This book aims to raise awareness of IgG4-related sclerosing cholangitis among practicing physicians and to equip readers with a sound understanding of the principles of diagnosis and treatment. Clinical, serological, and histopathological features are clearly described and imaging appearances on all relevant modalities are illustrated, covering the bile duct and other involved organs. Differential diagnosis from other diseases, including primary sclerosing cholangitis and cholangiocarcinoma, is precisely explained. Information is then presented on all significant current and emerging therapeutic strategies, including steroids, immunosuppressive drugs, and rituximab. Finally, attention is drawn to significant prognostic features. While IgG4-related sclerosing cholangitis is now a widely acknowledged condition, most practitioners are still liable to misdiagnose it owing to a lack of familiarity with its presenting features. This book should help to rectify the situation and will be an asset for all who may encounter the disease in clinical practice.

This book offers a detailed review of the pathological and imaging features, diagnosis and treatment of IgG4-Related Kidney Disease (IgG4-RKD). IgG4-related disease (IgG4-RD), which is characterized by an elevated serum IgG4 level and infiltration of systemic organs by IgG4 positive plasma cells, is a newly recognized systemic disease. Diverse renal manifestations including specific tubulointerstitial nephritis, membranous nephropathy, and tumor-like urological diseases extending to the pelvis and urinary tract in IgG4-RD have been reported and are recently attracting attention as IgG4-RKD. In this book, leading researchers in the field present the latest insights into the broad spectrum of IgG4-RKD characteristics. In addition, they provide a detailed explanation of the pathology of IgG4-RKD including comparisons between the kidney and other affected organs, such as the lacrimal, salivary glands and pancreas in the histopathological section. IgG4-Related Kidney Disease will have a major impact on future immunological and nephrological studies and offers a useful resource not only for nephrologists but also general physicians and investigators in related fields.

Autoimmune (IgG4-related) Pancreatitis and Cholangitis reviews the breadth of clinical, imaging, histological, laboratory, and imaging features associated with IgG4-associated systemic disease, especially AIP and IAC. Written by experts in their fields, each chapter includes an overview of existing data as well as the most up-to-date scientific information and emerging data. The book also addresses areas of uncertainty and controversy, briefly highlighting clinical and research needs relative to the respective topic. Comprehensive and easy to use, Autoimmune (IgG4-related) Pancreatitis and Cholangitis is a valuable resource for physicians who deal with or are interested in these complex disease processes, including gastroenterologists, hepatologists, and surgeons.

A practical guide to autoimmune liver diseases through pathogenesis, diagnosis, and management In Autoimmune Liver Disease Management and Clinical Practice, practitioners will learn about the current state of autoimmune liver disease and how to focus on their diagnosis and treatment. The four-part book begins with a thorough investigation of current immunological thinking as it relates to the autoimmunity of the liver. It also covers the four major hepatic autoimmune liver diseases in both adults and children, their management and the role of liver transplantation, and learned approaches to patient management and empowerment. Expert authors in the field have come together to provide a thorough examination of autoimmune liver disease to help support clinicians assisting patients. The text provides an in-depth look at topics including: ● The four major hepatic autoimmune liver diseases, their diagnosis, and potential disease management ● The use (and misuse) of autoantibodies in diagnosis and treatment ● The role and timing of liver transplantation and the impact of recurrent autoimmune liver disease as well as de novo autoimmune hepatitis ● Optimal approaches to managing patients and keeping care personalised With breadth, depth and current-day relevance, Autoimmune Liver Disease sheds light on recent developments in management of liver disease for practitioners, nurses, and health care professionals.

This volume focuses on IgG4-related disease (IgG4-RD), a novel clinical entity involving multiple organs and of unknown origin, associated with the abundant infiltration of IgG4-positive cells. It consists of nine chapters written by prominent experts in the field and discusses the disease concept, diagnosis and treatment, as well as recent findings on its pathogenesis and pathophysiology. As such, it offers an invaluable source of information for researchers and clinicians alike.

This book provides a comprehensive overview of the diagnosis and management of diseases of the biliary tree. Topics covered include: congenital biliary abnormalities, genetic cholangiopathies, autoimmune cholangiopathies, inflammatory and drug-related cholangiopathies, and cholangiocarcinoma. Given their particular importance (even for clinicians working with adults), pediatric conditions are also examined. In addition, a special section is devoted to pregnancy and diseases of the biliary tree, and to transplants and diseases of the biliary tree. Each chapter offers up-to-date information on the management of the diseases discussed. Moreover, the book addresses new treatments for autoimmune cholestatic liver diseases, reflecting the new therapeutic targets that have recently been discovered. Examples include farnesoid X receptor (FXR) and peroxisome proliferator-activated receptor (PPAR)-agonists, together with new drugs that affect the composition of bile flow. Given its scope, the book offers a valuable guide for a broad range of practitioners.

This book provides detailed information on the nosology, pathology, pathogenesis, clinical presentation, diagnosis and treatment of fibro-inflammatory disorders, rare diseases that often display systemic organ involvement. Among the conditions considered are IgG4-related disease, sclerosing cholangitis, Hashimoto’s and Riedel’s thyroiditis, retroperitoneal fibrosis/chronic periaortitis, mediastinal fibrosis, Erdheim-Chester disease, gadolinium-induced fibrosis, and sclerosing mesenteritis. This group of entities is still poorly defined and is characterized by the common denominator of chronic inflammatory infiltrate admixed with abundant fibrosis. IgG4-related disease is the prototypical example. Systemic Fibroinflammatory Disorders is the first book to draw together information on these conditions. As these diseases often require an interdisciplinary approach, the book is addressed to specialists of different disciplines, especially internists, rheumatologists, nephrologists, clinical immunologists, and hematologists.