New approaches in the treatment of Adamantiades-Behçet's disease

To update clinicians on the recent advances in the treatment of Adamantiades-Behçet's disease.

Interferon-alpha-2a and infliximab have proved able to induce prompt remission in the vast majority of Adamantiades-Behçet's patients with DMARD-resistant uveoretinitis. Efficacy of interferon-alpha-2a has also been reported for mucocutaneous lesions, arthritis, and (more anecdotally) for neuro-Behçet, while results from small case series suggest that infliximab is beneficial for mucocutaneous lesions and (more anecdotally) for arthritis and gastro-intestinal manifestations. Two cases of neuro-Behçet treated with infliximab showed a complete resolution. Finally, in a randomized controlled trial of patients with mucocutaneous, arthritic manifestations, or both, etanercept effectively suppressed mucocutaneous lesions.A different approach is tolerization by oral administration of the 336-351 peptide of the human heat shock protein 60 (thought to have a pathogenic role in Adamantiades-Behçet's disease-associated uveitis), linked to recombinant cholera B-toxin B-subunit. Preliminary results have shown that tolerization is safe and effective in preventing relapses of uveitis.

Biologic agents have proved effective in patients resistant to conventional treatment. However, disease subsets characterized by severe morbidity and mortality such as vasculo-Behçet and neuro-Behçet still pose major therapeutic challenges. Further studies are needed to devise better treatment strategies for severe Adamantiades-Behçet's disease.