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Published on: February 8, 2019
Vascular involvement in Behçet's syndrome: a retrospective analysis of associations and the time course
Koray Tascilar1, Melike Melikoglu2, Serdal Ugurlu1
1Department of Rheumatology, Istanbul University Cerrahpasa Medical Faculty, Istanbul and Department of Biostatistics and Medical Informatics, Trakya University Medical Faculty, Edirne, Turkey.
Insights
Behçet's syndrome often involves vascular complications. While deep vein thrombosis (DVT) is common and often occurs alone, other venous diseases and pulmonary artery involvement cluster together, but extrapulmonary artery involvement is distinct.
Area of Science:
- Vascular Medicine
- Rheumatology
- Internal Medicine
Background:
- Behçet's syndrome (BS) is a multisystem inflammatory disorder.
- Vascular involvement is a significant manifestation of BS, affecting both venous and arterial systems.
- Understanding patterns of vascular events in BS is crucial for patient management.
Purpose of the Study:
- To explore the associations and timing of various vascular events in Behçet's syndrome.
- To identify distinct patterns of vascular involvement within the venous and arterial trees.
- To estimate the risk of recurrent vascular events in BS patients.
Main Methods:
- Chart survey of vascular involvement type and timing in BS patients.
- Phi correlation coefficients to assess cross-relationships of vascular involvement.
- Multiple correspondence analysis to identify patterns of vascular involvement.
- Estimation of vascular recurrence risk.
Main Results:
- 14.7% of BS patients (882/5970) had vascular involvement.
- Deep vein thrombosis (DVT) was the most frequent event (67.1%), often isolated and in the legs.
- Cumulative 5-year risk of recurrent vascular events was 38.4%.
- Significant correlations and clustering observed between pulmonary artery involvement (PAI), dural sinus thrombosis (DST), Budd-Chiari syndrome (BCS), and vena cava syndromes (IVCS/SVCS).
- Extrapulmonary artery involvement (EPAI) patients were older and clustered separately from other vascular manifestations.
- DVT clustered separately due to its tendency to occur solitarily.
Conclusions:
- Solo DVT, predominantly in the legs, is the most common vascular manifestation in Behçet's syndrome.
- Various venous diseases in BS, along with pulmonary artery involvement, tend to occur together.
- Extrapulmonary artery involvement represents a distinct pattern of vascular disease in Behçet's syndrome.
Objective:
Some features of Behçet's syndrome (BS) tend to go together. We aimed to explore the association and timing of various vascular events in both the venous and the arterial vascular tree.
Methods:
We conducted a chart survey on the type and time of vascular involvement of BS. The cross-relationships of involvement were assessed by phi correlation coefficients. Multiple correspondence analysis was used to identify patterns of vascular involvement. The risk of vascular recurrence was also estimated.
Results:
We identified 882 patients with vascular involvement among 5970 BS patients (14.7%). Deep vein thrombosis (DVT), almost always in the legs, was the most frequent single vascular event (592/882; 67.1%). The cumulative risk of a recurrent vascular event was 38.4% at 5 years. Patients with extrapulmonary artery involvement (EPAI) were significantly older than those with venous and pulmonary artery involvement (PAI). There were significant correlations between dural sinus thrombosis (DST) and PAI, Budd-Chiari syndrome (BCS) and inferior vena cava syndrome (IVCS) and between IVCS and superior vena cava syndrome (SVCS). Multiple correspondence analysis further indicated clustering of PAI, DST, BCS, IVCS and SVCS. However, EPAI and DVT clustered separately from forms of vascular disease, the separate clustering of the DVT being attributed to its propensity to occur solo.
Conclusion:
The most common type of vascular involvement in BS is solo DVT, almost always occurring in the legs. Various forms of venous disease in BS segregate together and PAI is included in this group. EPAI segregates separately.
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