{
  "abstract": "Introduction There are only a few bDMARDs with evidence of efficacy for the treatment of patients with systemic sclerosis (SSc) with progressive disease non-responsive to csDMARDs. The aim of this study was to assess the efficacy of rituximab in SSc patients with progressive skin involvement and/or polyarthritis and/or interstitial lung disease (ILD) non-responsive to methotrexate or cyclophosphamide or mycophenolate.Material and Methods 40 patients, who fulfilled the 2013 ACR/EULAR classification criteria (7 males / 33 females, 8 lcSSc / 32 dcSSc, mean±SD age 47.3±13.1 years, disease duration 6.5±4.6 years, modified Rodnan skin score (mRSS) 16±12, disease activity (ESSG, European Scleroderma Study Group) 3.9±2.0 with progressive skin thickening and/or arthritis and/or ILD non-responsive to methotrexate/myocphenolate/cyclophosphamide were treated with 1 (n=40), 2 (n=36), 4 (n=25) or 6 (n=13) series of rituximab (1g i.v. at day 0 and 14, every 6 months). Before each series, patients were assessed by a rheumatologist for mRSS, finger-to-palm (FTP) distance, inter-labial/-incisal distance, and disease activity (Visual Analog Scale (VASph); ESSG), and filled out questionnaires assessing global function (Scleroderma Health Assessment Questionnaire, SHAQ), quality of life (Medical outcomes study Short Form 36, SF-36), fatigue (Fatigue Impact Scale, FIS), depression (Beck’s Depression Inventory-II, BDI-II), physical activity (Human Activity Profile, HAP), gastrointestinal (UCLA-SCTC-GIT 2.0) and pulmonary (St. George´s Respiratory Questionnaire, SGRQ) symptoms. Furthermore, peripheral blood was analyzed for routine laboratory parameters (e.g., CRP, ESR, C3 and C4 complement levels), and stored for biobanking.Results Over 36 months of rituximab therapy, we observed a significant decrease in skin thickening (mRSS) and disease activity (ESSG, VASph). Lung function remained stable over time, with no significant decline in FVC at any follow-up point. Regarding SGRQ, the activity domain showed dramatic, significant, and sustained improvement, while, unexpectedly, the impacts domain worsened. Furthermore, the SGRQ symptoms domain and the total score decreased (improved) numerically without reaching statistical significance. Physical disability (HAQ) decreased significantly. Among the SHAQ domains, pain, finger ulcer burden, and the global score also improved significantly during follow-up.Regarding the laboratory parameters, no significant changes were observed in C3, C4, and ESR over the course of follow-up. No significant changes were seen in fatigue (FIS), depressive symptoms (BDI-II), gastrointestinal symptoms (UCLA-SCTC-GIT 2.0), physical activity (HAP), and quality of life (SF-36).Conclusions These findings suggest that long-term use of rituximab provides sustained clinical benefit in patients with progressive SSc unresponsive to csDMARDs, particularly improvement of skin involvement, disease activity, and disability, while stabilizing lung volumes and patients‘ reported respiratory burden.Abstract P.277 Table 1",
  "authors": [
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Oreská Sabína"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Daniel Zavada"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Aneta Jirankova"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Anna Bobrova"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Katerrina Bobrova"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Eliska Sudkova"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Emma Polouckovs"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Barbora Hermankova"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Prague, Czech Republic"
      ],
      "name": "Hana Wünch"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Karel Pavelka"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Ladislav Senolt"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Jiri Vencovsky"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Radim Becvar"
    },
    {
      "affiliations": [
        "Institute of Rheumatology, Department of Rheumatology, st Faculty of Medicine, Charles University, Prague, Czech Republic"
      ],
      "name": "Michal Tomcík"
    }
  ],
  "title": "P.277 Rituximab in patients with systemic sclerosis non-responsive to csDMARDs – preliminary results of a prospective observational monocentric study over 36 months",
  "uid": "d4f0b98c-f31b-51b1-8bc0-236a9ebcff90"
}