Comprised of the authoritative work of international experts, this fully-updated second edition ofScleroderma 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.
Section I. Introduction.- Historical Perspective of Scleroderma.-
Section II. Epidemiology, Genetics and Classification.- Epidemiology,
Environmental and Infectious Risk Factors.- Genetic Factors.- Disease Subsets
in Clinical Practice.- Evolving Concepts of Diagnosis and Classification.-
Overlap Syndromes.- Juvenile Localized and Systemic Scleroderma.- Morphea
(Localized Scleroderma).- Scleroderma Mimics.- Systems Biology Approaches to
Understanding the Pathogenesis of Systemic Sclerosis.- Section III: The
Biological Basis of Systemic Sclerosis.- Introduction: The Etiopathogenesis
of Systemic Sclerosis: An Integrated Overview.- Pathology of Systemic
Sclerosis.- Inflammation and Immunity.- Autoantibodies as Markers and
Possible Mediators of Scleroderma Pathogenesis.- The Clinical Aspects of
Autoantibodies.- Mechanisms of Vascular Disease.- Biomarkers in Systemic
Sclerosis.- Pathophysiology of Fibrosis in Systemic Sclerosis.- Overview of
Animal Models.- Section IV. Cardiovascular Manifestations and Management.-
Raynauds Phenomenon, Digital Ulcers, and Nailfold Capillaroscopy.- Renal
Crisis and Other Renal Manifestations of Scleroderma.- Cardiac Involvement.-
Section V. Pulmonary Manifestations and Management.- Overview of Lung
Involvement: Diagnosis, Differential Diagnosis, and Monitoring.- Clinical
Assessment of Lung Disease.- Treatment of Interstitial Lung Disease.-
Pathogenesis of Pulmonary Arterial Hypertension.- Clinical Assessment of
Pulmonary Hypertension.- The Management of Pulmonary Arterial Hypertension in
the Setting of Systemic Sclerosis.- Section VI. Gastrointestinal
Manifestations and Management.- Overview of Gastrointestinal Tract
Involvement.- Upper Gastrointestinal Tract: Manifestations of Systemic
Sclerosis.- Small and Large Intestinal Involvement and Nutritional Issues.-
Section VII. Skin, Musculoskeletal and Other Complications.- Calcinosis.-
Evaluation and Management of Skin Disease.- Assessment and Management of
Progressive Skin Involvement in Diffuse Scleroderma.- Skeletal Muscle
Involvement.- Tendons, Joints, and Bone.- Cancer in Systemic Sclerosis.-
Overlooked Manifestations.- Pregnancy.- Section VIII: Management and
Outcome Assessment.- Introduction to Section VIII: Management and Outcome
Assessment.- Immunomodulatory, Immunoablative, and Biologic Therapies.-
Investigative Approaches to Drug Therapy.- Drug Development and Regulatory
Considerations for SSc Therapies.- Physical and Occupational Therapy.-
Psychosocial Issues and Care for Patients with Systemic Sclerosis.- Clinical
Trial Design in Systemic Sclerosis.- Measuring Disease Activity and Outcomes
in Clinical Trials.- Innovative Approaches to Clinical Trials in Systemic
Sclerosis.- Molecular Stratification by Gene Expression as a Paradigm for
Precision Medicine in Systemic Sclerosis.- Section IX. Case Studies: Approach
to Complex Clinical Problems.- Managing the Ischemic Finger in Scleroderma.-
Scleroderma Renal Crisis.- Rapid Diffuse Skin Disease with Progressive Joint
Contractures.- Management of the Scleroderma Patient with Pulmonary Arterial
Hypertension Failing Initial Therapy.- Pseudo-obstruction with Malabsorption
and Malnutrition.- Progressive Interstitial Lung Disease Non-Response to
Cyclophosphamide.- Coping with the Disfigurement of Scleroderma: Facial,
Skin, and Hand Changes.- Managing Complicated Digital Ulcers
John Varga, MD
John and Nancy Hughes Distinguished Professor
Feinberg School of Medicine
Director, Northwestern Scleroderma Program
Chicago, Illinois, USA
Christopher P. Denton, PhD, FRCP
Professor of Experimental Rheumatology
Centre for Rheumatology and Connective Tissue Diseases
Division of Medicine
University College London
London, United Kingdom
Fredrick M. Wigley, MD
Martha McCrory Professor of Medicine
Department of Medicine/Rheumatology
The Johns Hopkins University School of Medicine
Baltimore, Maryland, USA
Yannick Allanore, MD
Service de Rhumatologie A
Université Paris Descartes,
Hôpital Cochin
Paris, France
Masataka Kuwana, MD, PhD
Professor
Department of Allergy and Rheumatology
Nippon Medical School GraduateSchool of Medicine
Tokyo, Japan