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.

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 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 up-to-date information on all aspects of autoimmune pancreatitis, a unique form of pancreatitis characterized clinically by frequent presentation with obstructive jaundice and dramatic response to steroids, histologically by a lymphoplasmacytic infiltrate with fibrosis, and radiologically by pancreatic enlargement. Current concepts regarding the disease and its classification into subtypes 1 and 2 are explained, and clinical, serological, and histopathological findings are carefully described. Imaging features on all the relevant modalities are illustrated, covering both the pancreas and other involved organs. Current and emerging therapeutic strategies, including steroids, immunomodulatory drugs, and rituximab, are then discussed. The reader will find the book to be an excellent aid to the diagnosis of autoimmune pancreatitis and its differentiation from pancreatobiliary malignancies, as well as a clear guide to treatment.

Clinical Pancreatology Since the book Clinical Pancreatology for Practising Gastroenterologists and Surgeons was first published sixteen years ago, the knowledge and clinical management of pancreatic diseases have developed markedly. Thanks to the development of the translational research and the from bench to bedside concept, much progress from the lab has been applied to clinical practice. In addition, several highly relevant clinical trials published over the last years have resulted in the update and optimisation of clinical guidelines. A new and validated classification of severity and complications of acute pancreatitis is firmly rooted in clinical practice and has been the basis for the development of minimally invasive approaches to pancreatic necrosis. The etiopathogenic knowledge of chronic pancreatitis and other pancreatopaties, like that associated with diabetes mellitus, has developed significantly. Especially important has been the development of the field of cystic pancreatic tumours, which has been reflected in the publication of several guidelines and consensus reports over the last few years. Most research efforts have focused on pancreatic cancer, which have led and will further lead to a significant increase in the therapeutic armamentarium against this devastating disease. Finally, many newly published studies have changed the concept, causes, clinical relevance, diagnosis and treatment of exocrine pancreatic insufficiency. This new edition of Clinical Pancreatology for Practising Gastroenterologists and Surgeons has enjoyed the collaboration of the world’s leading experts in each of the areas of clinical pancreatology with the aim of facilitating gastroenterologists, surgeons, oncologists, internists, nutritionists, diabetologists, paediatricians, radiologists, pathologists and other specialists in their decision making when facing patients with pancreatic diseases in their daily clinical practice. All in all, this book supplies an indispensable update of the relevant aspects of clinical pancreatology.

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.

Epithelial tissue does not spring to mind as an obvious source of autoimmune phenomena. Yet, genetic predisposition and influence of various environmental and epigenetic factors may lead to epithelium becoming a springboard for the development of autoimmune diseases, such as Sjögren’s syndrome, primary biliary cholangitis, autoimmune pancreatitis, or IgG4-related diseases. This book is intended as an introduction to the problem of “autoimmune epithelitis” and diseases closely related to the immune disturbances of the epithelium, with special emphasis on those affecting exocrine glands. Both theoretical and practical knowledge, presented by authors from a wide range of medical specialties, should be of help for medical professionals who have to deal with this difficult problem in their daily practice.