Behçet's disease associated with superior vena cava syndrome without thrombosis.
Clin Rheumatol
; 26(5): 804-6, 2007 May.
Article
in En
| MEDLINE
| ID: mdl-16538386
ABSTRACT
Behçet's disease is a multisystemic vasculitis of unknown etiology, which is characterized by recurrent urogenital ulceration, cutaneous eruptions, ocular manifestations, arthritis and vasculitis, and its diagnosis is based on clinical criteria. Superior vena cava (SVC) thrombosis is a rare but well-recognized manifestation of Behçet's disease, whereas SVC syndrome due to vasculopathy, without evidence of thrombosis, has not yet been described in the literature. The authors report the case of a patient with Behçet's disease, who presented SVC syndrome with reduction in the lumen of the SVC due to thickening of the vessel wall, without evidence of thrombosis upon computed tomography and magnetic angioresonance. The patient received early anticoagulant therapy, corticosteroid and monthly cyclophosphamide pulse therapy. Clinical control without recurrence was observed after 6 months of follow-up. Behçet's disease should be suspected in young patients presenting with SVC syndrome, in the absence of thrombosis or of a hypercoagulable state.
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Database:
MEDLINE
Main subject:
Superior Vena Cava Syndrome
/
Behcet Syndrome
Type of study:
Diagnostic_studies
/
Risk_factors_studies
Limits:
Adult
/
Humans
/
Male
Language:
En
Year:
2007
Type:
Article