{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T09:58:12Z","timestamp":1793354292772,"version":"4.5.0"},"reference-count":323,"publisher":"BMJ","issue":"4","license":[{"start":{"date-parts":[[2023,11,8]],"date-time":"2023-11-08T00:00:00Z","timestamp":1699401600000},"content-version":"unspecified","delay-in-days":7,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Scleroderma Clinical Trial Consortium","id":[{"id":"https:\/\/ror.org\/05j12ct50","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/100001845","name":"Scleroderma Research Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100001845","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2023,10,2]]},"published-print":{"date-parts":[[2023,11]]},"abstract":"\n Objective<\/jats:bold>\n The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is highly variable and different from continuously progressive idiopathic pulmonary fibrosis (IPF). Most proposed definitions of progressive pulmonary fibrosis or SSc-ILD severity are based on the research data from patients with IPF and are not validated for patients with SSc-ILD. Our study aimed to gather the current evidence for severity, progression and outcomes of SSc-ILD.\n <\/jats:p>\n \n Methods<\/jats:bold>\n A systematic literature review to search for definitions of severity, progression and outcomes recorded for SSc-ILD was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines in Medline, Embase, Web of Science and Cochrane Library up to 1 August 2023.\n <\/jats:p>\n \n Results<\/jats:bold>\n A total of 9054 papers were reviewed and 342 were finally included. The most frequent tools used for the definition of SSc-ILD progression and severity were combined changes of carbon monoxide diffusing capacity (DLCO) and forced vital capacity (FVC), isolated FVC or DLCO changes, high-resolution CT (HRCT) extension and composite algorithms including pulmonary function test, clinical signs and HRCT data. Mortality was the most frequently reported long-term event, both from all causes or ILD related.\n <\/jats:p>\n \n Conclusions<\/jats:bold>\n The studies presenting definitions of SSc-ILD \u2018progression\u2019, \u2018severity\u2019 and \u2018outcome\u2019 show a large heterogeneity. These results emphasise the need for developing a standardised, consensus definition of severe SSc-ILD, to link a disease specific definition of progression as a surrogate outcome for clinical trials and clinical practice.\n <\/jats:p>\n PROSPERO registration number CRD42022379254.Cite Now<\/jats:p>","DOI":"10.1136\/rmdopen-2023-003426","type":"journal-article","created":{"date-parts":[[2023,11,8]],"date-time":"2023-11-08T11:52:45Z","timestamp":1699444365000},"page":"e003426","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":25,"title":["Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review"],"prefix":"10.1136","volume":"9","author":[{"given":"Liubov","family":"Petelytska","sequence":"first","affiliation":[{"name":"Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland"},{"name":"Department 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