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Cardiac and pulmonary manifestations of Behçet syndrome
1Department of Pathology, Mayo Clinic, Rochester, Minnesota.
Insights
Behçet syndrome, a systemic inflammatory disease, can affect the heart and lungs. This case highlights rare cardiac and pulmonary complications, including myocarditis and pulmonary artery aneurysms.
Area of Science:
- Cardiovascular Medicine
- Rheumatology
- Pulmonology
Background:
- Behçet syndrome is a multisystemic inflammatory disorder.
- Cardiac and pulmonary involvement in Behçet syndrome are infrequent but can be severe.
- Pathologic documentation of these major organ involvements is limited.
Purpose of the Study:
- To describe the pathologic findings of a fatal case of Behçet syndrome.
- To highlight the co-existence of myocarditis, pulmonary thromboangiitis, and pulmonary artery aneurysms in Behçet syndrome.
Main Methods:
- Autopsy examination of a patient with a fatal outcome attributed to Behçet syndrome.
- Histopathological analysis of cardiac and pulmonary tissues.
Main Results:
- The case demonstrated co-existing myocarditis, pulmonary thromboangiitis, and pulmonary artery aneurysms.
- These findings represent uncommon but severe manifestations of Behçet syndrome.
Conclusions:
- Behçet syndrome can present with severe, life-threatening cardiac and pulmonary pathology.
- Autopsy findings underscore the systemic nature and potential for diverse organ involvement in Behçet syndrome.
Abstract:
Behçet syndrome is a systemic disease rather than the initial more narrowly characterized triple symptom-complex of recurrent oral and genital ulcers and chronic iridocyclitis. Major cardiac and pulmonary manifestations of the disease are relatively uncommon and their pathologic features have seldom been documented. Described herein is a fatal case of Behçet syndrome with co-existing myocarditis, pulmonary thromboangiitis, and pulmonary artery aneurysms.