Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733-748.
American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165:277-304.
Fischer A, Antoniou KM, Brown KK, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46:976-987.
Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4:e000212.
Wells AU, Brown KK, Flaherty KR, et al. What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51:1800692.
Olson A, Hartmann N, Schlenker-Herceg R, et al. Prevalence of progressive fibrosing interstitial lung disease. Poster presented at: European Respiratory Society International Congress; September 15-19, 2018; Paris, France. Poster 62357.
Dellaripa PF. Interstitial lung disease in the connective tissue diseases; a paradigm shift in diagnosis and treatment. Clin Immunol. 2018;186:71-73.
Wu W, Jordan S, Becker MO, et al. Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model. Ann Rheum Dis. 2018; 77:1326-1332.
Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177:1248-1254.
Zhang XJ, Bonner A, Hudson M, et al. Association of gastroesophageal factors and worsening of forced vital capacity in systemic sclerosis. J Rheumatol. 2013;40:850-858.
Salazar GA, Kuwana M, Wu M, et al. KL-6 but not CCL-18 is a predictor of early progression in systemic sclerosis-related interstitial lung disease. J Rheumatol. 2018;45:1153-1158.
Chowaniec M, Skoczyńska M, Sokolik R, et al. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56:249-254.
Wells AU, Denton C. Interstitial lung disease in connective tissue disease--mechanisms and management. Nat Rev Rheumatol. 2014;10:728-739.
Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655-2666.
Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4:708-719.
ClinicalTrials.gov. A study of the efficacy and safety of tocilizumab in participants with systemic sclerosis (SSc) (focuSSced). https://clinicaltrials.gov/ct2/show/NCT02453256. Accessed October 23, 2018.
ClinicalTrials.gov. A trial to compare nintedanib with placebo for patients with scleroderma related lung fibrosis. https://clinicaltrials.gov/ct2/show/NCT02597933. Accessed October 23, 2018.
ClinicalTrials.gov. Scleroderma Lung Study III - combining pirfenidone with mycophenolate (SLSIII). https://clinicaltrials.gov/ct2/show/NCT03221257. Accessed October 23, 2018.
Khanna D, Albera C, Fischer A, et al. An open-label, phase II study of the safety and tolerability of pirfenidone in patients with scleroderma-associated interstitial lung disease: the LOTUSS Trial. J Rheumatol. 2016;43:1672-1679.
ClinicalTrials.gov. Safety and tolerability of pirfenidone in participants with systemic sclerosis-related interstitial lung disease (SSc-ILD) (LOTUSS). https://clinicaltrials.gov/ct2/show/NCT01933334. Accessed October 23, 2018.
Distler O, Brown KK, Distler JHW, et al. Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS™). Clin Exp Rheumatol. 2017;35:75-81.
Khanna D, Denton CP, Jahreis A, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet. 2016;387:2630-2640.
Khanna D, Jahreis A, Furst DE. Tocilizumab treatment of patients with systemic sclerosis: clinical data. J Scleroderma Relat Disord 2017;2:S29-S35.
ClinicalTrials.gov. A study of pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease. https://clinicaltrials.gov/ct2/show/NCT03099187. Accessed October 23, 2018.
Maher TM, Corte TJ, Fischer A, et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: design of a double-blind, randomised, placebo-controlled phase II trial. BMJ Open Respir Res. 2018;5:e000289.
ClinicalTrials.gov. Efficacy and safety of nintedanib in patients with progressive fibrosing interstitial lung disease (PF-ILD) (INBUILD®). https://clinicaltrials.gov/ct2/show/NCT02999178. Accessed October 23, 2018.
Nihtyanova PI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol. 2014;66:1625-1635.
Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760-1769.
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.
Noble PW, Albera C, Bradford WZ, et al. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47:243-253.
Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365:1079-1087.
Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071-2082.

Faculty

Athol Wells, MD

Professor
Interstitial Lung Disease Unit
Royal Brompton and Harefield NHS Foundation Trust
London, United Kingdom

Disclosures

Disclosure: Athol Wells, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; Roche Holding AG
Served as a speaker or a member of a speakers bureau for: Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; Roche Holding AG

Christopher R. Denton, PhD, FRCP

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

Disclosures

Disclosure: Christopher R. 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; Inventiva Pharma; Roche Holdings AG; Sanofi Aventis
Received grants for clinical research from: CSL Behring; GlaxoSmithKline; Inventiva Pharma; Roche Holdings AG

Editor

Marinella Calle, PhD, CMPP

Scientific Director, WebMD Global, LLC

Disclosures

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

Content Reviewer

Nafeez Zawahir, MD

Associate CME Clinical Director

Disclosures

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

CPD

Identifying Patients With Progressive Fibrosing Interstitial Lung Diseases

Authors: Athol Wells, MD; Christopher P. Denton, PhD, FRCP
CPD Released: 11/19/2018
THIS ACTIVITY HAS EXPIRED FOR CREDIT
Valid for credit through: 11/19/2019, 11:59 PM EST

Start Activity

Target Audience and Goal Statement

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

The goal of this activity is to improve physician knowledge regarding the identification of patients with a progressive fibrosing phenotype and potential therapeutic options, including antifibrotic treatment, for patients with progressive fibrosing interstitial lung diseases (PF-ILDs).

