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.
The 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. Our goal in writing this short book was to provide easily accessible information in a pocket-sized format. We hope we have helped both the g- eral rheumatologist who only encounter these problems occasionally. We have focused on clinical presentation, di- nostic processes, and current management. We hope, the- fore, that we will help our patients with these potentially devastating conditions by improving diagnosis and therapy. Richard A. Watts Ipswich, UK David G. I. Scott Norwich, UK v Contents Part I Introduction 1 Defnitions 3 1 1 General Introduction 3 1 2 Defnitions 4 2 Classifcation and Epidemiology 7 2 1 Introduction 7 2 2 ACR (1990) Criteria 8 2 3 Chapel Hill Consensus Defnitions 9 2 4 Epidemiology 9 2 5 Etiological Factors 10 References 11 3 General Presentation of the Vasculitides 13 3 1 Introduction 13 3 2 Pattern Recognition 13 3 3 Laboratory Investigations 16 3 4 Differential Diagnosis 18 References 19 4 General Principles of Treatment 21 4 1 Treatment 21 4 2 Remission Induction 21 4. 2. 1 Large Vessel Disease . . . . . . . . . . . . . . 22 4. 2. 2 Medium Vessel Disease . . . . . . . . . . . . 22 4. 2. 3 Medium/Small Vessel Vasculitis . . . . . 22 4. 2. 4 Small Vessel Vasculitis . . . . . . . . . . . . .
Vasculitis refers to a group of disorders in which the blood vessels are destroyed through inflammation. This condition affects both arteries and veins. The primary cause of vasculitis is leukocyte migration and the subsequent damage. There can be several symptoms of this disease such as lung infiltrates, joint swelling, unintentional weight loss, acute visual loss, perforations, livedo reticularis, gangrene, and inflammation of the kidney's filtration units. Diagnosis may involve laboratory testing of body fluids and blood, as well as biopsy of involved tissues or organs. Treatments are focused on stopping the inflammation process and suppressing the immune system. Corticosteroids such as prednisone are commonly utilized for this purpose. This book is a valuable compilation of topics, ranging from the basic to the most complex advancements in the clinical management of vasculitis. Those in search of information to further their knowledge will be greatly assisted by it.
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 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 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 this issue of Rheumatic Disease Clinics, guest editors Drs. Eli Miloslavsky and Anisha Dua bring their considerable expertise to the topic of Vasculitis. Top experts in the field cover key topics such as vasculitis mimics and other related conditions; vasculitis and pregnancy: disease state and medication management; CNS vasculitis: PACNS and CNS manifestations of systemic vasculitis; ENT manifestations of vasculitis; isolated aortitis workup and management; and more. Contains 14 relevant, practice-oriented topics including peds vasculitis: Kawasaki’s and COVID MIS-C; emerging therapies EGPA; managing immunosuppression in vasculitis patients in the time of COVID; future of vasculitis: DEI in vasculitis clinical trials, pathway targets, precision medicine, PROs, models for damage/relapse, biomarkers; and more. Provides in-depth clinical reviews on vasculitis, offering actionable insights for clinical practice. Presents the latest information on this timely, focused topic under the leadership of experienced editors in the field. Authors synthesize and distill the latest research and practice guidelines to create clinically significant, topic-based reviews.
"Vasculitis" describes an inflammatory process that involves the blood vessels and contributes to vascular damage. Autoimmunity, infections, drugs, and malignancies have been considered among potential etio-pathogenic factors. In vasculitis, the inflammation might develop in either a systemic or an organ-specific form and might exist as an independent pathology "primary vasculitis" or as a presentation of an existing primary pathology, that is, "secondary vasculitis". This book Vasculitis In Practice-An Update on Special Situations - Clinical and Therapeutic Considerations unlike many publications in the field, uses a different evidence-based approach to organ-specific vascular inflammatory diseases. The authors highlighted the unmet needs from the 1994 Chapel Hill Consensus Conference introducing the latest clinically relevant definitions for the different forms of vasculitis revised in 2012. The identification, classification, and management of kidney disease with different types of vasculitis with an evidence-based update on proposed therapeutic strategies are presented in this publication.
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
The third edition of this definitive and highly-regarded reference work provides a comprehensive review of vasculitis, a fascinating array of life-threatening and minor conditions caused by inflammatory syndromes and diseases that affect the blood vessels. The text uniquely brings together concepts from both the biological and clinical aspects of vasculitis. Research in clinical immunology now invigorates the entire area of vasculitis and shapes a rational approach to pathogenesis, diagnosis, and treatment, which is the substance of this text. Over 40 chapters cover known vasculitic conditions, and are illustrated with over 250 full-colour photographs of clinical and pathologic findings, diagrams, and tables. Separate sections are devoted to basic science, clinical manifestations commonly seen in vasculitis, imaging and percutaneous interventions, individual diseases and syndromes, as well as conditions which can mimic vasculitis. Many of the conditions are rare, and it is the exceptional clinician who has much experience with more than a few of these; thus, the invaluable experience of the international team of authors makes this edition truly indispensible. Now in the Oxford Textbooks in Rheumatology series, this new edition of Vasculitis is published with a concurrent online version, which features access to the full content of the textbook, contains links from the references to primary research journal articles, allows full text searches, and provides access to figures and tables that can be downloaded to PowerPoint. This volume is the definitive reference for rheumatologists, clinical immunologists, and general internists, and will also be of interest to dermatologists, gastroenterologists, cardiologists, pulmonologists, nephrologists, neurologists, pathologists, vascular surgeons, and pediatricians.
