Autoimmune connective tissue diseases, including systemic lupus erythematosus, Sjögren’s syndrome, systemic sclerosis, dermatomyositis, rheumatoid arthritis, and mixed connective tissue diseases are complex conditions that commonly involve multiple organs and systems. The pathogenesis of many autoimmune diseases is elusive, and the disease course is extremely variable, with indolent or rapidly progressive case subsets. The heterogeneity and patient-to-patient variability of autoimmune connective tissue disease extend to their clinical manifestations, autoantibody profiles, speed of disease progression, the constellation of target organ effects, response to treatment, and survival. Despite the significant advances in diagnosis and treatment achieved in the last decades, due to a relative lack of biomarkers and prognostic indicators, it is still difficult to predict disease progression and prevent the development of severe and life-threatening disease manifestations. In addition, recently, there has been a shift in patient treatment, from treating patients after contracting diseases to personalized medicine, in which diseases are prevented and treated according to individual variability in genes, phenotypical features, environment, and lifestyle for each group of patients. For this reason, this Research Topic aims to promote a greater understanding of the prognostic and predictive factors in connective tissue diseases to predict long-term outcomes and improve the use of tailored treatments.

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.

An algorithmic approach to interpreting renal pathology, updated in light of recent advances in understanding and new classification schemes.

Autoimmune diseases are characterized by the occurrence of antibodies reacting with self-constituents of the body. The fully updated third edition of Autoantibodies is an in-depth review of the main autoantibodies identified up to now, with particular emphasis on those that display a diagnostic or prognostic clinical value. The new edition covers recent scientific advances, diagnostic techniques, and therapeutic technologies. Each chapter is focused on a single family of autoantibodies. This important reference contains historical notes, definitions, origins and sources of antigens recognized genetic associations, mediated pathogenic mechanisms, methods of detection, as well as clinical utility (disease prevalence and association, diagnostic value, sensitivity and specificity, prognostic value). This is an ideal reference for anyone involved in the field of autoimmune diseases. - Presents all known, important autoantibodies in a single source, focusing on the antibodies needed for autoimmune disorder diagnosis - Includes clinical applications for each autoantibody along with general information - Organized by disease and disorder type, by autoantibody family, and completely cross-referenced

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.

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.

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.