{
  "abstract": "Introduction Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by fibrosis, vasculopathy, and specific autoantibodies. Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease. Their coexistence, termed Reynolds’ syndrome (RS), is rare and poorly characterized. Current knowledge is limited to small series and case reports.Material and Methods Retrospective observational study of RS patients followed at the Rheumatology outpatient clinic of a tertiary hospital. Cases fulfilled the 2013 ACR/EULAR classification criteria for SSc and had a concomitant diagnosis of PBC. For each case, one control with SSc alone was selected, matched for sex, SSc subtype, age at baseline and disease duration. Baseline was defined as the date of PBC diagnosis in overlap cases, and a corresponding visit with similar SSc disease duration in controls. Clinical and immunological data were retrieved from medical records.Results Twelve RS patients were included, all female, with mean age at baseline 55.5 ± 12.5 years. Mean age at SSc diagnosis was 49.3 ± 13.6 years and at PBC diagnosis 54.8 ± 12.7 years. Most patients (83.3%) were first diagnosed with SSc, followed by PBC; the median interval between diagnoses was 3.0 years (IQR 10.25). All received ursodeoxycholic acid with good biochemical response; three (25.0%) developed cirrhosis. At baseline, RS patients showed cholestatic liver involvement with median alkaline phosphatase 128.5 U/L (IQR 165.5) and GGT 85.5 U/L (IQR 175.0). Anti-mitochondrial antibodies were present in 66.7%.Cases and controls were well balanced for baseline characteristics. Compared to controls, RS patients had lower DLCO (65.9% vs 80.4% predicted, p=0.001). There was a trend toward more sicca symptoms (50.0% vs 8.3%, p=0.07). All RS patients had centromere ANA pattern and anti-centromere antibody positivity, compared to 66.7% of controls (p=0.09). No significant differences were seen in other clinical features, lung function (FVC), or autoantibody profiles.Conclusions In this cohort of patients with RS, the condition occurred exclusively in women and was most often characterized by an initial SSc diagnosis followed by PBC. RS patients showed a predominance of the limited cutaneous subtype, universal anti-centromere antibody, and a trend toward higher prevalence of sicca symptoms. In addition, they had a significantly lower DLCO compared with matched SSc controls, suggesting greater pulmonary vascular involvement; aside from this finding, most other clinical features and autoantibody profiles were similar between groups. These results emphasize the need for systematic lung function monitoring in RS patients and support further studies to define the clinical course of this rare overlap.Abstract P.347 Table 1Baseline clinical and immunological characteristics of patients with Reynolds syndrome compared with matched systemic sclerosis controls",
  "authors": [
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Miguel Natal"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Mariana Diz-Lopes"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Carlos Marques-Gomes"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Bárbara Esteves"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal",
        "Hospital do Divino Espírito Santo - Department of Rheumatology, Ponta Delgada, Portugal"
      ],
      "name": "Marina Oliveira"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal",
        "ULS Gaia Espinho - Department of Rheumatology, Vila Nova de Gaia, Portugal"
      ],
      "name": "Catarina Rua"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Ana Rocha Sá"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Sara Amaro Lopes"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Lúcia Costa"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Raquel Miriam Ferreira"
    },
    {
      "affiliations": [
        "ULS São João - Department of Rheumatology, Porto, Portugal"
      ],
      "name": "Georgina Terroso"
    }
  ],
  "title": "P.347 Clinical and serological profile of systemic sclerosis-primary biliary cholangitis overlap (reynolds syndrome): a matched case-control analysis",
  "uid": "632fd0a8-620d-56ea-a82f-e117572b2b18"
}