Schoenfeld SR, Castelino FV. Interstitial lung disease in scleroderma. Rheum Dis Clin North Am. 2015;41: 237-248.
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Berezne A, Ranque B, Valeyre D, et al. Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol. 2008;35:1064-72.
Sottile PD, Iturbe D, Katsumoto TR, et al. Outcomes in systemic sclerosis-related lung disease after lung transplantation. Transplantation. 2013;95:975-980.
Schachna L, Medsger TA Jr, Dauber JH, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2006;54:3954-3961.
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Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49:271-280.
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King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083-2092.
Seibold JR, Denton CP, Furst DE, et al. Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. Arthritis Rheum. 2010;62:2101-2108.
Khanna D, Saggar R, Mayes MD, et al. A one-year, phase I/IIa, open-label pilot trial of imatinib mesylate in the treatment of systemic sclerosis-associated active interstitial lung disease. Arthritis Rheum. 2011;63:3540-3546.
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Burt RK, Shah SJ, Dill K, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet. 2011;378:498-506.
van Laar JM, Farge D, Sont JK, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA. 2014;311:2490-2498.
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Khanna D, Nagaraja V, Tseng CH, et al. Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis-associated interstitial lung disease trials. Arthritis Res Ther. 2015;17:372.
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Hoffmann-Vold AM, Aaloken TM, Lund MB, et al. Predictive value of serial high-resolution computed tomography analyses and concurrent lung function tests in systemic sclerosis. Arthritis Rheumatol. 2015;67:2205-2212.
Flaherty KR, Mumford JA, Murray S, et al. Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003;168:543-548.
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Caron M, Hoa S, Hudson M, et al. Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease. Eur Respir Rev. 2018;27. pii: 170102. doi: 10.1183/16000617.0102-2017.
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Winstone TA, Assayag D, Wilcox PG, et al. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest. 2014;146:422-436.
Lopes AJ, Capone D, Mogami R, et al. Systemic sclerosis-associated interstitial pneumonia: evaluation of pulmonary function over a five-year period. J Bras Pneumol. 2011;37:144-151.
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Kowal-Bielecka O, Chwiesko-Monarowska S, Bernatowicz PL, et al. The arachidonate 5-lipoxygenase activating protein gene polymorphism is associated with the risk of scleroderma-related interstitial lung disease: a multicentre European Scleroderma Trials and Research group (EUSTAR) study. Rheumatology. 2017;56:844-852.
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Schupp J, Becker M, Gunther J, et al. Serum CCL18 is predictive for lung disease progression and mortality in systemic sclerosis. Eur Respir J. 2014;43:1530-1532.
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CPD

Key Advances in the Treatment of Systemic Sclerosis Interstitial Lung Disease (SSc-ILD)

Authors: Oliver Distler, MD; Jacob van Laar, MD, PhD; Vanessa Smith, MD, PhD; Anna-Maria Hoffmann-Vold, MD, PhD; Yannick Allanore, MD, PhD
CPD Released: 8/29/2018
THIS ACTIVITY HAS EXPIRED FOR CREDIT
Valid for credit through: 8/29/2019, 11:59 PM EST

Target Audience and Goal Statement

This educational activity is intended for an international audience of non-US rheumatologists, pulmonologists, allergists, and clinical immunologists.

The treatment landscape for patients with SSc-ILD is evolving. Physicians have a critical need to understand the pathogenesis of SSc-ILD, the rationale and mode of action for the development of new treatments, and the efficacy and safety profiles of current and emerging therapies.

Upon completion of this activity, participants will have increased knowledge regarding the:

Complexity of SSc-ILD and evolving treatment landscape
Key data on current and emerging treatments for SSc-ILD
Expert opinions on the implications for clinical practice

Disclosures

WebMD Global requires each individual who is in a position to control the content of one of its educational activities to disclose any relevant financial relationships occurring within the past 12 months that could create a conflict of interest.

Moderator

Oliver Distler, MD

Professor
Department of Rheumatology
University Hospital Zurich
Zurich, Switzerland

Disclosures

Disclosure: Oliver Distler, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: AbbVie Inc.; AnaMar; Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; CSL Behring; GlaxoSmithKline; Inventiva; Italfarmaco; Medac; Merck & Co., Inc.; Mitsubishi Tanabe Pharma; Novartis Pharmaceuticals Corporation; Pfizer Inc.; Target BioScience AG; UCB Pharma, Inc.
Received grants for clinical research from: Actelion Pharmaceuticals, Ltd; Bayer HealthCare; Mitsubishi Tanabe Parma; Roche Holdings AG

Faculty

Yannick Allanore, MD, PhD

Professor
Paris Descartes University
Cochin Hospital
Paris, France

Disclosures

Disclosure: Yannick Allanore, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Actelion Pharmaceuticals, Ltd; Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; Inventiva; Medac; Roche Holdings AG
Received grants for clinical research from: Roche Holdings AG; Inventiva

Anna -Maria Hoffmann-Vold, MD, PhD

Doctor
Oslo University Hospital
Oslo, Norway

Disclosures

Disclosure: Anna-Maria Hoffmann-Vold, MD, PhD, has disclosed no relevant financial relationships.

