Anca Associated 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.

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.

Proceedings of the Fourth International Workshop on ANCA and the Second International Colloquium on Wegener's Granulomatosis and Vasculitic Disorders held in Lubeck, Germany, May 28-30, 1992

This issue of the Rheumatic Disease Clinics of North America provides the rheumatologist with the latest information on diagnosis and treatment of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Topics covered inlucde epidemiology; history; pathogenesis; autoantibodies in ANCA-associated vasculitis (including drug-induced ANCAs); pauci-immune renal disease; ocular manifestations; diagnostic approach (including mimickers); Wegener's Granulomatosis; Churg-Strauss Syndrome; microscopic polyangiitis; outcome measures; and the future of ANCA-Associated Vasculitis.

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) comprise microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Two serotypes are recognized: myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA. Renal involvement is a common and severe manifestation associated with increased mortality. The incidence varies geographically, but studies are difficult to compare due to heterogeneous methodology and inclusion criteria. AAV is commonly found in the elderly, but there are little data on outcome and optimal treatment in the highest age groups. This thesis focuses on the epidemiology of AAV: incidence, geographical distribution, and outcome. In Paper I annual incidence rates and outcome were compared between nephritis in AAV and nephritis in systemic lupus erythematosus (SLE) in two geographically defined populations in Sweden. Even though SLE is twice as common as AAV, ANCA-associated nephritis outnumbered lupus nephritis by three to one, and was significantly more severe in terms of mortality and development of end stage renal disease. In Paper II associations between ANCA serotype and geographical and demographic factors were investigated in a large multi-centre study of 1408 patients with renal biopsy-proven AAV. PR3-ANCA was associated with male gender, younger age and higher glomerular filtration rate. PR3-ANCA was also associated with higher latitude and lower ultraviolet radiation levels, but analyses of subgroups suggested that genetic rather than environmental explanations might be more important for this geographical gradient. In Paper III a consecutive cohort of 151 elderly patients with MPA and GPA was studied with a focus on treatment, mortality and renal survival. Patients who had received immunosuppressive treatment with cyclophosphamide or rituximab had better survival rates compared to less intensively treated or untreated patients. Severely impaired renal function at diagnosis was associated with worse outcome in terms of both patient and renal survival. In Paper IV the elderly cohort was extended to 202 patients. In this study we found that treatment with cyclophosphamide or rituximab was associated with the development of less permanent organ damage, and not with higher utilization of in-hospital care. However, high doses of glucocorticoids were associated with fatal infections and treatment-related damage.

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.

​​​ Inpatient Dermatology is a concise and portable resource that synthesizes the most essential material to help physicians with recognition, differential diagnosis, work-up, and treatment of dermatologic issues in the hospitalized patient. Complete with hundreds of clinical and pathologic images, this volume is both an inpatient dermatology atlas and a practical guide to day-one, initial work-up, and management plan for common and rare skin diseases that occur in the inpatient setting. Each chapter is a bulleted, easy-to-read reference that focuses on one specific inpatient dermatologic condition, with carefully curated clinical photographs and corresponding histopathologic images to aid readers in developing clinical-pathologic correlation for the dermatologic diseases encountered in the hospital. Before each subsection the editors share diagnostic pearls, explaining their approach to these challenging conditions. This book is structured to be useful to physicians, residents, and medical students. It spans dermatology, emergency medicine, internal medicine, infectious disease, and rheumatology. Inpatient Dermatology is the go-to guide for hospital-based skin diseases, making even the most complex inpatient dermatologic issues approachable and understandable for any clinician.

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.