Treating the Different Phenotypes of Behçet's Syndrome

Abstract

Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.

Long-term effectiveness and safety of secukinumab for treatment of refractory mucosal and articular Behçet’s phenotype: a multicentre study

“…Indeed, the predominant phenotypes have already been defined and are thought to be governed by different disease mechanisms requiring diversified treatment modalities. 23 Here we report the results of a retrospective study conducted on a series of 15 patients with the mucosal and articular phenotype of BS and treated with secukinumab. The observed effectiveness of secukinumab in this phenotype has a strong pathophysiological basis.…”

Succinivibrionaceae is dominant family in fecal microbiota of Behçet’s Syndrome patients with uveitis

“…During the course of the disease, BS can involve the mucosa, skin, or eyes, vessels, nervous and gastrointestinal system [ 22 , 23 , 46 ]. Several cluster and association studies reported clusters of common overlapping involvements.…”

“…Similarly, Hussein et al reported that genital ulcers and systemic vasculitis were protective factors for the development of vision threatening eye disease [ 50 ]. We focused on BS patients with eye involvement since, each involvement has its own demographic and clinical characteristics, and also medical treatment and prognosis of each manifestation differs greatly [ 46 ]. Oezguen N et al have subjected neuro-Behçet’s disease for the analysis of gut microbiota.…”

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

“…For non-parenchymal disease with CVST, first-line therapy is also high-dose steroids with an oral taper but further immunosuppression is not usually necessary as relapses are uncommon [24]. Use of anticoagulation is debated given increased risk of bleeding, but a short course is advised (Grade C) [22,25,26]. Recurrence of CVST is rare if systemic inflammation is well controlled, and accordingly, long-term anticoagulation is not indicated [24,25].…”

“…Use of anticoagulation is debated given increased risk of bleeding, but a short course is advised (Grade C) [22,25,26]. Recurrence of CVST is rare if systemic inflammation is well controlled, and accordingly, long-term anticoagulation is not indicated [24,25]. Similarly, recurrence of dissection in the setting of vasculitis may be prevented with early pulse steroids [29].…”