Vanessa Smith, MD, PhD

Professor
Ghent University
Ghent University Hospital
Ghent, Oost Vlaanderen, Belgium

Disclosures

Disclosure: Vanessa Smith, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Actelion Pharmaceuticals, Ltd; Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.
Served as a speaker or a member of a speakers bureau for: Accord Healthcare; Actelion Pharmaceuticals, Ltd
Received grants for clinical research from: Actelion Pharmaceuticals, Ltd; Belgium Fund for Scientific Research in Rheumatic diseases; Fund of Scientific Research Flanders; Galapagos NV; Roche Holdings AG

Jacob van Laar, MD, PhD

Professor
Department of Rheumatology and Clinical Immunology
University Medical Center Utrecht
Utrecht, the Netherlands

Disclosures

Disclosure: Jacob van Laar, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Eli Lilly and Company; Merck Sharp & Dohme Corp.; Roche
Received grants for clinical research from: AstraZeneca Pharmaceuticals LP; Merck Sharp & Dohme Corp.; Roche

Steering Committee

Luca Richeldi, MD, PhD (Chair)

Professor
Catholic University of the Sacred Heart
Agostino Gemelli University Hospital
Rome, Italy

Disclosures

Disclosure: Luca Richeldi, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Biogen Idec Inc.; Boehringer Ingelheim Pharmaceuticals, Inc.; InterMune, Inc.; Roche
Served as a speaker or a member of a speakers bureau for: Boehringer Ingelheim Pharmaceuticals, Inc.; Cipla Ltd.; Roche; Vertex Pharmaceuticals Incorporated
Received grants for clinical research from: InterMune, Inc.

Members

Kevin Brown, MD

Professor
Vice Chair
National Jewish Health
Denver, Colorado, United States

Disclosures

Disclosure: Kevin Brown, MD, has disclosed the following relevant financial relationships:
Other: Aeolus Pharmaceuticals, Inc.; AstraZeneca Pharmaceuticals LP; aTyr Pharma, Inc.; Biogen Idec Inc.; Boehringer Ingelheim Pharmaceuticals, Inc.; FibroGen, Inc.; Galapagos NV; Galecto Biotech; Genentech, Inc.; Gilead Sciences, Inc.; MedImmune Inc.; Novartis Pharmaceuticals Corporation; Patara Pharma, LLC; ProMetic Pharma Inc.; Roche; Third Pole Therapeutics

Vincent Cottin, MD, PhD

Professor
University of Lyon
Hôpital Louis Pradel
Lyon, France

Disclosures

Disclosure: Vincent Cottin, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Boehringer Ingelheim Pharmaceuticals, Inc.; Galapagos NV; Gilead Sciences, Inc.; Promedior; Roche
Served as a speaker or a member of a speakers bureau for: Boehringer Ingelheim Pharmaceuticals, Inc.; Roche
Received grants for clinical research from: Boehringer Ingelheim Pharmaceuticals, Inc.; Roche

Christopher P. Denton, PhD, FRCP

Professor
Royal Free Hospital and University College London
London, United Kingdom

Disclosures

Disclosure: Christopher P. Denton, PhD, FRCP, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Actelion Pharmaceuticals, Ltd; Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; Corbus Pharmaceuticals Holdings, Inc.; Inventiva; Sanofi Aventis; Roche
Received grants for clinical research from: CSL Behring; GlaxoSmithKline; Inventiva; Roche

Marco Matucci-Cerenic, MD, PhD

Professor
University of Florence
Florence, Italy

Disclosures

Disclosure: Marco Matucci-Cerenic, MD, PhD, has disclosed the following relevant financial relationships:
Served as a speaker or a member of a speakers bureau for: Actelion Pharmaceuticals, Ltd; Bristol-Myers Squibb Company; Pfizer Inc.
Received grants for clinical research from: Actelion Pharmaceuticals, Ltd

Madelon M.C. Vonk, MD, PhD

Assistant Professor
Radboud University Medical Centre
Nijmegen, the Netherlands

Disclosures

Disclosure: Madelon M.C. Vonk, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Actelion Pharmaceuticals, Ltd; Boehringer Ingelheim Pharmaceuticals, Inc.
Received grants for clinical research from: Actelion Pharmaceuticals, Ltd; Therabel Pharma NV

Editor

Marinella Calle, PhD, CMPP

Scientific Director, WebMD Global, LLC

Disclosures

Disclosure: Marinella Calle, PhD, CMPP, has disclosed no relevant financial relationships.

