The Importance of Demographic and Geographical Factors on the Incidence and Outcome of Systemic Small Vessel Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies

The Importance of Demographic and Geographical Factors on the Incidence and Outcome of Systemic Small Vessel Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies

Author: Maria Weiner

Publisher: Linköping University Electronic Press

Published: 2019-11-04

Total Pages: 82

ISBN-13: 9176850005

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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.


Book Synopsis The Importance of Demographic and Geographical Factors on the Incidence and Outcome of Systemic Small Vessel Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies by : Maria Weiner

Download or read book The Importance of Demographic and Geographical Factors on the Incidence and Outcome of Systemic Small Vessel Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies written by Maria Weiner and published by Linköping University Electronic Press. This book was released on 2019-11-04 with total page 82 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis

Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis

Author: Renato Alberto Sinico

Publisher: Springer Nature

Published: 2019-09-13

Total Pages: 336

ISBN-13: 3030022390

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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.


Book Synopsis Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis by : Renato Alberto Sinico

Download or read book Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis written by Renato Alberto Sinico and published by Springer Nature. This book was released on 2019-09-13 with total page 336 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Hospital-Based Dermatopathology

Hospital-Based Dermatopathology

Author: Mai P. Hoang

Publisher: Springer Nature

Published: 2020-02-28

Total Pages: 973

ISBN-13: 3030358208

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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.


Book Synopsis Hospital-Based Dermatopathology by : Mai P. Hoang

Download or read book Hospital-Based Dermatopathology written by Mai P. Hoang and published by Springer Nature. This book was released on 2020-02-28 with total page 973 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Systemic Vasculitides: Current Status and Perspectives

Systemic Vasculitides: Current Status and Perspectives

Author: Franco Dammacco

Publisher: Springer

Published: 2016-10-13

Total Pages: 429

ISBN-13: 331940136X

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In spite of their relatively low prevalence, systemic vasculitides have been the object of intensive basic and clinical investigations over the last few years. As a consequence, important advancements have been achieved: from updated diagnostic and classification criteria and a more rational nomenclature to the recognition of an expanding spectrum of clinical manifestations and potentially devastating complications; from the recognition of the viral etiology of conditions such as HCV-related cryoglobulinemic vasculitis and HBV-associated polyarteritis nodosa to newly named variable vessel vasculitis exemplified by Behçet’s disease; from single-organ vasculitis such as central nervous system vasculitis to the more recently emerging features of the IgG4-related, immune-mediated diseases that are showing remarkable clinical heterogeneity. In addition, intriguing pathogenetic hypotheses are being reported for certain chronic, relapsing vasculitides that are improving our understanding of their biology and basic pathophysiology. New avenues are being explored that will hopefully allow a deeper comprehension of the relationships between certain virus-driven vasculitides and lymphoproliferation, and possibly lead to the identification of novel biomarkers that may be used to single out patients at an increased risk of relapse. This explosion of knowledge is obviously resulting in state-of-the-art, personalized treatments of systemic vasculitides. This book is a collection of reviews on the major vasculitides, written by scientists and clinicians with a multi-year experience in this field. We hope it will provide the reader with a stimulating container of new advances in scientific knowledge and more rational therapeutic approaches to this fascinating chapter of pathology.


Book Synopsis Systemic Vasculitides: Current Status and Perspectives by : Franco Dammacco

Download or read book Systemic Vasculitides: Current Status and Perspectives written by Franco Dammacco and published by Springer. This book was released on 2016-10-13 with total page 429 pages. Available in PDF, EPUB and Kindle. Book excerpt: In spite of their relatively low prevalence, systemic vasculitides have been the object of intensive basic and clinical investigations over the last few years. As a consequence, important advancements have been achieved: from updated diagnostic and classification criteria and a more rational nomenclature to the recognition of an expanding spectrum of clinical manifestations and potentially devastating complications; from the recognition of the viral etiology of conditions such as HCV-related cryoglobulinemic vasculitis and HBV-associated polyarteritis nodosa to newly named variable vessel vasculitis exemplified by Behçet’s disease; from single-organ vasculitis such as central nervous system vasculitis to the more recently emerging features of the IgG4-related, immune-mediated diseases that are showing remarkable clinical heterogeneity. In addition, intriguing pathogenetic hypotheses are being reported for certain chronic, relapsing vasculitides that are improving our understanding of their biology and basic pathophysiology. New avenues are being explored that will hopefully allow a deeper comprehension of the relationships between certain virus-driven vasculitides and lymphoproliferation, and possibly lead to the identification of novel biomarkers that may be used to single out patients at an increased risk of relapse. This explosion of knowledge is obviously resulting in state-of-the-art, personalized treatments of systemic vasculitides. This book is a collection of reviews on the major vasculitides, written by scientists and clinicians with a multi-year experience in this field. We hope it will provide the reader with a stimulating container of new advances in scientific knowledge and more rational therapeutic approaches to this fascinating chapter of pathology.

