{
  "abstract": "Introduction Biologic drugs use is growing with special or compassionate permissions. The main indication for biologics is rapidly progressive systemic sclerosis (SSc) and SSc associated interstitial lung disease (SSc-ILD). We aimed to analyze long term outcome of patients with SSc-ILD treated with tocilizumab (TCZ), rituximab (RTX), and abatacept (ABA) which became available in the last decades.Material and Methods Retrospective data on patients with SSc-ILD followed at our center from 1995 to 2025 years was extracted. Variables including disease duration, death, survival, clinical features, and changes in pulmonary function (FVC and DLCO) were analyzed and compared between patients treated with biologics (BD) and those with conventional drugs (CD). Student’s T-test, Chi-squared test and Kaplan-Mayer survival curve were used for statystic analysis (p<0.05 significant).Results 106 patients, 86 females, mean age 60.1+/-13.7 years were included in the study; 35 patients received BD after failure of CD (7 received more than one drug); two patients underwent lung transplantation (1-successful). The mean disease duration was 14.2 +/-8.9 years, 56.1% had diffuse SSc, 67.3% had antibodies to topoisomerase; 44 (41.5%) patients died during follow-up. There were no significant differences between bd and cd groups in age at ssc onset, disease duration, antibodies, malignancies, digital ulcers, pulmonary hypertension, heart, kidney and git involvement, use of cyclophosphamide, methotrexate and azathioprine, survival and death ( figure 1). BD patients had more diffuse SSc (74.3% vs. 56.1%, p=0,056), arthritis (88.6% vs. 58.8%, p<0.001), myositis (48.6% vs. 23.6%, p<0.005); more patients were treated with mycophenolate mofetil (80% vs. 59.4%, p<0.028). During follow-up BD patients had significant reduction in FVC (79.6% vs. 63.5%, p<0.001) and DLCO (62.2% vs. 45.2%, p<0.0005).Conclusions More severe and complicated patients were treated with BD; all of them received BD after failure of at least one CD. The initiation of BD was mainly driven by SSc-ILD, rapidly progressive skin fibrosis, arthritis and myositis. We could not demonstrate stabilization in pulmonary function, improved survival and reduced mortality in patients treated with BD. Our results support the urgent need in biomarkers for appropriate patient’s stratification. As delay in in treatment is crucial, research on the early up-front treatment combination in SSc patients’ care is needed.Abstract P.124 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Balbir-Gurman Alexandra"
    },
    {
      "affiliations": [
        "Pulmonary Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Yaniv Dotan"
    },
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Katya Dolnikov"
    },
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Yonit Tavor"
    },
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Sami Giryes"
    },
    {
      "affiliations": [
        "Medical Imaging Division, Pulmonary Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Luda Guralnik"
    },
    {
      "affiliations": [
        "Pulmonary Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Ana Solomonov"
    },
    {
      "affiliations": [
        "Medical Imaging Division, Pulmonary Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Jasmin Atalla"
    },
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine-Technion, Haifa, Israel"
      ],
      "name": "Yolanda Braun-Moscovici"
    }
  ],
  "title": "P.124 Characteristics of scleroderma patients with lung involvement treated with biologic drugs, a single center experience",
  "uid": "09cae1ef-977c-5524-9565-23d4fce9601b"
}