{
  "abstract": "Introduction The early trajectory of skin fibrosis provides insights into the disease course of patients with systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. We have previously developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis in SSc. In our previous study (from the EUSTAR database), late skin fibrosis was not uncommon, affecting approximately one-fifth of patients. 1 Our current aim was to further externally validate (and refine) our conceptual models of late skin fibrosis in patients with SSc.Material and Methods An analysis of patients enrolled in the comprehensive Johns Hopkins Scleroderma Center Research Registry.We defined three conceptual models of late skin fibrosis (figure 1), based upon our previous EUSTAR analysis.1Had worsening during first 5 years and then improved, and later worsened from the improvement.Worsening for the first time after 5 years.Worsening in the first 5 years and stayed high after 5 years (i.e. failure to improve with worsening within 5-year window).Based upon the known minimal clinically important difference, we defined ‘worsening’ and ‘improvement’ of mRSS as an increase or decrease 5 units, respectively. Descriptive statistics were used to understand the data.Results We included 573 patients from the Johns Hopkins cohort. Most were female (83.4%); white (75.5%) or black/African American (16.9%) race. Number of patients with limited and diffuse cutaneous SSc were 248 and 265 at baseline. Mean (SD) duration from first non-Raynaud’s phenomenon to baseline mRSS was 18.3 (12.1) months. Most common medication exposure (ever) were MMF (58.5%), prednisone (44.5%), HCQ (34.7%), MTX (29.5%), IVIg (13.6%), and cyclophosphamide (10.3%). One-fifth of patients among the whole cohort (n=127/573, 22.2%) experienced late mRSS worsening. Scenario A (worsening after initial improvement) was most commonly observed (n=36, 28.3%); and similar proportions with Scenario B (n=23, 18.1%) and Scenario C (n=16, 12.6%). Anti-RNA polymerase was overrepresented in Scenario A (41.4%) compared to Scenario B or C (15.0% and 7.7%, respectively). Around 40% (n=52) had late skin worsening, but did not fit any of our conceptual models.Conclusions We further validated our conceptual models of late skin fibrosis, with a prevalence of these in ~13% of the total SSc patients. Our findings differ from our initial study where Scenario A was rare, likely due to differences between patient populations, especially the higher prevalence of anti-RNA polymerase patients in our cohort.Reference Hughes, et al. Rheumatology 2023. PMID 35731139.Abstract OC.36 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, The University of Manchester, Manchester, United Kingdom"
      ],
      "name": "Michael Hughes"
    },
    {
      "affiliations": [
        "Department of Biostatistics, School of Public Health, University of Michigan, Michigan, USA"
      ],
      "name": "Suiyuan Huang"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, USA"
      ],
      "name": "Rachel Wallwork"
    },
    {
      "affiliations": [
        "Centre for Rheumatology, Division of Medicine, University College London, London, UK"
      ],
      "name": "Medha Kanitkar"
    },
    {
      "affiliations": [
        "Centre for Rheumatology, Division of Medicine, University College London, London, UK"
      ],
      "name": "Christopher Denton"
    },
    {
      "affiliations": [
        "University of Pittsburgh School of Medicine, Pittsburgh, USA"
      ],
      "name": "Robyn Domsic"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, USA"
      ],
      "name": "Ami Shah"
    },
    {
      "affiliations": [
        "Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, USA"
      ],
      "name": "Laura Hummers"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, Michigan, USA"
      ],
      "name": "Dinesh Khanna"
    }
  ],
  "title": "OC.36 External validation and exploration of conceptual models of late skin fibrosis in patients with systemic sclerosis",
  "uid": "e7558cec-bded-5ebc-88a6-5285a927ccbd"
}