{
  "abstract": "Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotising vasculitis characterised by tissue and blood hypereosinophilia. Cardiac involvement has previously been identified as the most significant predictor of mortality.Objective To identify and characterise previously untreated patients with EGPA and their cardiac manifestations at disease onset.Methods A retrospective monocentre study was conducted on 103 patients. Upon patient presentation, detailed multidisciplinary physical evaluations and laboratory diagnostics were performed. Cardiac events were defined as EGPA-related abnormalities in ECG, echocardiography, cardiac MRI, invasive coronary angiography or cardiac histopathology.Results Cardiac manifestations were diagnosed in 36 of 103 patients with EGPA (35%). Patients with EGPA with cardiac involvement (EGPA-CI) exhibited typical symptoms of EGPA concomitant with elevated cardiac biomarkers. Pericarditis (77%), cardiomyopathy with cardiac failure (55%) and definite myocarditis (36%) were the most common diagnoses, which in some cases resulted in cardiogenic shock (14%) or cardiac arrest (6%). Vasospastic coronary arteries causing myocardial infarctions were identified as a life-threatening cardiac manifestation in 8% of patients with EGPA-CI. Overall, patients with EGPA-CI presented with significantly higher disease activity and worse prognosis, with elevated median eosinophilic count and C-reactive protein levels.Conclusions Cardiac involvement is a common initial manifestation of EGPA and is associated with elevated systemic disease activity. In addition to pericarditis and myocarditis, coronary vasospasms were identified as a rare and severe manifestation of EGPA that may mimic myocardial infarction. Consequently, at the early stage of disease, patients may present with advanced cardiac impairment, emphasising the necessity of prompt diagnostic and therapeutic intervention to positively affect prognosis.",
  "authors": [
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Markus A Schramm"
    },
    {
      "affiliations": [
        "Department of Pneumology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Björn C Frye"
    },
    {
      "affiliations": [
        "Interdisciplinary Medical Intensive Care, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Dawid L Staudacher"
    },
    {
      "affiliations": [
        "Department of Diagnostic and Interventional Radiology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Christopher L Schlett"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Franz Thiele"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Reinhard E Voll"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Jens Thiel"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany"
      ],
      "name": "Nils Venhoff"
    }
  ],
  "title": "Coronary vasospasms and other cardiac manifestations in Eosinophilic Granulomatosis with Polyangiitis: Clinical impact and frequency in a monocentre study of 103 patients",
  "uid": "b81331d8-ba85-591b-a935-5d5b5f3866dc"
}