{
  "abstract": "Introduction Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis and peripheral circulatory disturbances. Conditions involving multiple autoimmune diseases, including SSc, are defined as overlap syndrome (OS). In Japan, clinical features of SSc with overlap syndrome (OL SSc) have not been sufficiently investigated. This study aims to clarify the clinical characteristics of OL SSc through a retrospective analysis.Material and Methods We conducted a retrospective cohort study of 345 patients registered in the Tokyo Women’s Medical University SSc Database. The cohort included both diffuse and limited SSc subtypes (102 and 243 patients, respectively), with a female-to-male ratio of 10:1, mean age 68.6 ± 13 years, and mean disease duration of 18.8 ± 11.8 years. Patients were divided into two groups: SSc alone and OL SSc, and their clinical features were compared.Results OL SSc was identified in 131 patients, with the most common overlapping condition being Sjögren’s syndrome (SS; 89 cases), followed by rheumatoid arthritis (18), systemic lupus erythematosus (12), vasculitis (6), and polymyositis/dermatomyositis (5). SS was the most frequent overlap (67.9%). The OL SSc group had a significantly higher proportion of female patients than the SSc-alone group (p<0.05), particularly in SS cases (p<0.01). Patients with SS overlap were older on average than those in other groups. In terms of complications, the OL SSc group had significantly higher rates of pulmonary hypertension and upper gastrointestinal involvement (p<0.05), as well as arthritis and renal impairment (p<0.01). Among these, SSc patients with SS overlap showed a lower mortality rate and older age at death compared to other groups (p<0.05).Conclusions Approximately 40% of SSc patients had overlap syndrome, most commonly with SS. The OL SSc group included more elderly and female patients and exhibited higher frequencies of pulmonary hypertension, gastrointestinal lesions, arthritis, and renal complications. Notably, SSc patients with SS overlap had better prognoses and distinct clinical features. Although SS overlap in SSc has been considered rare, this study suggests it may represent a subgroup with more favorable outcomes. Thus, recognizing SS overlap may be essential for prognosis prediction and therapeutic decision-making in SSc management.",
  "authors": [
    {
      "affiliations": [
        "Adashi Medical Center, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Kae Takagi"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Department of Collagen Disease and Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Yuki Ichimura"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Department of Collagen Disease and Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Akiko Tochimoto"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Department of Collagen Disease and Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Tomoaki Higuchi"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Department of Collagen Disease and Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Yasushi Kawaguchi"
    }
  ],
  "title": "P.342 The prognosis for pulmonary hypertension in systemic sclerosis complicated by Sjögren’s syndrome is favorable",
  "uid": "0ea15e2c-af65-509f-abad-7d627146430b"
}