{
  "abstract": "Introduction Systemic sclerosis (SSc) is a chronic multisystem autoimmune disease. Approximately half of patients with SSc are affected by interstitial lung disease (ILD), the leading cause of SSc-related mortality. Given the evolving therapeutic landscape, we conducted a systematic literature review (SLR) to gather evidence on the efficacy and safety of currently approved and pipeline therapies for the treatment of SSc and SSc-ILD.Material and Methods This slr included randomised controlled trials (rcts), single-arm trials and non-randomised comparative trials published in english with no date restriction ( table 1). Relevant studies were identified through Embase, MEDLINE and CENTRAL databases. Conference proceedings (from 2010) from the American College of Rheumatology (ACR) annual meeting, the European Alliance of Associations for Rheumatology congress and the World Scleroderma Congress, as well as the United States National Institutes of Health Clinical Trial Registry (from 2018) were searched. Additional manual searches of bibliographies from peer-reviewed SLRs/meta-analyses (from 2021) were also conducted. Screening was performed by two reviewers working in parallel, while data extraction was performed by one reviewer and validated by a second. Study risk of bias was assessed using the Cochrane Collaboration’s Risk of Bias 2 tool for RCTs and the Risk of Bias in Non-Randomized Studies of Interventions tool for non-RCTs and single-arm trials.Results Literature searches were conducted on 1 April 2025. Key findings for forced vital capacity (FVC), modified Rodnan skin score (mRSS), percent predicted diffusion capacity of the lungs for carbon monoxide (ppDLCO), survival and safety will be presented. Considerable variability was observed across the included trials in study designs and outcomes. Despite the availability of currently approved therapies, patients with SSc/SSc-ILD continue to experience some clinical burden.Conclusions Our SLR revealed significant heterogeneity in study designs, populations, outcome definitions, and findings, limiting the comparability of included trials. The high clinical burden underscores the need for well-designed and placebo-controlled trials of more effective and well-tolerated therapies. The ongoing Phase 2/3 BLISSc-ILD study ( NCT05878717) is evaluating the efficacy and safety of belimumab in patients with SSc-ILD; it will be important to assess the results from this trial in the context of the clinical evidence base.Funding GSK (GSK Study 219961).Abstract P.113 Table 1Study eligibility criteria for the SLR",
  "authors": [
    {
      "affiliations": [
        "Precision AQ, Boston, MA, USA"
      ],
      "name": "Emily Glowienka"
    },
    {
      "affiliations": [
        "Precision AQ, Boston, MA, USA"
      ],
      "name": "Ian Steinfield"
    },
    {
      "affiliations": [
        "Precision AQ, Boston, MA, USA"
      ],
      "name": "Kevin Coady"
    },
    {
      "affiliations": [
        "Precision AQ, Vancouver, BC, Canada"
      ],
      "name": "Carole Lunny"
    },
    {
      "affiliations": [
        "Precision AQ, Vancouver, BC, Canada"
      ],
      "name": "Florence R Wilson"
    },
    {
      "affiliations": [
        "Precision AQ, Vancouver, BC, Canada"
      ],
      "name": "Alicia N Pepper"
    },
    {
      "affiliations": [
        "Clinical Sciences - Research & Development, GSK, London, United Kingdom"
      ],
      "name": "Svetlana Nihtyanova"
    },
    {
      "affiliations": [
        "Rheumatology - Global Medical Affairs, GSK, Collegeville, PA, USA"
      ],
      "name": "Roger A Levy"
    },
    {
      "affiliations": [
        "Clinical Research - Respiratory, Immunology and Inflammation Research Unit, GSK, Stevenage, United Kingdom"
      ],
      "name": "Elaine Irving"
    },
    {
      "affiliations": [
        "Global Real-World Evidence & Health Outcomes Research, GSK, Toronto, ON, Canada"
      ],
      "name": "Daniel Moldaver"
    },
    {
      "affiliations": [
        "Department of Rheumatology - University Hospital Zurich, University of Zurich, Zurich, Switzerland"
      ],
      "name": "Cosimo Bruni"
    }
  ],
  "title": "P.113 Heterogeneity in treatment outcomes for systemic sclerosis with or without interstitial lung disease: a systematic literature review",
  "uid": "457a799f-91d7-5ce9-9522-6a7a60dccfc9"
}