Treatment of Primary Glomerulonephritis

Treatment of Primary Glomerulonephritis

Author: Claudio Ponticelli

Publisher: OUP Oxford

Published: 2009-05-14

Total Pages: 511

ISBN-13: 0191008117

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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.


Book Synopsis Treatment of Primary Glomerulonephritis by : Claudio Ponticelli

Download or read book Treatment of Primary Glomerulonephritis written by Claudio Ponticelli and published by OUP Oxford. This book was released on 2009-05-14 with total page 511 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Paediatric Nephrology

Paediatric Nephrology

Author: Lesley Rees

Publisher: Oxford University Press, USA

Published: 2012-06-28

Total Pages: 657

ISBN-13: 0199601372

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This pocket-sized book is designed to provide up-to-date information for the general paediatrican and paediatric nephrologist, including advice on care of emergencies, chronic disorders and covering common and rare conditions. It is highly relevant for the day-to-day care of patients on the ward or in the outpatient clinic.


Book Synopsis Paediatric Nephrology by : Lesley Rees

Download or read book Paediatric Nephrology written by Lesley Rees and published by Oxford University Press, USA. This book was released on 2012-06-28 with total page 657 pages. Available in PDF, EPUB and Kindle. Book excerpt: This pocket-sized book is designed to provide up-to-date information for the general paediatrican and paediatric nephrologist, including advice on care of emergencies, chronic disorders and covering common and rare conditions. It is highly relevant for the day-to-day care of patients on the ward or in the outpatient clinic.

Uncommon Causes of Stroke

Uncommon Causes of Stroke

Author: Julien Bogousslavsky

Publisher: Cambridge University Press

Published: 2001-05-24

Total Pages: 418

ISBN-13: 9780521771450

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An essential resource for diagnosis and treatment of stroke patients outside the usual clinical categories.


Book Synopsis Uncommon Causes of Stroke by : Julien Bogousslavsky

Download or read book Uncommon Causes of Stroke written by Julien Bogousslavsky and published by Cambridge University Press. This book was released on 2001-05-24 with total page 418 pages. Available in PDF, EPUB and Kindle. Book excerpt: An essential resource for diagnosis and treatment of stroke patients outside the usual clinical categories.

Oxford Textbook of Vasculitis

Oxford Textbook of Vasculitis

Author: Gene V. Ball

Publisher: Oxford University Press, USA

Published: 2014

Total Pages: 690

ISBN-13: 0199659869

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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.


Book Synopsis Oxford Textbook of Vasculitis by : Gene V. Ball

Download or read book Oxford Textbook of Vasculitis written by Gene V. Ball and published by Oxford University Press, USA. This book was released on 2014 with total page 690 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Updates in the Diagnosis and Treatment of Vasculitis

Updates in the Diagnosis and Treatment of Vasculitis

Author: Lazaros Sakkas

Publisher: BoD – Books on Demand

Published: 2013-02-20

Total Pages: 286

ISBN-13: 953511008X

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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.


Book Synopsis Updates in the Diagnosis and Treatment of Vasculitis by : Lazaros Sakkas

Download or read book Updates in the Diagnosis and Treatment of Vasculitis written by Lazaros Sakkas and published by BoD – Books on Demand. This book was released on 2013-02-20 with total page 286 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.

Vasculitis in Clinical Practice

Vasculitis in Clinical Practice

Author: Richard A. Watts

Publisher: Springer

Published: 2015-05-26

Total Pages: 232

ISBN-13: 3319148710

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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.


Book Synopsis Vasculitis in Clinical Practice by : Richard A. Watts

Download or read book Vasculitis in Clinical Practice written by Richard A. Watts and published by Springer. This book was released on 2015-05-26 with total page 232 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.