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Published on: June 30, 2023
Behçet's Disease and Intracardiac Thrombosis: A Report of Three Cases
Nurşen Düzgün1, Orhan Küçükşahin, Kayhan Çetin Atasoy
1Department of Internal Medicine-Rheumatology, Ankara University, Faculty of Medicine, Akademik Yerleşke, Sıhhıye, 06100 Ankara, Turkey.
Insights
Behçet
Area of Science:
- Cardiovascular Medicine
- Rheumatology
- Immunology
Background:
- Behçet's disease is a rare multisystemic inflammatory disorder.
- Intracardiac thrombus and pulmonary vascular involvement are uncommon manifestations.
- Budd-Chiari syndrome can be a co-occurring condition.
Purpose of the Study:
- To report on three cases of Behçet's disease with intracardiac thrombus and pulmonary vascular disease.
- To evaluate the efficacy of immunosuppressive therapy in managing these cardiovascular complications.
Main Methods:
- Case series presentation of three patients diagnosed with Behçet's disease.
- Treatment involved first-line therapy with corticosteroids and monthly intravenous cyclophosphamide.
- Clinical outcomes, including thrombus resolution and disease recurrence, were monitored.
Main Results:
- All three patients achieved remission without recurrence following immunosuppressive treatment.
- Intracardiac thrombi resolved, and pulmonary vascular thromboses regressed.
- One patient with Budd-Chiari syndrome also showed positive response to therapy.
Conclusions:
- Combined corticosteroid and cyclophosphamide therapy is effective for intracardiac thrombus and pulmonary vascular involvement in Behçet's disease.
- Behçet's disease should be considered in the differential diagnosis of intracardiac masses, particularly in endemic populations.
- Early diagnosis and aggressive immunosuppression can prevent disease progression and recurrence.
Abstract:
We present three patients with Behçet's disease associated with intracardiac thrombus and pulmonary vascular involvement. One of these patients had also Budd-Chiari syndrome. All patients were treated with corticosteroid plus monthly intravenous cyclophosphamide as first line treatment and with no recurrences. Immunosuppressive therapy was successful in the treatment of intracardiac thrombus and also in the regression of pulmonary vascular thromboses in these patients. Intracardiac thrombus in Behçet's disease is rarely seen. Behçet's disease should be remembered in the differential diagnosis of the patients with intracardiac mass, especially in patients from the Mediterranean and Middle East populations.
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