{
  "abstract": "Introduction Autologous hematopoietic stem cell transplantation (AHSCT) improves survival and prevents progression in diffuse cutaneous systemic sclerosis (dcSSc) patients. A complication of AHSCT is cytokine release syndrome (CRS), which is a systemic inflammatory response triggered by the release of proinflammatory cytokines, ranging from moderate symptoms to fatal organ failure. Little is known regarding frequency, clinical course, or risk factors of CRS in SSc in the context of AHSCT. This study aims to address this knowledge gap.Material and Methods Data from SSc patients who underwent AHSCT according to the ASTIS protocol between September 2011 and November 2024 at two Dutch university hospitals were analysed retrospectively. CRS was graded according to the American Society for Transplantation and Cellular Therapy. Univariate logistic regression analysis was conducted to assess associations between CRS severity (grade 0-1 vs >1) and clinical characteristics.Results In total, N=71 patients were included, 40.8% (N=29) was female, mean age was 47.6±9.6 years, and the median disease duration was 13 months (IQR 7-23). 87.1% had dcSSc and 12.9% had limited cutaneous SSc. At baseline the mean percentage of predicted diffusing capacity of the lungs for carbon monoxide (DLCO) was 63.3%±18.3%, of forced vital capacity (FVC) was 83.5%±16.3% and clinically relevant interstitial lung disease (FVC<85% and/or DLCO<85% and positive HRCT findings) was present in 38 patients (53,5%), and cardiac involvement was present in 27.1% (N=19).Clinical response following AHSCT was observed in 92.6% (N= 63). The median length of hospital stay was 18 days (IQR16-21). CRS occurred in 63(88.7%) patients; of those N=32(50.8%) classified as grade 1, N=22(34.9%) as grade 2, N=4(6.3%) as grade 3, N=2(3.2%) as grade 4, and N=3(4.8%) cases had a fatal outcome, grade 5 (figure 1). In 12.7% admission to ICU within 1 week after ATG administration was reported. All CRS patients were treated with glucocorticoids, and one patient was treated with tocilizumab and eculizumab. A DLCO <60% of predicted was significantly associated with a higher CRS grade (OR 2.940, 95%CI:1.113-7.771). Age was not significantly associated with a CRS grade (OR 1.004, 95%CI:0.956-1.055).Conclusions CRS is common in patients undergoing AHSCT for SSc, but severe CRS grades and fatal outcomes are rare. CRS was found to be associated with a low DLCO prior to AHSCT.Abstract P.253 Figure 1CRS occurrence and severity in SSc patients (N=71) treated with AHSCT. Values shown as numbers and percentages",
  "authors": [
    {
      "affiliations": [
        "University Medical Center Utrecht, Department of Rheumatology and Clinical Immunology, Utrecht, The Netherlands"
      ],
      "name": "Charlotte Schimmel"
    },
    {
      "affiliations": [
        "Leiden University Medical Center, Department of Rheumatology, Leiden, The Netherlands"
      ],
      "name": "Eva Hoekstra"
    },
    {
      "affiliations": [
        "University Medical Center Utrecht, Department of Rheumatology and Clinical Immunology, Utrecht, The Netherlands"
      ],
      "name": "Maaike Koops"
    },
    {
      "affiliations": [
        "Alma Mater Studiorum University of Bologna, Department of Biomedical and Neuromotor Sciences, Bologna, Italy"
      ],
      "name": "Jacopo Ciaffi"
    },
    {
      "affiliations": [
        "University Medical Center Utrecht, Utrecht, Department of Hematology, Utrecht, The Netherlands"
      ],
      "name": "Anna van Rhenen"
    },
    {
      "affiliations": [
        "Leiden University Medical Center, Department of Hematology, Leiden, The Netherlands"
      ],
      "name": "Peter von dem Borne"
    },
    {
      "affiliations": [
        "Leiden University Medical Center, Department of Rheumatology, Leiden, The Netherlands"
      ],
      "name": "Tom Huizinga"
    },
    {
      "affiliations": [
        "University Medical Center Utrecht, Department of Rheumatology and Clinical Immunology, Utrecht, The Netherlands"
      ],
      "name": "Jacob van Laar"
    },
    {
      "affiliations": [
        "Leiden University Medical Center, Department of Rheumatology, Leiden, The Netherlands"
      ],
      "name": "Jeska de Vries-Bouwstra"
    },
    {
      "affiliations": [
        "University Medical Center Utrecht, Department of Rheumatology and Clinical Immunology, Utrecht, The Netherlands"
      ],
      "name": "Julia Spierings"
    }
  ],
  "title": "P.253 Unveiling the storm: prevalence and risk factors for cytokine release syndrome in systemic sclerosis patients undergoing autologous hematopoietic stem cell transplantation",
  "uid": "f100d40a-dcc0-5468-acbd-82a78470ed5b"
}