Adalimumab treatment for life threatening pulmonary artery aneurysm in Behçet disease: a case report

Abstract

Behçet's disease (BD) is a multisystem disorder characterized by vasculitis. Pulmonary vascular problems such as pulmonary artery aneurysms (PAA) are reported to indicate poor prognosis and high mortality. We describe a 43-year-old man who presented with life threatening bilateral PAA and thromboembolic disease due to BD. He was treated with prednisone and pulse cyclophosphamide and was poorly responsive to the conventional immunosuppression. The introduction of adalimumab therapy stabilized his PAA. We report that the inhibition of TNF-alpha using the neutralizing monoclonal antibody adalimumab has the potential to induce rapid, complete, and long-lasting remission in a life-threatening manifestation of BD.

Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients

“…Until now, the off-label use of anti-TNF in BD patients with extra-ocular manifestations was based on reports on small series or single cases. Overall, the majority of patients treated with IFX, ADA or ETA showed improvement of mucocutaneous [15,16,30e33], gastrointestinal [15,20,21,34e37], neurologic [12,17e19,38e41] and cardiovascular manifestations [22e24, 42,43]. Recent literature reviews on anti-TNF treatment in BD reported up to 90% improvement in extra-ocular manifestations, which substantiate the recommendations on the use of anti-TNF in BD treatment [4,44].…”

Pulmonary artery aneurysms in Behçet disease

“…86 Other tumor necrosis factor-a blockers, which include etanercept and adalimumab, would also be effective for the treatment of PAA in Behçet's disease patients. 77 Pulmonary artery aneurysm patients with thrombophlebitis and/or pulmonary emboli who are on anticoagulants are at risk of massive bleeding and so anticoagulants should be used with great caution, especially in patients with hemoptysis. 76 Alternative management strategies to conservative treatment include surgical and transcatheter interventions.…”

Update on the therapy of Behçet disease

“…Case report [Belzunegui et al 2008] Vascular involvement Case report [Lee et al 2010] Rituximab Eye involvement Case report [Sadreddini et al 2008] Randomized single-blind study [Davatchi et al 2010] Gevokizumab Eye involvement Open label study [Gul et al 2012] Canakinumab Eye involvement Case report [Ugurlu et al 2012] Extraocular disease Case report Anakinra Extraocular disease Case report [Botsios et al 2008] Tocilizumab Neuro-Behçet Case reports [Urbaniak et al 2012;Shapiro et al 2012] Eye involvement Case report [Hirano et al 2012] …”