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Systemic sclerosis (SSc) is a multisystem autoimmune disease involving vasculopathy and immune activation that leads to the deposition of extracellular matrix and the development of fibrosis, resulting in cardinal clinical problems. Systemic Sclerosis - Recent Advances and New Perspectives provides a comprehensive overview of the pathophysiology, diagnosis, and specific management of the clinical manifestations of SSc. The book addresses the current understanding of the causes and consequences of inflammatory activation, chromosomal instability, and activating factors. Special emphasis is placed on diagnosis, from the recognition of very early systemic sclerosis and consideration of its treatment to diagnostic methods for microvascular and pulmonary involvement. It also highlights the less life-threatening but very common manifestations of bone and skeletal muscle involvement, which affect patient quality of life.
Systemic sclerosis (scleroderma) is an incurable connective tissue disease of unknown etiology. Three key processes play a pivotal role in the pathogenesis: immune dysregulation and inflammation, endothelial injury and vasculopathy, and fibrosis. Tissue fibrosis is the dominant and characteristic feature that affects the skin and visceral organs. Life expectancy of scleroderma patients has improved over recent years, mainly because of better treatment of organ involvement and complications; however, no curative disease-modifying therapies exist to date. This book aims to provide students, trainees, rheumatologists, and other specialists interested in this disease with a comprehensive overview of novel pathogenetic mechanisms, management approaches, and therapeutic targets of several major vascular and fibrotic manifestations, and is useful insight into a number of usually neglected aspects of scleroderma.
Systemic sclerosis (scleroderma) is an incurable connective tissue disease of unknown etiology. Three key processes play a pivotal role in the pathogenesis: immune dysregulation and inflammation, endothelial injury and vasculopathy, and fibrosis. Tissue fibrosis is the dominant and characteristic feature that affects the skin and visceral organs. Life expectancy of scleroderma patients has improved over recent years, mainly because of better treatment of organ involvement and complications; however, no curative disease-modifying therapies exist to date. This book aims to provide students, trainees, rheumatologists, and other specialists interested in this disease with a comprehensive overview of novel pathogenetic mechanisms, management approaches, and therapeutic targets of several major vascular and fibrotic manifestations, and is useful insight into a number of usually neglected aspects of scleroderma.
Comprised of the authoritative work of international experts, this fully-updated second edition of Scleroderma builds upon the well-regarded approach in the first edition to provide integrated, concise, and up-to-date synthesis of current concepts of pathogenesis and modern approaches to management of systemic sclerosis (scleroderma). With a multidisciplinary approach to comprehensive care, this book is easily accessible for health care professionals in many fields. The new edition includes extensive updated material based on major developments in the field, with new chapters on personalized medicine, cancer complications, global perspectives on scleroderma, and more. It presents a succinct and thoughtful synthesis of current pathomechanistic concepts, providing a valuable reference tool for basic and translational investigators working in the field. Scleroderma: From Pathogenesis to Comprehensive Management serves as an essential, all-inclusive resource for rheumatologists, pulmonologists, cardiologists, gastroenterologists, nephrologists and all those involved in the care of scleroderma patients.
Copy 2, Gift of Mrs. E. Carwile LeRoy, 2009.
Interstitial Lung Diseases: New Insights for the Healthcare Professional: 2013 Edition is a ScholarlyBrief™ that delivers timely, authoritative, comprehensive, and specialized information about Diagnosis and Screening in a concise format. The editors have built Interstitial Lung Diseases: New Insights for the Healthcare Professional: 2013 Edition on the vast information databases of ScholarlyNews.™ You can expect the information about Diagnosis and Screening in this book to be deeper than what you can access anywhere else, as well as consistently reliable, authoritative, informed, and relevant. The content of Interstitial Lung Diseases: New Insights for the Healthcare Professional: 2013 Edition has been produced by the world’s leading scientists, engineers, analysts, research institutions, and companies. All of the content is from peer-reviewed sources, and all of it is written, assembled, and edited by the editors at ScholarlyEditions™ and available exclusively from us. You now have a source you can cite with authority, confidence, and credibility. More information is available at http://www.ScholarlyEditions.com/.
