{
  "abstract": "Introduction Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by vasculopathy, autoimmune activation and fibrosis of the skin and internal organs including the lungs. Mediastinal lymphadenopathy is not typical in SSc and should raise suspicion for alternative or coexisting conditions. Sarcoidosis is a primary T-cell mediated disease that commonly affects the lungs and lymph nodes. The coexistence of SSc and sarcoidosis is rare and poses diagnostic and therapeutic challenges.Material and Methods We describe two female patients with overlapping SSc and sarcoidosis, presenting with distinct timelines of disease onset.Results The first patient is 64-year-old woman who presented with a 6-month dyspnea on excertion, that was steadily worsening. On physical examination sclerodactyly was detected. Lung auscultation revealed velcro sounds at lower lobes bilaterally. The patient reported Raynaud’s phenomenon for at least a year as well as gastroesophageal reflux symptoms. Antinuclear antibodies (ANA) were positive at 1:1280 titer with speckled pattern and anti-Topoisomerase highly positive. Further evaluation included pulmonary function tests with restrictive pattern and decreased DLCO (FVC 73%, DLCO 67%). High-resolution computed tomography imaging showed ground glass opacities, and some septal thickening; a pattern compatible with nonspecific interstitial pneumonia. Additionally, bilateral symmetric hilar lymphadenopathy, measured 2.3cm in size, was present. Given the unexpected lymphadenopathy, an endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was performed which revealed non-caseating granulomas. The findings were consistent with pulmonary sarcoidosis. The patient was treated with coricosteroids and rituximab.The second patient is a 62-year-old lady with SSc since 1995. Initial presentation included arthritis, Raynaud, puffy hands, telangiectasias, ANA positive. She was treated with methotrexate . Over the course of the disease she developed digital ulcers, as well as skin and lung fibrosis and was treated with mycophenolate mofetil. In 2017, she developed persistent fever and dyspnea. A diagnostic workout revealed bilateral hilar lymphadenopathy and biopsy confirmed the presence of non-caseating granulomas. Corticosteroid therapy was initiated , resulting in an inital improvement. However, two years later she experienced a recurrence of fever and hilar lymphadenopathy. At that time she was treated with corticosteroids and rituximab with clinical response.Conclusions The co-occurrence of SSc and sarcoidosis, although rare, highlights the importance of considering alternative or coexisting diagnoses in SSc patients presenting with atypical radiologic findings, such as significant mediastinal lymphadenopathy. The therapeutic approach was challenging, as prolonged corticosteroid use carries significant risks in SSc, including renal crisis, while the use of rituximab in sarcoidosis—a predominantly T-cell mediated disease—is limited.Abstract P.345 Figure 1",
  "authors": [
    {
      "affiliations": [
        "University of Thessaly, Faculty of Medicine, Department of Rheumatology And Clinical Immunology, Larissa, Greece"
      ],
      "name": "Theodora Simopoulou"
    },
    {
      "affiliations": [
        "University of Thessaly, Faculty of Medicine, Department of Rheumatology And Clinical Immunology, Larissa, Greece"
      ],
      "name": "Dimitrios Bogdanos"
    },
    {
      "affiliations": [
        "University of Thessaly, Faculty of Medicine, Department of Rheumatology And Clinical Immunology, Larissa, Greece"
      ],
      "name": "Christina Katsiarh"
    }
  ],
  "title": "P.345 Mediastinal lymphadenopathy in SSc patients",
  "uid": "fca569ab-341a-5654-bbfc-9f812b4e0049"
}