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  1. Anna-Maria Hoffmann-Vold1,
  2. Toby M Maher2,
  3. Edward E Philpot3,
  4. Ali Ashrafzadeh4,
  5. Oliver Distler5
  1. 1Oslo University Hospital, Department of Rheumatology, Oslo, Norway
  2. 2National Heart and Lung Institute, Imperial College London and Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom
  3. 3Respiratory Center of Excellence, IQVIA, Durham, NC, United States of America
  4. 4Rheumatology Center of Excellence, IQVIA, San Diego, CA, United States of America
  5. 5University Hospital Zurich, Department of Rheumatology, Zurich, Switzerland


Background Interstitial lung disease in systemic sclerosis (SSc-ILD) occurs frequently and carries a high burden of morbidity and mortality. To date, there are no existing guidelines for screening, diagnosis and management of SSc-ILD that would aid early recognition and treatment and improve the care of these patients.

Objectives To develop expert consensus recommendations for the identification and management of SSc-ILD.

Methods Based on the results of a comprehensive systematic literature analysis conducted in line with NICE/CRD and IQWiG guidelines and PRISMA methodology, evidence-based statements on SSc-ILD risk, screening, diagnosis, treatment and follow-up were developed. A modified Delphi process was then used to establish consensus statements for the identification and management of SSc-ILD. Briefly, an expert panel of 27 European-based pulmonologists, rheumatologists and internists with experience in treating SSc-ILD was established. Between July and November 2018, the panel took part in 3 rounds of online surveys, a face-to-face discussion and a WebEx meeting to establish consensus-based recommendations for the management of SSc-ILD. Statements were categorised by topic: risk factors (including biomarkers); screening; diagnosis; assessment of severity; treatment initiation; treatment options; disease progression; treatment escalation; other management options. Panellists indicated their level of agreement with proposed statements on a scale of 1 (strong disagreement) to 7 (strong agreement), and consensus was considered achieved when ≥80% either disagreed (score of 1–3) or agreed (score of 5–7) with a statement. Based on panel feedback, statements that did not reach consensus were modified and re-voted in later rounds.

Results At the close of the Delphi process, the panel agreed on the following:

  1. Risk factors: The presence of anti–topoisomerase I antibodies, male gender and diffuse cutaneous SSc all increase risk for ILD

  2. Screening: All SSc patients should undergo screening for ILD, using HRCT and lung function testing. Frequency of screening using HRCT should be guided by risk of developing ILD, in combination with clinical symptoms and lung function

  3. Diagnosis and assessment of severity: Use of HRCT to diagnose SSc–ILD and assess severity, with supporting findings from lung function testing and clinical assessment, is also recommended

  4. Treatment initiation and options: All patients with severe or progressive SSc–ILD should be considered for pharmacotherapy, with mycophenolate mofetil and cyclophosphamide recommended as treatments. Patients not receiving treatment should be followed closely for signs of disease progression

  5. Disease progression: Indicators of progression include sustained decline in lung function, worsening of clinical symptoms, and change in extent and/or pattern of fibrosis on HRCT

  6. Treatment escalation: Patients with inadequate treatment responses should be considered for treatment escalation. Suitability for lung transplant should be evaluated early, especially for patients diagnosed with advanced disease. Autologous haematopoietic stem cell transplant may be considered in carefully selected patients

Conclusion These evidence-based expert consensus recommendations, developed using a modified Delphi process, provide important guidance for the identification and management of SSc-ILD.

Acknowledgement Funding: Boehringer Ingelheim

Disclosure of Interests Anna-Maria Hoffmann-Vold Grant/research support from: Received research funding or other remuneration from Boehringer Ingelheim, GSK, and Actelion, Consultant for: Received consulting fees or other remuneration from Boehringer Ingelheim, GSK, and Actelion, Speakers bureau: Actelion and Boehringer Ingelheim, Toby M Maher Shareholder of: Has stock options or bond holdings in a for-profit corporation in Apellis, Grant/research support from: Received funds from BI advisory board participation and conference travel. Received research funding and/or consulting fees or other remuneration from GSK, UCB, AstraZeneca, Roche, Bayer, Biogen Idec, Cipla, Prometic, and Sanumed, Consultant for: Toby Maher has received consultancy or speakers fees from Apellis, AstraZeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Galapagos, GlaxoSmithKline R&D, Indalo, Pliant, ProMetic, Roche, Samumed, and UCB; and has received consultancy fees from Galecto, Edward E Philpot Employee of: Employee of IQVIA, Ali Ashrafzadeh Employee of: Employee of IQVIA, Oliver Distler Grant/research support from: Prof. Distler received research funding from Actelion, Bayer, Boehringer Ingelheim and Mitsubishi Tanabe to investigate potential treatments of scleroderma and its complications, Consultant for: Prof. Distler has/had consultancy relationship within the last 3 years with Actelion, AnaMar, Bayer, Boehringer Ingelheim, ChemomAb, espeRare foundation, Genentech/Roche, GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, MedImmune, Mitsubishi Tanabe Pharma, Pharmacyclics, Novartis, Pfizer, Sanofi, Serodapharm and UCB in the area of potential treatments of scleroderma and its complications. In addition, he had/has consultancy relationship within the last 3 years with A. Menarini, Amgen, Abbvie, GSK, Mepha, MSD, Pfizer and UCB in the field of arthritides and related disorders

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