PURPOSE OF REVIEW: To update clinicians on the recent advances in the treatment of Adamantiades-Behçet's disease. RECENT FINDINGS: 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. SUMMARY: 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.
PURPOSE OF REVIEW: To update clinicians on the recent advances in the treatment of Adamantiades-Behçet's disease. RECENT FINDINGS: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. SUMMARY: 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.