The Importance Of Demographic And Geographical Factors On The Incidence And Outcome Of Systemic Small Vessel Vasculitis Associated With Anti Neutrophil Cytoplasmic Antibodies

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 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 book provides a concise reference of the histologic and clinical findings of dermatologic conditions encountered in the inpatient setting. The text is divided into twenty chapters. Histopathologic images and corresponding clinical photographs facilitate clinical pathologic correlation of the conditions discussed in each chapter. Bulleted summaries for quick easy-to-read reference and diagnostic pearls are provided for each of the discussed entities. Each chapter ends with several case studies in which clinical presentation, histologic interpretation and work-up of these challenging scenarios are outlined. This book represents an international collaboration and a wealth of clinical expertise and years of experience of authors from Africa, Asia, Europe, North and South America. Hospital-Based Dermatopathology is a useful diagnostic guide for general pathologists, pathology and dermatology trainees, medical students, dermatopathologists, as well as dermatologists, hospitalists, and inpatient clinicians. It also serves as a useful guide in rendering histologic diagnosis for hospital-based or inpatient skin biopsies.

Offering up-to-date, comprehensive coverage of disease progression, diagnosis, management, and prognosis, Textbook of Pediatric Rheumatology is the definitive reference in the field. For physicians caring for children with rheumatic diseases, this revised 8th Edition is an unparalleled resource for the full spectrum of rheumatologic diseases and non-rheumatologic musculoskeletal disorders in children and adolescents. Global leaders in the field provide reliable, evidence-based guidance, highlighted by superb full-color illustrations that facilitate a thorough understanding of the science that underlies rheumatic disease. Offers expanded coverage of autoinflammatory diseases, plus new chapters on Takayasu Arteritis and Other Vasculitides, Mechanistic Investigation of Pediatric Rheumatic Diseases, Genetics and Pediatric Rheumatic Diseases, and Global Issues in Pediatric Rheumatology. Reflects the changes in diagnosis, monitoring, and management that recent advances have made possible. Covers the latest information on small molecule treatment, biologics, biomarkers, epigenetics, biosimilars, and cell-based therapies, helping you choose treatment protocols based on the best scientific evidence available today. Features exhaustive reviews of the complex symptoms, signs, and lab abnormalities that characterize these clinical disorders.

Ferri’s Clinical Advisor 2018 is the most efficient, intuitive, and thorough resource of its kind, trusted by physicians to provide current diagnosis and treatment recommendations for hundreds of common medical conditions. The renowned "5 books in 1" format organizes vast amounts of data in a user-friendly, accessible manner, allowing quick retrieval of essential information. You’ll find guidance on diseases and disorders, differential diagnoses, and laboratory tests– updated annually by experts in key clinical fields. Medical algorithms and clinical practice guidelines round out the core content. Updated content by experts in key clinical fields helps you keep pace with the speed of modern medicine. Popular "5 books in 1" format includes cross-references, outlines, bullets, tables, boxes, and algorithms to help expedite search. Features 30 all-new topics, including Zika virus, hepatitis E, asthma-COPD overlap syndrome, drug-induced parenchymal lung disease, binge eating disorder, hereditary breast and ovarian cancer syndrome, transient global amnesia, and more. Updates 900 topics with the latest developments in medicine over the past year. Contains significantly expanded coverage throughout, including nearly 200 new illustrations, more than 100 new tables and boxes, 50 new differential diagnoses, and 30 new algorithms.

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.

Primary glomerulonephritis is a common renal disease which may eventually lead to chronic renal failure. Treatment of glomerulonephritis is difficult. In particular the use of glucocorticoids and immunosuppressive drugs require expertise, knowledge of the drugs, and careful monitoring of the patient. Paradoxically, the treatment of glomerulonephritis has become even more complicated in the recent years, after the introduction of a number of newer immunosuppressive drugs. Extensively updated since publication of the first edition in 1997, this comprehensive yet concise guide to the treatment of even the most complex patients with primary glomerular diseases is full of practical information collected and organized in an easy-to-read manner. It encompasses the possible treatments of the different types of primary glomerulonephritis, including diseases intrinsic to the kidney of unknown or uncertain aetiology. Each of the main chapters is devoted to a single primary glomerulonephritis and follows a similar format to allow easy access of information. The book contains not only an evidence-based review of the topic, but also practical recommendations from internationally recognized experts in the field.