Abstract
This systematic review summarises current evidence to help guide treatment decisions for patients with systemic sclerosis (SSc) associated interstitial lung disease (ILD). A systematic search of the literature (January 2012−April 2018), including grey literature (searched between 1992 and 2011), was conducted using multiple electronic databases. Guidelines, meta-analyses, randomised controlled trials and observational studies reporting on risk stratification, screening, diagnosis, treatment, and management outcomes for patients with SSc-ILD were included. A quality assessment of the included evidence was undertaken.
In total, 2464 publications were identified and 280 included. Multiple independent risk factors for ILD in patients with SSc were identified, including older age, male gender and baseline pulmonary function. High-resolution computed tomography (HRCT) has been used for characterising ILD in patients with SSc, and pulmonary function tests are a key adjunctive component in the diagnostic and monitoring pathway. The clinical value of biomarkers relating to SSc-ILD diagnosis or assessment for disease progression is unknown at present. Immunosuppressive therapy (monotherapy or combined therapy) is the current standard of care for SSc-ILD; long-term evidence for effective and safe treatment of SSc-ILD is limited.
Identification of patients at risk for SSc-ILD remains challenging. HRCT and pulmonary function tests are key to diagnosing and monitoring for disease progression. Although immunosuppressive therapy is considered current first-line treatment, it is partly associated with adverse effects and long-term follow-up evidence is limited. Novel therapies and biomarkers should be further explored in well-controlled clinical studies.
Footnotes
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Conflict of interest: Dr. Hoffmann-Vold had a consultancy relationship and/or has received research funding from Actelion, Bayer, Boehringer Ingelheim, GlaxoSmithKline and Roche.
Conflict of interest: TMM has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB and has received consultancy or speakers fees from Apellis, Astra Zeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R&D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB.
Conflict of interest: Dr. Philpot has nothing to disclose.
Conflict of interest: Dr. Ashrafzadeh is an employee of IQVIA, which has been supported by Boehringer Ingelheim for this study.
Conflict of interest: Professor Distler reports grants and personal fees from Actelion, personal fees from Abbvie, personal fees from Acceleron Pharma, personal fees from Anamar, personal fees from Amgen, grants and personal fees from Bayer, grants and personal fees from Boehringer Ingelheim, personal fees from Catenion, personal fees from CSL Behring, personal fees from Ergonex, personal fees from GSK, personal fees from Inventiva, personal fees from Italfarmaco, personal fees from iQvia, personal fees from Medac, personal fees from Medscape, grants and personal fees from Mitsubishi, personal fees from MSD, personal fees from Lilly, personal fees from Novartis, personal fees and non-financial support from Pfizer, personal fees from Roche, personal fees from Target BioScience, personal fees from UCB, personal fees from Baecon Discovery, personal fees from Blade Therapeutics, personal fees from Curzion Pharmaceuticals, personal fees from Glenmark Pharmaceuticals, personal fees from Competitive Drug Development International Ltd, personal fees from Galapagos NV, outside the submitted work; In addition, Dr. Distler has a patent US8247389, EP2331143 issued.
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- Received May 1, 2020.
- Accepted December 9, 2020.
- Copyright ©ERS 2021
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