Front Matter
    Pages i-xxi
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    Introduction
        Front Matter
        Pages 1-1
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        Historical Perspective of Scleroderma
            Christopher P. Denton, Marco Matucci-Cerinic
        Pages 3-7
    Epidemiology, Genetics and Classification
        Front Matter
        Pages 9-9
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        Epidemiology, Environmental, and Infectious Risk Factors
            Bochra Jandali, Luc Mouthon, Maureen D. Mayes
        Pages 11-26
        Influence of Ethnicity and Sex Bias in Systemic Sclerosis
            Pravitt Gourh, Cosimo Bruni
        Pages 27-35
        Genetic Factors
            Shervin Assassi, Javier Martin, Yannick Allanore
        Pages 37-52
        Classification and Disease Subsets in Clinical Practice
            Sindhu R. Johnson, Frank H. J. Van den Hoogen, Robyn T. Domsic
        Pages 53-70
        Clustering and Evolving Concepts for Subclassification
            Vincent Sobanski, Monique Hinchcliff
        Pages 71-78
        The Clinical Aspects of Autoantibodies
            Masataka Kuwana, Jeska de Vries-Bouwstra
        Pages 79-94
        Systemic Sclerosis Overlap Syndromes
            Pia Moinzadeh, Christopher P. Denton
        Pages 95-105
        Juvenile Localized and Systemic Scleroderma
            Lauren V. Graham, Amy S. Paller, Ivan Foeldvari
        Pages 107-121
        Morphea (Localized Scleroderma)
            Noelle M. Teske, Heidi T. Jacobe
        Pages 123-147
        Scleroderma Mimics
            Laura K. Hummers, Alan Tyndall
        Pages 149-157
    The Biological Basis of Systemic Sclerosis
        Front Matter
        Pages 159-159
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        Introduction: The Etiopathogenesis of Systemic Sclerosis—An Integrated Overview
            Carol Feghali-Bostwick, David J. Abraham, John Varga
        Pages 161-169
        Pathology of Systemic Sclerosis
            Lisa M. Rooper, Frederic B. Askin
        Pages 171-190
        Inflammation and Immunity in Systemic Sclerosis
            Francesco Boin, Armando Gabrielli, Carlo Chizzolini
        Pages 191-203
        Autoantibodies as Markers and Possible Mediators of Scleroderma Pathogenesis
            Christopher A. Mecoli, Kimberly Doering Maurer, Antony Rosen, Livia Casciola-Rosen
        Pages 205-216
        Mechanisms of Vascular Disease
            Mirko Manetti, Bashar Kahaleh
        Pages 217-246

    Biomarkers in Systemic Sclerosis
        Francesco Del Galdo, Elizabeth R. Volkmann, Robert Lafyatis
    Pages 247-263
    Cellular and Molecular Mechanisms of Fibrosis in Systemic Sclerosis
        Maria Trojanowska, John Varga, David Lagares
    Pages 265-289
    Overview of Animal Models
        Yoshihide Asano, Jörg H. W. Distler
    Pages 291-306

Cardiovascular Manifestations and Management

    Front Matter
    Pages 307-307
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    Raynaud’s Phenomenon, Digital Ulcers and Nailfold Capillaroscopy
        Michael Hughes, Marco Matucci-Cerinic
    Pages 309-322
    Renal Crisis and Other Renal Manifestations
        Christopher P. Denton, Marie Hudson
    Pages 323-338
    Cardiac Involvement: Evaluation and Management
        Luna Gargani, Matteo Mazzola, Sanjiv J. Shah
    Pages 339-366

Pulmonary Manifestations and Management

    Front Matter
    Pages 367-367
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    Overview of Lung Involvement
        Athol U. Wells
    Pages 369-375
    Clinical Assessment of ILD
        Anna-Maria Hoffmann-Vold, Kristin B. Highland, Elisabeth Bendstrup
    Pages 377-387
    Lung Imaging Perspective in Scleroderma
        Jonathan G. Goldin
    Pages 389-399
    Treatment of Interstitial Lung Disease in Systemic Sclerosis
        Muriel Elhai, Bruno Crestani, Maria-Sinziana Muraru-Carbrune, Yannick Allanore
    Pages 401-419
    Pathogenesis of Pulmonary Arterial Hypertension
        Markella Ponticos, Michael H. Lee, Alan M. Holmes, Rubin M. Tuder
    Pages 421-444
    Clinical Assessment of Pulmonary Hypertension
        Stephanie M. Hon, Benoit Lechartier, Marc Humbert, Harrison W. Farber
    Pages 445-453
    The Management of Pulmonary Arterial Hypertension in the Setting of Systemic Sclerosis
        Stephen C. Mathai, Olivier Sitbon
    Pages 455-467

