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The many faces of Behçet's disease
Jeffrey F B Chick1, Jared D Miller, Ryan J Bair
1aDepartment of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts bDepartment of Internal Medicine, Stanford Hospital and Clinics, Stanford University School of Medicine, Stanford, California cDepartment of Radiation Oncology, University of Chicago Medical Center, Pritzker School of Medicine, Chicago, Illinois, USA.
Journal of Cardiovascular Medicine (Hagerstown, Md.)
|September 12, 2012
Summary
Behçet's disease, a rare inflammatory condition, can affect multiple organ systems. This case highlights severe vascular and systemic complications diagnosed using advanced imaging.
Area of Science:
- Internal Medicine
- Rheumatology
- Vascular Medicine
Background:
- Behçet's disease is a chronic, multisystem inflammatory disorder of unknown etiology.
- It is characterized by recurrent oral and genital ulcers, skin lesions, and ocular inflammation.
- Vascular involvement is a major cause of morbidity and mortality.
Observation:
- A 23-year-old male presented with constitutional symptoms (fevers, night sweats), lymphadenopathy, visual impairment, and aphthous ulcers.
- Clinical presentation suggested a systemic inflammatory process.
- Diagnostic workup was initiated to identify the underlying cause and extent of disease.
Findings:
- The patient was diagnosed with Behçet's disease based on characteristic clinical features.
- Advanced imaging revealed extensive multisystem complications.
- These included deep vein thrombosis, mediastinal fibrosis, bilateral chylothoraces, chylopericardium, and coronary artery aneurysms.
Implications:
- This case underscores the diverse and severe systemic manifestations of Behçet's disease, particularly vascular complications.
- Multimodality imaging is crucial for comprehensive assessment and management of patients with Behçet's disease.
- Early recognition and aggressive management are vital to prevent life-threatening complications.
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