{
  "abstract": "Introduction Diffuse systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by fibrosis, vasculopathy, and autoantibody production. The diffuse cutaneous subtype typically presents with early skin sclerosis, severe Raynaud’s phenomenon, and multi-organ involvement. Management relies on vasodilators and immunosuppressive therapy, with prognosis determined by internal organ disease severity.Material and Methods case reportResults We report a 69-year-old woman initially diagnosed with limited cutaneous SSc in 1996 (antinuclear antibody, centromere pattern 1:1000; anti-centromere positivity), who progressed to diffuse cutaneous SSc in 2016 (anti-Scl-70 positive). Her disease course was complicated by recurrent digital ulcers from 2002, primary biliary cholangitis since 2009, and pulmonary involvement with nonspecific interstitial pneumonia (NSIP) since 2012.Digital ulcers were refractory to nifedipine 60 mg/day, prompting escalation to alprostadil in 2013 and iloprost in 2016, both initially effective. Iloprost was successfully re-administered in 2019 and 2024 but discontinued in 2023 and 2025 due to symptomatic hypotension. Sildenafil (up to 150 mg/day, 2024–2025) proved ineffective. Bosentan, initiated in January 2025, was discontinued after re-hospitalization in August 2025 for multiple ulcers and chronic osteomyelitis of the right fifth digit. More recently, tadalafil 20 mg/day was introduced with tolerance, though efficacy remains to be determined. Systemic vasodilator use was further restricted by concomitant venous leg ulcers.Osteomyelitis developed in the right second digit in 2019, requiring multiple antibiotic courses (flucloxacillin, amoxicillin–clavulanate, clindamycin, trimethoprim–sulfamethoxazole) following Staphylococcus aureus isolation, with relapse in 2020 managed with ciprofloxacin. Extension to the right third digit in 2023 necessitated further antimicrobial therapy and subsequent amputation in 2024. Chronic osteomyelitis of the right fifth digit prompted surgical consideration in May 2025. In August 2025, follow-up MRI revealed progression to the second and fourth digits of the right hand and the left third digit, requiring multidisciplinary management with Orthopedics and Infectious Diseases.Mycophenolate mofetil (2017–2019) was used for progressive skin fibrosis and NSIP but discontinued due to recurrent osteomyelitis. Nintedanib (2021–2024) was initiated for progressive pulmonary fibrosis but withdrawn due to severe diarrhea and weight loss. Despite cessation of systemic therapy, pulmonary disease has remained clinically stable.Conclusions This case illustrates the complex therapeutic challenges in SSc management, where recurrent infections contraindicated immunosuppression, intolerance limited antifibrotic therapy, and vasodilator-related hypotension compromised vascular control. Phosphodiesterase-5 inhibitors remain recommended, with the therapeutic impact of tadalafil still under evaluation.",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Sara Amaro Lopes"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Mariana Diz-Lopes"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Carlos Marques-Gomes"
    },
    {
      "affiliations": [
        "Rheumatology Department, Hospital do Divino Espírito Santo, Ponta Delgada, Portugal, Ponta Delgada, Portugal"
      ],
      "name": "Marina Oliveira"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Bárbara Fernandes Esteves"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Miguel Correia Natal"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Ana da Rocha Sá"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Bruno Miguel Fernandes"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Lúcia Costa"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Raquel Miriam Ferreira"
    },
    {
      "affiliations": [
        "Rheumatology Department, ULS São João, Porto, Portugal, Porto, Portugal"
      ],
      "name": "Georgina Terroso"
    }
  ],
  "title": "P.193 Refractory digital ulcers and chronic osteomyelitis complicating diffuse systemic sclerosis: a real-life case",
  "uid": "0da3e772-2ef0-5f8d-870f-e6e9ee52b607"
}