Walid Amara, MD

Scientific Director, WebMD Global, LLC

Disclosures

Walid Amara,MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: BIOTRONIK; SERVIER
Served as a speaker or a member of a speakers bureau for: Bayer HealthCare Pharmaceuticals; Boehringer Ingelheim Pharmaceuticals, Inc.; Boston Scientific; Bristol-Myers Squibb Company; Livanova; MEDA; Medtronic, Inc.; MSD; Novartis; Pfizer Inc.; Physiomed; St. Jude;Servier

Content Reviewer

Nafeez Zawahir, MD

CME Clinical Director, Medscape, LLC

Disclosures

Disclosure: Nafeez Zawahir, MD, has disclosed no relevant financial relationships.

Accreditation Statements

For Physicians

The Faculty of Pharmaceutical Medicine of the Royal Colleges of Physicians of the United Kingdom (FPM) has reviewed and approved the content of this educational activity and allocated it 0.50 continuing professional development credits (CPD).

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From Medscape Education

CPD

Key Advances in the Treatment of Systemic Sclerosis Interstitial Lung Disease (SSc-ILD)

Authors: Oliver Distler, MD; Jacob van Laar, MD, PhD; Vanessa Smith, MD, PhD; Anna-Maria Hoffmann-Vold, MD, PhD; Yannick Allanore, MD, PhDFaculty and Disclosures

THIS ACTIVITY HAS EXPIRED FOR CREDIT

CPD Released: 8/29/2018

Valid for credit through: 8/29/2019, 11:59 PM EST

processing....

Contents of This CPD Activity

All sections of this activity are required for credit.

Section 1. Key Advances in the Treatment of Systemic Sclerosis Interstitial Lung Disease (SSc-ILD)

Dr Distler introduces the program and presents the different segments
Oliver Distler, MD

Section 2. Pathogenesis of SSc-ILD and Rationale for New Treatment Options

Dr van Laar presents the mechanisms implicated in SSc-ILD pathogenesis and discusses current and investigational treatments
Jacob van Laar, MD, PhD

Section 3. Overview of SSc-ILD Burden and Unmet Needs

Dr Smith presents the unmet needs in SSc-ILD and prognostic factors of this pathology
Vanessa Smith, MD, PhD

Section 4. Identifying SSc-ILD Patients at Risk of Progression

Dr Hoffmann-Vold addresses SSc-ILD progression and the factors, particularly biomarkers, than can be used to identify a risk of progression
Anna-Maria Hoffmann-Vold, MD, PhD

Section 5. Established Treatment Options for SSc-ILD

Dr Allanore presents the key data for established treatment options of SSc-ILD
Yannick Allanore, MD, PhD

Section 6. Emerging and Investigational Treatments for SSc-ILD

Dr Distler presents the rationale and the key data for antifibrotic therapies in SSc-ILD
Oliver Distler, MD

Educational Impact Challenge

Before you begin this activity, please assess your clinical knowledge by completing this brief survey. Answering these questions again after the activity will allow you to see what you learned and to compare your answers with those of your peers.

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Disclaimer

The educational activity presented above may involve simulated case-based scenarios. The patients depicted in these scenarios are fictitious and no association with any actual patient is intended or should be inferred.

The material presented here does not necessarily reflect the views of WebMD Global, LLC or companies that support educational programming on medscape.org. These materials may discuss therapeutic products that have not been approved by the European Medicines Agency for use in Europe and off-label uses of approved products. A qualified healthcare professional should be consulted before using any therapeutic product discussed. Readers should verify all information and data before treating patients or employing any therapies described in this educational activity.

References

Faculty and Disclosures

Moderator

Oliver Distler, MD

Faculty

Yannick Allanore, MD, PhD

Anna -Maria Hoffmann-Vold, MD, PhD

Vanessa Smith, MD, PhD

Jacob van Laar, MD, PhD

Steering Committee

Luca Richeldi, MD, PhD (Chair)

Members

Kevin Brown, MD

Vincent Cottin, MD, PhD

Christopher P. Denton, PhD, FRCP

Marco Matucci-Cerenic, MD, PhD

Madelon M.C. Vonk, MD, PhD

Editor

Marinella Calle, PhD, CMPP

Walid Amara, MD

Content Reviewer

Nafeez Zawahir, MD

CPD

Key Advances in the Treatment of Systemic Sclerosis Interstitial Lung Disease (SSc-ILD)

Target Audience and Goal Statement

Disclosures

Moderator

Oliver Distler, MD

Faculty

Yannick Allanore, MD, PhD

Anna -Maria Hoffmann-Vold, MD, PhD

Vanessa Smith, MD, PhD

Jacob van Laar, MD, PhD

Steering Committee

Luca Richeldi, MD, PhD (Chair)

Members

Kevin Brown, MD

Vincent Cottin, MD, PhD

Christopher P. Denton, PhD, FRCP

Marco Matucci-Cerenic, MD, PhD

Madelon M.C. Vonk, MD, PhD

Editor

Marinella Calle, PhD, CMPP

Walid Amara, MD

Content Reviewer

Nafeez Zawahir, MD

Accreditation Statements

For Physicians

Instructions for Participation and Credit

Key Advances in the Treatment of Systemic Sclerosis Interstitial Lung Disease (SSc-ILD)

Contents of This CPD Activity

Educational Impact Challenge