Upon completion of this activity, participants will:

Have increased knowledge regarding the
- Characteristics of patients with a PF-ILD phenotype
Have greater competence related to
- Establishing differential diagnoses of diverse ILD profiles that are likely to show progressive fibrosis, based on patient case examples
Demonstrate greater confidence in their ability to
- Evaluate available information on antifibrotic therapy for patients with PF-ILDs

Faculty

Athol Wells, MD

Professor
Interstitial Lung Disease Unit
Royal Brompton and Harefield NHS Foundation Trust
London, United Kingdom

Disclosures

Disclosure: Athol Wells, MD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; Roche Holding AG
Served as a speaker or a member of a speakers bureau for: Bayer HealthCare; Boehringer Ingelheim Pharmaceuticals, Inc.; Roche Holding AG

Christopher R. Denton, PhD, FRCP

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

Disclosures

Disclosure: Christopher R. 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; Inventiva Pharma; Roche Holdings AG; Sanofi Aventis
Received grants for clinical research from: CSL Behring; GlaxoSmithKline; Inventiva Pharma; Roche Holdings AG

Editor

Marinella Calle, PhD, CMPP

Scientific Director, WebMD Global, LLC

Disclosures

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

Content Reviewer

Nafeez Zawahir, MD

Associate CME Clinical Director

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).

Contact WebMD Global

For questions regarding the content of this activity, contact the accredited provider for this CME/CE activity noted above. For technical assistance, contact [email protected]

Instructions for Participation and Credit

There are no fees for participating in or receiving credit for this online educational activity. For information about your eligibility to claim credit, please consult your professional licensing board.

This activity is designed to be completed within the time designated on the title page; physicians should claim only those credits that reflect the time actually spent participating in the activity. To successfully earn credit, participants must complete the activity online during the credit eligibility period that is noted on the title page.

Follow these steps to claim a credit certificate for completing this activity:

Read the information provided on the title page regarding the target audience, learning objectives, and author disclosures, read and study the activity content and then complete the post-test questions. If you earn a passing score on the post-test and we have determined based on your registration profile that you may be eligible to claim CPD credit for completing this activity, we will issue you a CPD credit certificate.
Once your CPD credit certificate has been issued, you may view and print the certificate from your CME/CE Tracker. CPD credits will be tallied in your CME/CE Tracker and archived for 6 years; at any point within this time period you can print out the tally as well as the certificates by accessing "Edit Your Profile" at the top of the Medscape Education homepage.

We encourage you to complete an Activity Evaluation to provide feedback for future programming.

You may now view or print the certificate from your CME/CE Tracker. You may print the certificate but you cannot alter it. Credits will be tallied in your CME/CE Tracker and archived for 6 years; at any point within this time period you can print out the tally as well as the certificates by accessing "Edit Your Profile" at the top of your Medscape homepage.

*The credit that you receive is based on your user profile.

From Medscape Education

CPD

Identifying Patients With Progressive Fibrosing Interstitial Lung Diseases

Authors: Athol Wells, MD; Christopher P. Denton, PhD, FRCPFaculty and Disclosures

THIS ACTIVITY HAS EXPIRED FOR CREDIT

CPD Released: 11/19/2018

Valid for credit through: 11/19/2019, 11:59 PM EST

processing....

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.

Page 1 of 4

CPD Information Download Slides

Print
Like Tweet

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. 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

Faculty

Athol Wells, MD

Christopher R. Denton, PhD, FRCP

Editor

Marinella Calle, PhD, CMPP

Content Reviewer

Nafeez Zawahir, MD

CPD

Identifying Patients With Progressive Fibrosing Interstitial Lung Diseases

Target Audience and Goal Statement

Faculty

Athol Wells, MD

Christopher R. Denton, PhD, FRCP

Editor

Marinella Calle, PhD, CMPP

Content Reviewer

Nafeez Zawahir, MD

Accreditation Statements

For Physicians

Instructions for Participation and Credit

Identifying Patients With Progressive Fibrosing Interstitial Lung Diseases

Educational Impact Challenge