TY - JOUR T1 - Assessment of recent evidence for the management of patients with systemic sclerosis-associated interstitial lung disease: a systematic review JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00235-2020 VL - 7 IS - 1 SP - 00235-2020 AU - Anna-Maria Hoffmann-Vold AU - Toby M. Maher AU - Edward E. Philpot AU - Ali Ashrafzadeh AU - Oliver Distler Y1 - 2021/01/01 UR - http://openres.ersjournals.com/content/7/1/00235-2020.abstract N2 - 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 to 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 sex 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.This systematic literature review on systemic sclerosis-associated interstitial lung disease summarises the evidence supporting approaches to disease management in clinical practice https://bit.ly/2ISdSNc ER -