
Background: Vascular involvement is an important cause of morbidity and mortality in patients with Behçet syndrome (BS). TNF inhibitors have been reported to be effective for almost all serious manifestations of BS but data on vascular involvement is still limited.
Objectives: To survey the efficacy and safety of infliximab (IFX) in BS patients with vascular involvement followed in a dedicated tertiary center.
Methods: We reviewed the charts of all BS patients who used IFX and identified those who used this drug for vascular involvement. A standard form was used for extracting data on demographic and clinical features, type of vascular involvement, concomitant immunosuppressives, duration of IFX use, relapses, adverse events and outcome.
Results: 83 patients (67 men, 16 women, mean age 39.5 ± 8.4 SD years) had used IFX for vascular involvement. The number of patients with each type of vascular involvement as the main indication for IFX, and previous treatment modalities right before IFX are presented in the
Conclusion: Infliximab seems to be beneficial in Behçet syndrome patients with vascular involvement, even in those who are refractory to immunosuppressives and corticosteroids. No further relapses occurred in 68% of the patients, and adverse events leading to discontinuation were observed in 18%.
| Arterial Involvement (n=36 ) | Venous Involvement (n=47 ) | |
| Main reason for infliximab use | Pulmonary artery aneurysm and/or thrombosis, n=29
| Vena cava superior and/or inferior thrombosis, n=5
|
| Previous treatment | ||
| First line infliximab | 4 | 13 |
| Corticosteroids | 34 | 38 |
| Cyclophosphamide (CYC) | 25 (15/25 refractory to CYC; 10/25 for maintenance after CYC) | 11 (8/11 refractory to CYC; 3/11 for maintenance after CYC) |
| Interferon-alpha | - | 13 |
| Azathioprine | 21 | 17 |
| Mycophenolate Mofetil | 1 | 2 |
| Cyclosporine A | - | 2 |
| None due to non-compliance | 2 | 1 |
| Outcome | ||
| Remission, still on infliximab | 16 | 23 |
| Discontinued due to remission | 4 | 8 |
| Discontinued due to adverse events | 5 | 9 |
| Discontinued due to relapse | 5 | 3 |
| Discontinued due to non-compliance | 5 | 3 |
| Died | 2 (1 had previously discontinued due to relapse) | 1 |
Disclosure of Interests: None declared