{
  "abstract": "Introduction Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc), occurring in 2.4-10% of patients, predominantly those with the diffuse cutaneous subtype. It is characterized by the abrupt onset of acute renal failure and malignant hypertension, historically associated with high mortality. Key risk factors include rapidly progressive diffuse skin involvement and the presence of RNA polymerase III antibodies. Although the introduction of ACE inhibitors has significantly improved patient outcomes, the role of immunosuppressive therapies in the management of SRC remains poorly understood.The aims of this study were to characterize the clinical profiles of scleroderma renal crisis (SRC) patients, evaluate the use of immunosuppressive therapies, and assess the association between these treatments and patient outcomes.Material and Methods We conducted a retrospective review of a prospectively maintained database containing data from 460 SSC patients. The analysis involved descriptive statistical methods.Results From 2003 to 2025, 22 patients were diagnosed with scleroderma renal crisis (SRC) and treated with ACE inhibitors. Of these, 20 had at least 24 months of follow-up and were divided into three outcome groups.Group A (n=6): Patients with a rapidly progressive course who died within four months. All had diffuse SSc and severe organ involvement. Five received intensive immunosuppression but did not improve.Group B (n=4): Patients who survived the initial episode but died within four years, mainly from sepsis or sudden cardiac death. Two patients with limited SSc required permanent dialysis and did not receive immunosuppression. Two patients with diffuse SSc treated with rituximab and/or mycophenolate mofetil had mixed renal outcomes.Group C (n=10): Patients who achieved long-term survival with renal function recovery. Most received continuous immunosuppressive therapy. Three patients with poor prognostic factors treated with rituximab had a surprisingly good outcome.Interestingly, three patients developed SRC more than six years after their SSc diagnosis, with two cases occurring after stopping immunosuppressive therapy.Conclusions Clinical vigilance for scleroderma renal crisis (SRC) should be maintained for more than five years after SSc diagnosis, as approximately 15% of cases may occur later, especially in patients with comorbidities. The use of immunosuppressive therapy appears to be associated with improved survival and better clinical outcomes in SRC.Abstract P.409 Table 1",
  "authors": [
    {
      "affiliations": [
        "Rambam Health Care Campus, Rheumatology Institute, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technolo, Haifa, Israel"
      ],
      "name": "Katya Dolnikov"
    },
    {
      "affiliations": [
        "Rambam Health Care Campus, Rheumatology Institute, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technolo, Haifa, Israel"
      ],
      "name": "Kochava Toledano"
    },
    {
      "affiliations": [
        "Beilinson Hospital, Liver Institute, Petach Tikva, Israel"
      ],
      "name": "Marius Braun"
    },
    {
      "affiliations": [
        "Rambam Health Care Campus, Rheumatology Institute, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technolo, Haifa, Israel"
      ],
      "name": "Sami Giryes"
    },
    {
      "affiliations": [
        "Rambam Health Care Campus, Rheumatology Institute, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technolo, Haifa, Israel"
      ],
      "name": "Yonit Tavor"
    },
    {
      "affiliations": [
        "Rambam Health Care Campus, Rheumatology Institute, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technolo, Haifa, Israel"
      ],
      "name": "Alexandra Balbir-Gurman"
    },
    {
      "affiliations": [
        "Rambam Health Care Campus, Rheumatology Institute, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technolo, Haifa, Israel"
      ],
      "name": "Yolanda Braun-Moscovici."
    }
  ],
  "title": "P.409 Phenotypic variations and clinical pathways in scleroderma renal crisis",
  "uid": "c7401f59-c753-56dc-8614-6259da5005ea"
}