{
  "abstract": "Introduction Systemic sclerosis (SSc) with symptomatic cardiac involvement is associated with a poor prognosis, with a 2-year mortality rate approaching 60%. Cardiac manifestations appear to be more severe in men and are often associated with pulmonary hypertension, although myocardial involvement is increasingly recognized as a major contributor to morbidity and mortality.Material and Methods Case 1: A 29-year-old woman with overlap syndrome of SSc and polymyositis (diagnosed in 2019, with positive anti-SCL70 and anti-PM/SCL100 antibodies) presented with diffuse cutaneous, articular, vascular, gastrointestinal, and muscular involvement, treated with nifedipine 30mg per day and methotrexate 20mg per week. In 2022, she developed acute chest pain, arrhythmic heart sounds, and pulmonary crackles. On admission, she had tachycardia (150 bpm) and elevated BNP (1002 pg/mL). Echocardiography revealed impaired left ventricular ejection fraction (LVEF), without obstructive coronary disease on catheterization. Cardiac MRI showed dilated cardiomyopathy, and endomyocardial biopsy confirmed lymphocytic myocarditis; viral studies were negative. Myocardial involvement secondary to SSc was assumed, and she was treated with intravenous cyclophosphamide (cumulative 6 g), followed by mycophenolate mofetil (2 g/day), leading to initial clinical and laboratory improvement. In February 2025, she was readmitted for acute heart failure with severely reduced LVEF (9%). Despite optimized immunosuppressive and supportive therapy, she died 22 days after admission.Results Case 2: A 40-year-old man, previously healthy, presented in 2022 with progressive dyspnea, orthopnea, and peripheral edema, along with a 5-month history of polyarthritis, Raynaud’s phenomenon, and puffy hands with proximal sclerodactyly. Laboratory workup revealed elevated BNP (286 pg/mL), troponin I (696 ng/mL), positive ANA (1:1000, speckled pattern), and positivity for anti-histone and anti-RNA polymerase III antibodies. Echocardiography suggested infiltrative cardiomyopathy, and cardiac MRI demonstrated moderately reduced LVEF (42%) with non-ischemic focal fibrosis, consistent with inflammatory cardiomyopathy. Nailfold capillaroscopy showed early scleroderma pattern. A diagnosis of SSc with cardiac involvement was established, and the patient received six monthly cycles of intravenous cyclophosphamide (1 g). He is currently maintained on mycophenolic acid (1440 mg/day) with complete recovery of biventricular systolic function.Conclusions Myocardial involvement in SSc may occur early and requires a high index of suspicion. Prompt recognition and aggressive immunosuppressive treatment can favorably alter disease trajectory. Nonetheless, cardiac disease in SSc remains associated with poor outcomes, underscoring the need for close monitoring and multidisciplinary management.",
  "authors": [
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Ana Da Rocha Sá"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Carlos Marques Gomes"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Mariana Diz Lopes"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Bárbara Esteves"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal",
        "Hospital Divino Espírito Santo - Rheumatology Department, Ponta Delgada, Portugal"
      ],
      "name": "Marina Oliveira"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Miguel Natal"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Sara Helena Amaro Lopes"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Georgina Terroso"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Raquel Miriam Ferreira"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Lúcia Costa"
    },
    {
      "affiliations": [
        "ULS São João - Rheumatology Department, Porto, Portugal"
      ],
      "name": "Salomé Garcia"
    }
  ],
  "title": "P.159 Early myocardial involvement in patients with systemic sclerosis",
  "uid": "27dd64a9-ea5d-5c55-b6fc-354d32396f0d"
}