Effectiveness and safety of low-dose interferon alpha-2a treatment in Behçet's Syndrome with refractory vascular or neurological involvement: a case series.

Objective: The aim of this study was to evaluate the effectiveness and safety of low-dose interferon alpha-2a (IFNα2a) in Behçet's syndrome (BS) patients with refractory vascular/cardiac or neurological involvement.
Methods: In this retrospective cohort study, we consecutively included 25 BS patients with refractory vascular/cardiac ( n  = 16) or neurological involvement ( n  = 9) who received IFNα2a treatment in our center between June 2018 and September 2021. The low-dose IFNα2a (3 million IU, every other day) was used as an add-on treatment with the continuation of glucocorticoids (GCs) and immunosuppressants.
Results: In total, 25 patients (20 males, 5 females) with a mean age of 31.92 ± 9.25 years were included. IFNα2a was administered for BS patients with refractory vascular/cardiac involvement ( n  = 16) and neurological involvement ( n  = 9). Before the initiation of IFNα2a, patients had insufficient response or intolerance to conventional therapies. After a median follow-up of 23 [interquartile range (IQR), 11-30] months, all patients achieved clinical improvement. The Behçet's disease Current Activity Form (BDCAF) score improved significantly (5 versus 0, median, p  < 0.0001). BS Overall Damage Index (BODI) and vasculitis damage index (VDI) remain stable ( p  > 0.05). Decrease in erythrocyte sedimentation rate [ESR; 24 (IQR, 12-43.5) versus 5 (IQR, 2.75-10.5) mm/h, p  = 0.0001] and C-reactive protein [CRP; 6.64 (IQR, 3.67-19.82) versus 1.24 (IQR, 0.24-3.12) mg/liter, p  < 0.005] was achieved effectively. The median GCs dosage tapered from 26.25 (IQR, 11.88-41.25) to 10.00 (IQR, 7.50-10.63) mg/d, p  < 0.0001. Immunosuppressants were also reduced in number ( p  < 0.005). No serious adverse events were observed during follow-up.
Conclusion: Our study suggests that low-dose IFNα2a, combined with GCs and immunosuppressants, is well-tolerated and effective for BS patients with refractory vascular/cardiac or neurological involvement and has a steroid- and immunosuppressant-sparing effect.
Competing Interests: Competing interests: The authors declare that there is no conflict of interest.
(© The Author(s), 2022.)