Vasculitis In Practice

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.

"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"" 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.

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, an inflammation of blood vessels, can be idiopathic or secondary to other conditions. Infections may also mimic idiopathic vasculitis, and the differential diagnosis is of paramount importance for the practicing physician. Vasculitides are not rare diseases. In fact, some vasculitides, such as giant cell arteritis, cutaneous vasculitis, and ANCA-associated vasculitis are relatively common in everyday practice. Vasculitis may rapidly lead to organ failure, and put patient's life in danger. Therefore, physicians of different specialties should diagnose vasculitis early, because early institution of treatment is crucial for the favorable outcome. In recent years progress has been made in the pathophysiology and treatment of vasculitis. This book reflects all new advances in pathogenetic mechanisms, diagnosis, and treatment of different types of vasculitis. The international panel of authors helps in achieving a balanced view on different aspects of vasculitis.

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.

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.