{
  "abstract": "Introduction Patients with systemic sclerosis (SSc) may refer sicca symptoms, mainly due to glandular fibrosis but also for coexisting Sjögren’s disease (SjD). The SSc/SjD overlap was described in 1965, but data on these patients remain limited and unexplored. This case-control study aimed to characterize a large cohort of Italian patients with overlapping SSc/SjD, and to identify clinical and immunological differences compared to patients with isolated SSc or SjD.Material and Methods Patients included in the SSc/SjD cohort fulfilled both the 2013 ACR/EULAR classification criteria for SSc and the 2016 ACR/EULAR criteria for SjD. The control groups (1:2 ratio) consisted of patients satisfying criteria for either SSc or SjD, consecutively seen between January and December 2024. Data were extracted from medical records and analyzed using GraphPad Prism (v.9).Results A total of 50 patients with SSc/SjD (98% women, mean age ± standard deviations (SD): 67±11 years) were included and compared to both SSc and SjD control groups ( table 1). Patients with SSc/SjD were significantly older than those with isolated SSc (p<0.01) or SjD (p<0.001) and had a longer disease duration than patients with SSc (p<0.001). Regarding autoimmunity, SSc/SjD patients presented more frequently positive ANA (p<0.01), anti-CENP (p<0.0001), and anti-TopoI (p<0.0001) antibodies than those with SjD. Anti-SSA antibodies were more common in SSc/SjD than in SSc (p<0.0001), whereas anti-SSB antibodies were less common in SSc/SjD than in SjD (p<0.0001). Sicca symptoms were more prevalent in SSc/SjD compared to SSc (p<0.0001). Interstitial lung disease (ILD) was more frequent in SSc/SjD than in SjD (p<0.0001), and comparable to SSc alone. SSc/SjD patients showed lower predicted diffusing capacity for carbon monoxide (DLCO) than both SSc (p=0.02) and SjD (p<0.01) groups. Cutaneous subsets and modified Rodnan skin scores were similar between SSc/SjD and SSc. The occurrence of telangiectasias, Raynaud’s phenomenon, digital ulcers, and osteolysis was comparable between SSc/SjD and SSc, although pitting scars were more frequent in SSc/SjD than in SSc (p=0.01). The prevalence of pulmonary hypertension, gastrointestinal involvement, and calcinosis was similar between SSc/SjD and SSc. Corticosteroid use was more common in SSc/SjD compared to SSc (p=0.04), while biologic agents use was less frequent in SSc/SjD than in SjD (p<0.05). Fibromyalgia was less common (p<0.0001), and neoplasia more frequent (p=0.02) in SSc/SjD than in SjD alone.Conclusions Presence of neoplasia and ILD with reduced DLCO characterize the SSc/SjD overlap. Active screening is recommended, as this may affect the outcomes and indicate the need for a personalized approach.Abstract P.343 Table 1Clinical-demographic profile of patients with SSc/SjD overlap compared to SScand SjD patientsAbstract P.343 Figure 1Comparison of DLCO distribution in SSc-SjD and SSc patients",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy",
        "LUM ‘G. Degennaro’ - Department of Medicine and Surgery, Casamassima, Italy., Casamassima, Italy"
      ],
      "name": "Stefano Stano"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy",
        "Rheumatology Unit - F. Miulli General Hospital, Department of Medicine and Surgery, LUM G. Degennaro, Casamassima, Italy"
      ],
      "name": "Fabio Cacciapaglia"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Maria Morrone"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Simone Perniola"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Marco Capodiferro"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Marco Fornaro"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Laura Coladonato"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Giuseppe Lopalco"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Aldo, Bari, Italy"
      ],
      "name": "Florenzo Iannone"
    }
  ],
  "title": "P.343 Overlap of systemic sclerosis and Sjögren’s disease: insights from an Italian case-control study",
  "uid": "e6ca210a-bda1-590d-b7df-9e67d6b93544"
}