{
  "abstract": "Introduction Systemic sclerosis (SSc) is an autoimmune, fibrotic disease in which interstitial lung disease (ILD) represents a leading cause of death. 1 Enlarged mediastinal lymph nodes (LNs) have been reported to be detectable by HRCT in SSc-ILD,2 and may predict ILD development.3 Here we aimed to quantify mediastinal lymphadenopathy (LAD) in SSc-ILD and determine its clinical value in assessing SSc-ILD activity.Material and Methods This cross-sectional, retrospective study included seventy-four adult SSc patients fulfilling 2013 ACR/EULAR criteria, 4 all with HRCT-confirmed ILD. One HRCT per patient was reviewed, to assess mediastinal stations using American Thoracic Society thresholds for LAD.5 Beyond binary presence/absence, a quantitative LAD burden was calculated by summing largest short-axis diameters across stations. ILD duration was defined as time since first HRCT detection. Autoantibody status, PFTs, and organ involvement, including cutaneous (mRSS) and upper gastrointestinal (GI) involvement (GERD, dysphagia, early satiety, bloating) were recorded. ILD progression was assessed by INBUILD criteria.6Results The cohort included seventy-four consecutive patients with ILD from 2 centres. 84% females, with 51% DcSSc. Anti-Scl70 antibodies were present in 61%, ACA in 16%. Twenty-six patients (35.1%) progressed in their ILD by 24 months. Sixty-nine (93.2%) were on IS (mainly MMF) at time of HRCTs. Twenty-eight patients (37.8%) presented at least one LAD. 54% of LAD patients experienced ILD progression vs 24% of patients without LAD (p=0.013). LAD was associated with GI involvement (p=0.023); patients with GI involvement had higher LAD burden than those without it (p=0.017). Patients with LAD had lower DLCO% than those without LAD (p=0.019), with LAD burden negatively correlated with DLCO% (p=0.0084). No significant correlations or associations were found with mRSS, disease subset, disease duration, FVC, and ILD duration.Conclusions Mediastinal LAD in SSc-ILD was strongly associated with upper GI involvement, and with ILD progression. The novel mediastinal LAD-GI association warrants further investigation on putative shared disease mechanisms. Independent validation on wider populations will identify the clinical value of LAD detection and quantification as a tool to stratify for ILD disease activity and progression.References doi: 10.1016/j.jaad.2021.10.065Farrokh D, Abbasi B, Fallah-Rastegar Y, Mirfeizi Z. The extrapulmonary manifestations of systemic sclerosis on chest high resolution computed tomography. Tanaffos. 2015;14(3):193–200. PMID: 26858765.doi: 10.1177/2397198320923545doi: 10.1136/annrheumdis-2013-204424doi: 10.2214/ajr.144.2.261doi: 10.1056/NEJMoa1908681Abstract P.088 Figure 2Comparison of LAD burden between patients with and without GI involvement. Patients with GI involvement displayed significantly higher LAD burden values compared to those without GI involvement (p = 0.017)Abstract P.088 Figure 1Association between mediastinal LAD and GI involvement in patients with SSc-ILD. The presence of LAD was significantly more frequent in patients with GI involvement compared to those without (82.1% vs. 54.4%, Fisher’s exact test, p = 0.023)Abstract P.088 Figure 3Association between mediastinal LAD and INBUILD progression in patients with SSc-ILD, The presence of LAD was significantly more frequent in patients with INBUILD progression compared to those without (53.6% vs. 23.9%, Fisher’s exact test, p = 0.013)Abstract P.088 Table 1Association of mediastinal LAD with DLCO% and GI involvement in SSc-ILD patients",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Valeria Rella"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Cinzia Rotondo"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Raffaele Barile"
    },
    {
      "affiliations": [
        "Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, NIHR Leeds Biomedical Research Centre, Leeds, United Kingdom"
      ],
      "name": "Marco Minerba"
    },
    {
      "affiliations": [
        "Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, NIHR Leeds Biomedical Research Centre, Leeds, United Kingdom"
      ],
      "name": "Lucy Thornton"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Addolorata Corrado"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Francesco Paolo Cantatore"
    },
    {
      "affiliations": [
        "Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, NIHR Leeds Biomedical Research Centre, Leeds, United Kingdom"
      ],
      "name": "Francesco Del Galdo"
    }
  ],
  "title": "P.088 Mediastinal lymphadenopathy in SSC-ILD is associated with upper gastrointestinal involvement and higher risk of ild progression: a bicentric retrospective observational study",
  "uid": "a8180fab-1dac-521b-939a-2a590f0ab9e3"
}