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Using data from longitudinal clinical trials and observational studies, we assessed definitions and patterns of progression, risk factors for progression, and implications for treatment.<\/jats:p><jats:p>SSc-ILD progression was commonly defined as exceeding specific thresholds of lung function worsening and\/or increasing radiographic involvement. One definition used in several studies is decline in forced vital capacity (FVC) of \u226510%, or \u22655\u201310% plus a decline in diffusing capacity of the lung for carbon monoxide \u226515%. Based on these criteria, 20\u201330% of patients in observational cohorts develop progressive ILD, starting early in the disease course and progressing at a highly variable rate.<\/jats:p><jats:p>Risk factors such as age, FVC, extent of fibrosis and presence of anti-topoisomerase I antibodies can help predict progression of SSc-ILD, though composite risk scores may offer greater predictive power. Whilst the variability of the disease course in SSc-ILD makes risk stratification of patients challenging, the decision to initiate, change or stop treatment should be based on a combination of the current disease state and the speed of progression.<\/jats:p>","DOI":"10.1183\/16000617.0340-2020","type":"journal-article","created":{"date-parts":[[2021,3,24]],"date-time":"2021-03-24T15:50:37Z","timestamp":1616601037000},"page":"200340","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":39,"title":["Natural variability in the disease course of SSc-ILD: implications for treatment"],"prefix":"10.1183","volume":"30","author":[{"given":"Madelon C.","family":"Vonk","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich A.","family":"Walker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth 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