Gastrointestinal Manifestations and Management

    Front Matter
    Pages 469-469
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    Overview of Gastrointestinal Tract Involvement
        Christopher P. Denton
    Pages 471-475
    Upper Gastrointestinal Tract: Manifestations of Systemic Sclerosis
        John O. Clarke, Nitin K. Ahuja
    Pages 477-493
    Lower Gastrointestinal Tract Involvement: Understanding the Interplay of Motility, the Microbiome, and Nutrition
        Elizabeth R. Volkmann, Zsuzsanna McMahan
    Pages 495-513

Skin, Musculoskeletal and Other Complications

    Front Matter
    Pages 515-515

    Evaluation and Management of Skin Disease
        Nicolas Hunzelmann, Flavia V. Castelino
    Pages 517-526
    Calcinosis
        Antonia Valenzuela, Lorinda Chung
    Pages 527-542
    Tendons, Joints, and Bone
        Elisabetta Zanatta, Maya H. Buch, Yannick Allanore
    Pages 543-566
    Skeletal Muscle Involvement
        Julie J. Paik, Britta Maurer
    Pages 567-573
    Cancer in Systemic Sclerosis
        Ami A. Shah, Masataka Kuwana
    Pages 575-586
    Overlooked Manifestations
        Ami A. Shah, Fredrick M. Wigley
    Pages 587-611
    Pregnancy
        Melody Chung, Lorinda Chung
    Pages 613-623
    Vaccinations in Patients with Systemic Sclerosis
        Elana J. Bernstein, John D. Pauling
    Pages 625-635

Management and Outcome Assessment

    Front Matter
    Pages 637-637
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    Overview of Management and Outcome Assessment in Systemic Sclerosis
        Christopher P. Denton, Ami A. Shah, Yannick Allanore
    Pages 639-644
    Management of Progressive Skin Involvement in Diffuse Scleroderma
        Eric Hachulla, Ariane L. Herrick
    Pages 645-655
    Hematopoietic Stem Cell Transplantation
        Jacob M. van Laar, Julia Spierings, Oliver Distler
    Pages 657-664
    Immune-Targeted Therapies in SSc
        Voon H. Ong, Yannick Allanore
    Pages 665-676
    Physical and Occupational Therapy
        Luc Mouthon, Christelle Nguyen, Janet L. Poole
    Pages 677-690
    Psychosocial Issues and Care for Patients with Systemic Sclerosis
        Brett D. Thombs, Linda Kwakkenbos
    Pages 691-699
    Measuring Disease Activity and Outcomes in Clinical Trials
        Dinesh Khanna, Alain Lescoat
    Pages 701-716
    Evolving Clinical Trial Design and Innovative Approaches
        Christopher P. Denton, Daniel E. Furst, Ulf Muller-Ladner, Dinesh Khanna
    Pages 717-727
    Drug Development and Regulatory Considerations for Systemic Sclerosis Therapies
        Peter K. Wung, Jean-Marc Guettier
    Pages 729-737

Case Studies: Approach to Complex Clinical Problems

    Front Matter
    Pages 739-739
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    Managing the Ischemic Finger in Scleroderma
        Fredrick M. Wigley, Thomas Reifsnyder
    Pages 741-748

    Scleroderma Renal Crisis: A Case Study
        Edward P. Stern, Christopher P. Denton
    Pages 749-751
    Rapid Diffuse Skin Disease with Progressive Joint Contractures
        Faye N. Hant, Richard M. Silver
    Pages 753-761
    Management of the Scleroderma Patient with Pulmonary Arterial Hypertension Failing Initial Therapy
        Christopher J. Mullin, Stephen C. Mathai
    Pages 763-773
    Pseudo-obstruction with Malabsorption and Malnutrition
        Tracy M. Frech
    Pages 775-778
    Progressive Interstitial Lung Disease Non-Responsive to Immunosuppressive Therapy
        Katherine C. Silver, Kristin B. Highland, Richard M. Silver
    Pages 779-786
    Body Image Distress in Scleroderma
        Shadi Gholizadeh, Rina S. Fox, Sarah D. Mills, Lisa R. Jewett, Brett D. Thombs, Vanessa L. Malcarne
    Pages 787-796
    Managing Complicated Digital Ulcers
        John D. Pauling, Marco Matucci-Cerinic
    Pages 797-805
    Lower Limb Ulceration
        Muditha Samaranayaka, Ariane L. Herrick, Nuala O’Donoghue
    Pages 807-814

Back Matter
Pages 815-835