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Related Experiment Videos

Pedro Madureira1, Mariana Rodrigues2, Edite Serrano3

  • 1Serviço de Reumatologia, Centro Hospitalar de São João, Porto, Portugal; Departamento de Reumatologia, Faculdade de Medicina do Porto, Porto, Portugal.

Revista Brasileira De Reumatologia
|January 3, 2015
PubMed
Summary

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Behçet's Disease (BD) can cause rare intracardiac thrombosis. Early diagnosis and combined treatment with anticoagulation and immunosuppression, including cyclophosphamide and prednisolone, are crucial for remission in these challenging cases.

Area of Science:

  • Cardiology
  • Rheumatology
  • Vascular Medicine

Background:

  • Behçet's Disease (BD) is a rare multisystemic vasculitis.
  • Intracardiac lesions and thrombosis are uncommon but severe manifestations of BD.
  • Diagnosis can be challenging, often mimicking infective endocarditis.

Purpose of the Study:

  • To report a rare case of intracardiac thrombosis in an adolescent with Behçet's Disease.
  • To highlight the diagnostic challenges and management strategies for this condition.
  • To emphasize the importance of prompt and aggressive treatment for favorable outcomes.

Main Methods:

  • Case report of an adolescent diagnosed with Behçet's Disease.
  • Utilized transthoracic echocardiography and contrast-enhanced cardiac MRI for diagnosis.
Keywords:
AnticoagulantesAnticoagulationBehçet's diseaseCiclofosfamidaCyclophosphamideDoença de BehçetIntracardiac thrombosisSuperior vena cava syndromeSíndrome da veia cava superiorTrombose intracardíaca

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  • Managed with surgical excision of lesions, anticoagulation, cyclophosphamide, and high-dose prednisolone.

    • Main Results:

    • The patient presented with fever and intracardiac lesions initially suspected as endocarditis.
    • Cardiac MRI revealed intracardiac thrombosis, superior vena cava syndrome, and pulmonary thromboembolism.
    • Complete remission was achieved with combined immunosuppressive and anticoagulant therapy post-surgery.

    Conclusions:

    • Intracardiac thrombosis is a rare but serious manifestation of Behçet's Disease with a poor prognosis.
    • A multimodal treatment approach involving anticoagulation and potent immunosuppression is essential for managing this complication.
    • Aggressive management, including surgery and pharmacotherapy, can lead to complete remission.