Scleroderma : From Pathogenesis to Comprehensive Management

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. Front Matter....Pages i-xxxv Front Matter....Pages 1-1 Historical Perspective of Scleroderma....Pages 3-7 Front Matter....Pages 9-9 Epidemiology, Environmental, and Infectious Risk Factors....Pages 11-24 Genetic Factors....Pages 25-38 Disease Subsets in Clinical Practice....Pages 39-48 Evolving Concepts of Diagnosis and Classification....Pages 49-64 Overlap Syndromes....Pages 65-74 Juvenile Localized and Systemic Scleroderma....Pages 75-89 Morphea (Localized Scleroderma)....Pages 91-113 Scleroderma Mimics....Pages 115-123 Systems Biology Approaches to Understanding the Pathogenesis of Systemic Sclerosis....Pages 125-129 Front Matter....Pages 131-131 Introduction: The Etiopathogenesis of Systemic Sclerosis – An Integrated Overview....Pages 133-139 Pathology of Systemic Sclerosis....Pages 141-159 Inflammation and Immunity....Pages 161-195 Autoantibodies as Markers and Possible Mediators of Scleroderma Pathogenesis....Pages 197-205 The Clinical Aspects of Autoantibodies....Pages 207-220 Mechanisms of Vascular Disease....Pages 221-244 Biomarkers in Systemic Sclerosis....Pages 245-260 Pathophysiology of Fibrosis in Systemic Sclerosis....Pages 261-280 Overview of Animal Models....Pages 281-293 Front Matter....Pages 295-295 Raynaud’s Phenomenon, Digital Ulcers and Nailfold Capillaroscopy....Pages 297-316 Front Matter....Pages 295-295 Renal Crisis and Other Renal Manifestations of Scleroderma....Pages 317-330 Cardiac Involvement: Evaluation and Management....Pages 331-356 Front Matter....Pages 357-357 Overview of Lung Involvement: Diagnosis, Differential Diagnosis and Monitoring....Pages 359-362 Clinical Assessment of Lung Disease....Pages 363-373 Treatment of Interstitial Lung Disease....Pages 375-383 Pathogenesis of Pulmonary Arterial Hypertension....Pages 385-401 Clinical Assessment of Pulmonary Hypertension....Pages 403-409 The Management of Pulmonary Arterial Hypertension in the Setting of Systemic Sclerosis....Pages 411-419 Front Matter....Pages 421-421 Overview of Gastrointestinal Tract Involvement....Pages 423-426 Upper Gastrointestinal Tract: Manifestations of Systemic Sclerosis....Pages 427-441 Small and Large Intestinal Involvement and Nutritional Issues....Pages 443-458 Front Matter....Pages 459-459 Calcinosis....Pages 461-471 Evaluation and Management of Skin Disease....Pages 473-488 Assessment and Management of Progressive Skin Involvement in Diffuse Scleroderma....Pages 489-498 Skeletal Muscle Involvement....Pages 499-505 Tendons, Joints, and Bone....Pages 507-523 Cancer in Systemic Sclerosis....Pages 525-532 Overlooked Manifestations....Pages 533-550 Pregnancy....Pages 551-561 Front Matter....Pages 563-563 Introduction to Section VIII: Management and Outcome Assessment....Pages 565-566 Front Matter....Pages 563-563 Immunomodulatory, Immunoablative, and Biologic Therapies....Pages 567-582 Investigative Approaches to Drug Therapy....Pages 583-594 Drug Development and Regulatory Considerations for SSc Therapies....Pages 595-601 Physical and Occupational Therapy....Pages 603-613 Psychosocial Issues and Care for Patients with Systemic Sclerosis....Pages 615-621 Clinical Trial Design in Systemic Sclerosis....Pages 623-635 Measuring Disease Activity and Outcomes in Clinical Trials....Pages 637-648 Innovative Approaches to Clinical Trials in Systemic Sclerosis....Pages 649-656 Molecular Stratification by Gene Expression as a Paradigm for Precision Medicine in Systemic Sclerosis....Pages 657-670 Front Matter....Pages 671-671 Managing the Ischemic Finger in Scleroderma....Pages 673-678 Scleroderma Renal Crisis....Pages 679-682 Rapid Diffuse Skin Disease with Progressive Joint Contractures....Pages 683-690 Management of the Scleroderma Patient with Pulmonary Arterial Hypertension Failing Initial Therapy....Pages 691-700 Pseudo-obstruction with Malabsorption and Malnutrition....Pages 701-705 Progressive Interstitial Lung Disease Nonresponse to Cyclophosphamide....Pages 707-712 Coping with the Disfigurement of Scleroderma: Facial, Skin, and Hand Changes....Pages 713-721 Managing Complicated Digital Ulcers....Pages 723-730 Back Matter....Pages 731-743