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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.
A strong clinical emphasis is present throughout this volume from the first section of commonly presenting problems through to the section addressing problems shared with a range of other clinical sub-specialties.
This work has broad applications in clinical medicine, ranging from prevention and treatment of organ and bone marrow transplant rejection, management of various autoimmune disorders (for example, rheumatoid arthritis), skin disease and asthma. Whereas traditionally only a small repertoire of immunosuppressive agents was available for clinical use, recent discoveries have significantly increased the number of approved agents, resulting in numerous trials to further evaluate their potential. There is also considerable interest in the potential of cell-based therapies (particularly hematopoietic stem and dendritic cell therapy) of allo- and autoimmunity. Important recent advances in the immunotherapy of allergic diseases are also covered in this book. This volume is intended both for practising physicians and surgeons and for biomedical scientists at the graduate/postdoctoral levels, and is designed to provide the theory behind these various approaches to immunosuppression, and to provide state-of-the-art reviews of current developments in each area.
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.
In 1992, the Falk Symposium No. 70 dealt with the topic `Immunology and Liver'. At that time basic mechanisms of immunology as well as immunopathogenetic mechanisms in viral and autoimmune liver diseases were discussed. Now, 7 years later, the Falk Symposium No. 114, held in Basel, Switzerland, October 20-21 1999 (Part I of the Basel Liver Week 1999), focused on immunology in autoimmune liver diseases. In the first section basic mechanisms of autoimmunity are presented, including the relevance of superantigens and the role of apoptosis. A further topic is the latest developments concerning animal models for autoimmune diseases. Recently the International Autoimmune Hepatitis Group newly defined and reclassified the syndrome of autoimmune hepatitis. Autoimmune hepatitis is now identified and studied in all parts of the world, including Asia and South America. A special variant of autoimmune hepatitis was identified as one organ manifestation of the autoimmune polyendocrine syndrome type 1, a genetic disease caused by mutations in a single transcription factor. Drug- and hepatitis-virus induced immune mediated liver diseases may serve as models for nonhepatic immune mediated disorders. DNA technology has increased our knowledge of the immunogenetic background of autoimmune liver diseases. Among the cholestatic immune mediated liver diseases, significant progress has been made concerning primary biliary cirrhosis, in particular regarding the identification of mitochondrial antigens and the characterisation of the immune reactions directed at them. The involvement of infectious agents in PBC as well as the definition of overlap syndromes is a particular focus for basic and clinical research in this area. Concerning the therapy of autoimmune liver diseases, corticosteroids and azathioprin remain the state of the art for autoimmune hepatitis, while bile acids have become well established in treatment of primary biliary cirrhosis as well as primary sclerosing cholangitis. New drugs in the future will include topical steroids such as budesonide and new immunosuppressive agents like mofetil/mycophenolate. Liver transplantation is the treatment of choice for end stage liver diseases; all autoimmune liver diseases are among the best candidates for liver transplantation. Hopefully, new therapeutic strategies based on the results obtained from experimental models will become everyday clinical practice in the next decade. Therefore this symposium concludes with a discussion.
This book provides a practical guide to the diagnosis and management of rheumatic disease in geriatric patients. It details information on the effect of ageing on musculoskeletal and immune systems and specifies impact of a variety of inflammatory and degenerative rheumatic disorders on the elderly. The clinical diagnostic approach to an older individual with musculoskeletal complaints is a cornerstone of this book, which includes also insights into the most important comorbidities for this age and discusses in depth available treatments and rehabilitation tools. The symptoms, imaging features and recommendations on the interpretation of specific laboratory tests for rheumatic diseases in geriatrics are also covered. This book encompasses traditionally associated with older age conditions including temporal arteritis, polymyalgia rheumatica, osteoarthritis, as well as common systemic autoimmune diseases that can produce atypical manifestations in the elderly. It will enable the readers to develop a thorough understanding of how to deal with these patients in their clinical practice. Rheumatic Disease in Geriatrics: Diagnosis and Management systematically describes how to diagnose and treat geriatric patients with a variety of rheumatic conditions and is a valuable resource for all medical professionals involved.
This book focuses on clinical presentation, diagnostic processes and current management of systematic vasculitis, and for this second edition the Editors have updated the treatment approach and nomenclature in line with current practice. Systemic vasculitides are a group of disorders which are of increasing importance. Many of these conditions are only rarely encountered by general physicians and often present significant diagnostic challenges. The Editors aim to provide easily accessible information in a pocket sized format. In doing so they hope to help both the generalist but also rheumatologists who only encounter these problems occasionally.
This pocket-sized book is designed to provide up-to-date information for the general paediatrican and paediatric nephrologist, including advice on care of emergencies, chronic disorders and covering common and rare conditions. It is highly relevant for the day-to-day care of patients on the ward or in the outpatient clinic.
This textbook details the latest diagnostic and management options available when treating patients with diseases affecting the oral mucosa. It comprehensively covers the content required to successfully pass dermatology and stomatology board examinations, while being a valuable resource for dentistry trainees. Chapters cover aspects of mucosal immunity and clinical scores along with a range of diseases. Didactic features including learning objectives enable the reader to better extrapolate the key concepts covered in each chapter. Diseases of the Oral Mucosa: Study Guide and Review presents an in depth overview of the latest diagnostic tools and management options available for diseases of the oral mucosa and is an ideal resource for trainee and practising dentists, dermatologists and stomatologists preparing to sit board examinations.
The prevalence of autoimmune diseases and rheumatic conditions is constantly increasing. Autoimmune diseases affect approximately 7-10% of the population of the United States, while more than 50,000,000 American adults suffer from some type of arthritis. The Heart in Rheumatic, Autoimmune and Inflammatory Diseases examines the complex mechanisms relating to cardiac diseases from a pathophysiological and clinical point of view. Autoimmune rheumatic diseases can affect the coronary vessels, myocardium, pericardium, heart valves and the conduction system. The diagnosis of these unique cardiac complications necessitates medical awareness and a high index of suspicion. Increased risk of advanced atherosclerosis plays a pivotal role in the development of cardiac diseases in systemic, rheumatic and autoimmune illnesses. Yet, other complex immune medicated mechanisms may contribute to the pathogenesis. Patients' optimal care requires coordination between the primary caregiver, the rheumatologist, immunologist and cardiologist. Screening for cardiovascular risk factors, recognition of high-risk patients and identification of subclinical cardiac conditions are of great importance. Moreover, regulation of inflammation, as well as abnormal immune responses and the initiation of early treatments should be the focus of patient management. A continuous attempt to identify novel therapeutic targets and change the natural history of the underlying disease and its cardiac manifestations is in progress. The book aims at providing the readers with a state of the art collection of up to date information regarding clinically important topics based on experts' perspectives. This book was a result of an extended coordinated collaboration of one-hundred and fifty-four distinguished scientists from thirty-one countries around the globe. - A review of common, as well as unusual (yet clinically significant) medical cardiac complications of prevalent rheumatic, autoimmune and inflammatory diseases. - Focuses on aspects of pathophysiological processes, clinical presentations, screening tests, prognostic implications and novel therapeutic approaches. - Presents an up-to-date "level of evidence and "strengths of recommendations for suggested therapies and reviews all randomized clinical trials, meta-analyses and other supporting published clinical findings.