Vasculitis

A comprehensive review of inflammatory syndromes and diseases that affect the blood vessels, this volume draws upon authors from all over the world to present informed discussions on all types of vasculitis and related conditions.

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.

The prompt diagnosis of systemic vasculitis is essential as a missed diagnosis can be disastrous. Imaging is of vital importance in achieving a correct diagnosis and in some cases also plays a role in endovascular treatment. In this book, the imaging features of the many different types of vasculitis are clearly demonstrated by means of numerous high-quality illustrations. All relevant imaging modalities are considered, and key distinctive characteristics are highlighted. In addition, each chapter discusses the etiology, epidemiology, pathogenesis, clinical presentation, biology, and treatment of the vasculitis in question. This book is the result of cooperation between expert teams from a range of countries. The wealth of illustrations and informative clinical case studies will prove invaluable for all who may be confronted with these problematic disorders.

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.

In this book, detailed information on the nosology, pathology, pathogenesis, clinical presentation, diagnosis and treatment of large- and medium-sized vessel and single-organ vasculitis is provided and critically discussed by the most expert physicians and researchers in the field. Among the conditions considered are giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, primary central nervous system vasculitis, isolated aortitis, isolated gastrointestinal vasculitis, cutaneous vasculitis and isolated genitourinary vasculitis. The role of histopathology in the diagnosis and prognosis of these vasculitis is evaluated, along with the part played by imaging studies in diagnosing and monitoring these diseases, while indications and limitations of the available imaging modalities are discussed as well. The expanding role of biological agents for the treatment of the large vessel vasculitis is addressed, as well as the current approaches to these diseases. This book will be a valuable companion in decision-making for medical practitioners, internists, specialists, researchers and postgraduate students interested in the intriguing fields of vasculitis and rare diseases.

Vasculitis is a condition that involves inflammation in the blood vessels. The condition occurs if the immune system attacks the blood vessels by mistake. This may happen as the result of an infection, a medicine, or another disease or condition (NIH). Textbook of Systemic Vasculitis is an in-depth guide to the epidemiology, classification and genetics of systemic vasculitis as well as imaging, symptoms and management of the disease. The book is divided into six sections, beginning with an introduction to the condition and its basic science, followed by sections on imaging, manifestations of vasculitis, principles of management, and a final section on a range of individual vasculitis syndromes. The fourth section covers vasculitis in various organs and systems of the human body. The text emphasises the details of pathological changes in the areas most commonly affected by the disease. 135 full colour images and illustrations enhance this comprehensive text. Textbook of Systemic Vasculitis is an up-to-date and forward looking guide to the condition, featuring potential future developments, as well as reviewing advances in treatment over the past half-century. Key Points Analyses symptoms of vasculitis, organ by organ Covers 16 different vasculitis syndromes in detail 135 full colour images and illustrations Discusses the future development of vasculitis treatment

"Vasculitis refers to diseases that cause blood vessel walls to thicken and narrow, cutting off blood supply to tissues and organs. Vasculitis encapsulates several conditions, and the cause of vasculitis is often unknown. This book explores various forms of vasculitis and options for treatment. Chapter One includes a detailed description of giant cell arteritis, a form of vasculitis involving inflammation of the middle and large arteries, including the latest management strategies. Chapter Two concerns different types of vasculitis affecting the lungs, their signs and symptoms, treatment, and complications needed for diagnosis. Chapter Three deals with eosinophilic granulomatosis with polyangiitis, a multisystemic necrotizing vasculitis characterized by allergic rhinitis, asthma, and peripheral eosinophilia. Chapter Four describes the common pathophysiology and cardiovascular complications arising in different vasculidities. Chapter Five reviews the pathophysiology, epidemiology, diagnostic investigation, and treatment of infection etiologies of Herpesviridae associated with vasculitis. Chapter Six elucidates the impact of vasculitis on the gastrointestinal system. Chapter Seven explains hypocomplementic urticarial vasculitis syndrome, defined as presence of greater than six months of urticaria along with hypocomplementemia and multi-system manifestations such as recurrent abdominal pain, uveitis, arthralgia and glomerulonephritis. Chapter Eight explicates the use of biologics in treating rheumatology disorders, with rituximab being recommended in organ-threatening, refractory systemic lupus erythematosus. Lastly, Chapter Nine discusses the pathophysiology and adverse effects of adenoviral vector vaccinations for SARS-CoV-2 and the importance of identifying the true epidemiology of adverse outcomes regarding promoting increased acceptance of COVID-19 vaccinations, while stressing that despite the possibility of adverse outcomes, the benefits of vaccination outweigh the risks of remaining unvaccinated"--

The author tells the story of her long and painful battle with Takayasu's arteritis, a life-threatening disease of the blood vessels.