Raynaud’s Phenomenon: A Guide to Pathogenesis and Treatment comprehensively reviews the understanding of a disorder that continues to challenge primary care clinicians and specialists alike. In the last decade, there have been important advances not only in understanding the pathophysiology of Raynaud’s Phenomenon (RP), but also in developing diagnostic methods and effective drug and non-drug therapies. Thoroughly discussing the various manifestations of RP, including childhood RP, RP secondary to connective tissue disease, and a variety of other associated disorders that include vascular perturbation that mimics RP, this title provides a wealth of new information available on normal and abnormal thermoregulation and helps physicians identify the best therapeutic approaches to treating RP. The work offers differential diagnosis options, reviews potential causes such as autoimmune disease, industrial trauma, drugs, and metabolic causes and provides clear recommendations for therapy. Engaging readers with case vignettes and a plethora of visual aids, Raynaud’s Phenomenon: A Guide to Pathogenesis and Treatment is a state-of-the-art, authoritative reference and invaluable contribution to the literature that will be of interest to physicians, patients, and individuals dealing with these disease processes.
Since the discovery of the circulating “fibrocyte” in 1994 as a collagen-producing cell of the peripheral blood, the physiologic and pathologic role of this unique cell populaton has grown steadily. This pioneering new book provides the first comprehensive review of the role of fibrocytes in wound repair, granuloma formation, antigen presentation, scar formation, and various fibrosing disorders such as interstitial lung disease and nephrogenic systemic fibrosis. It also includes discussions of the recent studies on the molecular signals that influence fibrocyte migration, proliferation, and function in the context of normal physiology and pathology. The chapters are contributed by the leading researchers in the field.
Gamma/delta (γδ) T-cells are a small subset of T-lymphocytes in the peripheral circulation but constitute a major T-cell population at other anatomical localizations such as the epithelial tissues. In contrast to conventional α/β T-cells, the available number of germline genes coding for T-cell receptor (TCR) variable elements of γδ T-cells is very small. Moreover, there is a prefential localization of γδ T-cells expressing given Vgamma and Vdelta genes in certain tissues. In humans, γδ T-cells expressing the Vg9Vd2-encoded TCR account for anywhere between 50 and >95% of peripheral blood γδ T-cells, whereas cells expressing non-Vd2 genes dominate in mucosal tissues. In mice, there is an ordered appearance of γδ T-cell „waves“ during embryonic development, resulting in preferential localization of γδ T-cells expressing distinct VgammaVdelta genes in the skin, the reproductive organs, or gut epithelia. The major function of γδ T-cells resides in local immunosurveillance and immune defense against infection and malignancy. This is supported by the identification of ligands that are selectively recognized by the γδ TCR. As an example, human Vgamma9Vdelta2 T-cells recognize phosphorylated metabolites („phosphoantigens“) that are secreted by many pathogens but can also be overproduced by tumor cells, providing a basis for a role of these γδ T-cells in both anti-infective and anti-tumor immunity. Similarly, the recognition of endothelial protein C receptor by human non-Vdelta2 γδ T-cells has recently been identified to provide a link for the role for such γδ T-cells in immunity against epithelial tumor cells and cytomegalovirus-infected endothelial cells. In addition to „classical“ functions such as cytokine production and cytotoxicity, recent studies suggest that subsets of γδ T-cells can exert additional functions such as regulatory activity and – quite surpisingly – „professional“ antigen-presenting capacity. It is currently not well known how this tremendous extent of functional plasticity is regulated and what is the extent of γδ TCR ligand diversity. Due to their non-MHC-restricted recognition of unusual stress-associated ligands, γδ T-cells have raised great interest as to their potential translational application in cell-based immunotherapy. Topics of this Research Focus include: Molecular insights into the activation and differentiation requirements of γδ T-cells, role of pyrophosphates and butyrophilin molecules for the activation of human γδ T-cells, role of γδ T-cells in tumor immunity and in other infectious and non-infectious diseases, and many others. We are most grateful to all colleagues who agreed to write a manuscript. Thanks to their contributions, this E-book presents an up-to-date overview on many facets of the still exciting γδ T-cells. Dieter Kabelitz & Julie Déchanet-Merville