for antigen internalization. Furthermore, a key mechanism of how B cells remember their cognate antigen is discussed in detail. Altogether, the discoveries presented provide a better understanding of B cell biology and help to explain some B cell-mediated pathogenicities, like autoimmune phenomena or the formation of B cell tumors, while also paving the way for eventually combating these diseases.

The Autoimmune Diseases, Sixth Edition, emphasizes the "3 P's" of 21st Century medicine: precision, prediction and prevention. Topics cover the modern systems approach to biology that involves large amounts of personalized, ongoing physiologic data ("omics") coupled with advanced methods of analysis, new tests of genetic engineering, such as CRISPR, auto inflammatory diseases, autoimmune responses to tumor immunotherapy, and information on normal immune response and disorders. Each of the major autoimmune disorders is discussed by researchers and clinical investigators experienced in dealing with patients. Chapters emphasize the immunologic basis of the disease as well as the use of immunologic diagnostic methods and treatments. The book also covers several cross-cutting issues related to the recognition and treatment of autoimmune diseases, including chapters on the measurement of autoantibodies and T cells, the use of biomarkers as early predictors of disease, and new methods of treatment. - Gives a thorough and important overview on the entire field, framing individual disease chapters with information that compares and contrasts each disorder and its therapy - Provides thorough, up-to-date information on specific diseases, along with clinical applications in an easily found reference for clinicians and researchers interested in certain diseases - Keeps readers abreast of current trends and emerging areas in the field - Ensures that content is not only up-to-date, but applicable and relevant - Includes new, updated chapters that emphasize hot topics in the field, e.g., research on auto inflammatory diseases and autoimmune responses following cancer immunotherapy

Written by the foremost researchers in the field, this book gathers together in a single source the many important clinical associations of antiphospholipid antibodies. Antibody-related clotting mechanisms and their relationship to conditions such as recurrent strokes, chorea, multi infarct dementias, a variety of spinal syndromes, Addison's Disease, recurrent miscarriages, and many more are discussed in depth. The importance of these antibodies in 'Primary,' 'Secondary,' and 'Catastrophic' Antiphospholipid Syndrome is highlighted. Each chapter is devoted to a specific internal system and the clinical effects this syndrome has on that system. This authoritative book is an essential addition to medical libraries as well as an invaluable reference for general physicians, internists, rheumatologists, neurologists, cardiologists, nephrologists, endocrinologists, gastroenterologists, pulmonologists, dermatologists, and obstetricians.

Polymyositis and Dermatomyositis provides extensive information regarding Polymyositis and Dermatomyositis (PM/DM), which is described as a heterogeneous disease complex. This book is divided into four sections: Part I (Clinical Features) covers the classification of PM/DM, details of the clinical presentation, and the disease's association with the other connective tissue disorders and malignancies. Part II (Etiology and Mechanisms) covers advances in the immunopathology and viral etiology of PM/DM along with a frequently recognized entity: inclusion body myositis. Part III (Diagnosis and Treatment) covers the histologic, muscle enzyme histochemical, electron microscopic, and resin histology features of PM/DM along with those electromyographic features that could help make a more accurate diagnosis. Part IV (Overview) summarizes the issues that may not have been clear and highlights differing and unsettled views or present available data. This text is directed to clinicians in private practice or in academic institutions concerned with PM/DM patients, including neurologists, rheumatologists, pediatricians, dermatologists, physiatrists, and neuromuscular investigators. This book is intended as well for neuromuscular pathologists who interpret muscle biopsy specimens and electromyographers who perform EMG studies to help determine the clinical diagnosis. Researchers in immunology and immunopathology of neuromuscular diseases will find discussions in this book invaluable.

This Open Access book presents practical approaches to managing patients affected by various rheumatological diseases, allowing readers to gain a better understanding of the various clinical expressions and problems experienced by these patients. Discussing rheumatology from an organ systems perspective, it highlights the importance ofdetailed musculoskeletal examinations when treating patients affected by rheumatological diseases. The book first explores the latest diagnostic approaches and offers key tips for accurate musculoskeletal examinations before addressing the various treatment modalities, with a particular focus on the most common joints involved in rheumatoid arthritis: the wrists and the metacarpophalangeal joints (2nd and 3rd). Featuring easy-to-understand flow diagrams and explaining the common medical problems associated with rheumatic disease, such as shortness of breath and anemia, it is not only a valuable resource to rheumatologists, but will also appeal to medical students, junior residents, and primary healthcare physicians.