{
  "abstract": "Introduction Currently, no validated biomarkers exist for systemic sclerosis (SSc). The modified Rodnan skin score (mRSS) is widely used, but may not fully capture disease course. Severe complications can occur even in patients with low scores. Cutaneous histology offers a promising candidate tissue-based marker, capturing fibrosis as well as inflammatory and vascular processes central to SSc pathophysiology. Additionally, CEMIP (cell migration-inducing protein), which is a hyaluronidase family protein with pro-inflammatory and pro-fibrotic properties in other diseases, may be a significant biomarker and mediator in the mechanisms of SSc. This study aimed to evaluate histological parameters as potential biomarkers of disease severity and to investigate CEMIP expression in SSc skin biopsies.Material and Methods Skin biopsies were obtained from the dorsal forearm (regardless of whether the skin was visibly affected at that site) of 36 patients with SSc (25 limited cutaneous forms, 11 diffuse forms) and 15 healthy controls matched for age and sex. Hematoxylin-eosin (HE), Masson’s trichrome (MT), and immunostaining for CD45, αSMA, and CEMIP were performed on FFPE tissues. The histological criteria and the corresponding methods of analysis are summarized in table 1. Quantification was conducted with QuPath 5.0.0, and semi-quantitative scoring was independently assessed by blinded evaluators.Results Classical histological evaluation revealed significant abnormalities in SSc compared with controls. Among the criteria analyzed, loss of epidermal papillae, intimal proliferation, hyalinized collagen, pigment incontinence and myofibroblast score were significantly altered in SSc skin. Importantly, microscopic alterations were already present in limited cutaneous SSc patients without clinical thickening (local mRSS = 0). Detailed results are provided in table 2. In addition, quantitative analysis demonstrated a marked upregulation of CEMIP expression in SSc skin (27.25%) compared with controls (5.03%; p < 0.01). Increased expression was observed in both the epidermis (p < 0.01) and dermis (p < 0.01), with particularly strong signals in endothelial cells and fibroblasts (p < 0.01).Conclusions Histological evaluation of skin biopsies is a promising complementary tool for the clinical assessment of SSc. It allows detection of microscopic abnormalities even when macroscopic involvement is absent. Incorporating quantitative analysis of CEMIP expression alongside classical histological criteria could improve the accuracy of disease severity scoring. Ongoing analyses aim to develop a histological score reflecting disease severity, including internal organ involvement. The marked increase in CEMIP expression in SSc-affected skin suggests a potential role in disease pathogenesis. Further mechanistic studies are needed to clarify its contribution to SSc progression.Abstract P.210 Table 1Analysis methods of histological parametersAbstract P.210 Table 2Quantitative and semi-quantitative analyses of histological parameters",
  "authors": [
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Pauline Salpetier"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Zoe Gendebien"
    },
    {
      "affiliations": [
        "Liège University Hospital, Department of Pathology, Liège, Belgium"
      ],
      "name": "Joan Somja"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Genevieve Paulissen"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Beatrice Andre"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Zelda Plener"
    },
    {
      "affiliations": [
        "Bordeaux University Hospital, Rheumatology Department, ImmunoConcept Laboratory, Bordeaux, France"
      ],
      "name": "Marie-Elise Truchetet"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Dominique De Seny"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Clio Ribbens"
    },
    {
      "affiliations": [
        "Liège University Hospital, Laboratory of Rheumatology, GIGA Research, Liège, Belgium"
      ],
      "name": "Celine Deroyer"
    }
  ],
  "title": "P.210 Skin tissue parameters and CEMIP: promising candidate biomarkers in systemic sclerosis",
  "uid": "8e359cc9-56e2-59c9-a5b1-7d8d8023b052"
}