Behçet’s Disease and Nervous System Involvement

Kürtüncü, Murat; Tüzün, Erdem; Akman‐Demir, Gülşen

doi:10.1007/s11940-016-0405-6

Cited by 22 publications

(8 citation statements)

References 76 publications

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“…CYC (1 g/month for 6-12 months or 0.8 g/m 2 ) has been associated also with some benefits in parenchymal neuro-BS (79,98). In a French study on 115 patients with parenchymal neuro-BS, the use of CYC (n = 53) resulted as effective as AZA (n = 40) and steroids alone (n = 19) in preventing relapses (48).…”

Section: Third-line Treatmentsmentioning

confidence: 99%

Treating the Different Phenotypes of Behçet's Syndrome

et al. 2019

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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.

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Section: Third-line Treatmentsmentioning

confidence: 99%

Treating the Different Phenotypes of Behçet's Syndrome

et al. 2019

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“…Acute attacks are usually treated with high-dose intravenous corticosteroids followed by maintenance treatment with oral steroids for 6-12 months. Relapses can be prevented by using an immunosuppressant such as azathioprine, mycophenolate mofetil or even cyclophosphamide if the disease is refractory to the other drugs [15] . Our patient probably had BD for many years but was not diagnosed.…”

Section: Discussionmentioning

confidence: 99%

Wrist Drop as a Manifestation of Behçet's Disease

Daher

Haddad

2017

European Journal of Case Reports in Internal Medicine

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“…16,92,98,99 The anti-IL-6 agent tocilizumab currently represents a valid therapeutic option for refractory BD, with a significant effect in patients with uveitis and promising effects on CNS involvement. 93,100,101 However, it is known that this drug has scarce efficacy on mucocutaneous manifestations of BD, 102 and that cases of drug-related cutaneous flares are reported. 103,104 The recognized crucial role of the Th17-mediated immune response led to the introduction in the BD therapy of the anti-IL-17 drug secukinumab, whose efficacy has been demonstrated in preliminary studies for the treatment of mucocutaneous and articular manifestations, while its role in the management of uveitis is controversial.…”

Section: Biologic Drugs and New Perspectivesmentioning

confidence: 99%

<p>Behçet’s Disease in Children: Diagnostic and Management Challenges</p>

Costagliola

Cappelli

Consolini

2020

TCRM

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Behçet's Disease (BD) is an inflammatory disease of unknown etiology with multisystemic involvement, being the main clinical manifestations represented by recurrent oral and genital ulcerations and uveitis. The disease has typically a chronic-relapsing course and may cause significant morbidity and mortality due to eye, vascular and neurological involvement. Although BD is more frequently diagnosed in adulthood, the disease onset can also be in pediatric age. Pediatric-onset BD is commonly featured by an incomplete clinical picture, and therefore the diagnosis represents a considerable clinical challenge for the physicians. The first classification criteria for pediatric BD, based on a scoring system, have been proposed few years ago. This work focuses on the main difficulties concerning both the diagnostic approach and the treatment of BD in pediatric age. The recommendation for the treatment of pediatric BD has been recently updated and allowed a considerable improvement of the therapeutic strategies. In particular, the use of anti-TNFα drugs as a second-line option for refractory BD, and as a firstline treatment in severe ocular and neurological involvement, has demonstrated to be effective in improving the outcome of BD patients. The knowledge about the molecular pathogenesis is progressively increasing, showing that BD shares common features with autoimmune and autoinflammatory disorders, and thus leading to the use of new biologic agents targeting the main mediators involved in the determination of BD. Anti-IL-17, anti-IL-23, anti-IL-1 and anti-IL-6 agents have shown promising results for the treatment of refractory BD in clinical trials and will represent an important alternative for the therapeutic approach to the disease.

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Behçet’s Disease and Nervous System Involvement

Cited by 22 publications

References 76 publications

Treating the Different Phenotypes of Behçet's Syndrome

Treating the Different Phenotypes of Behçet's Syndrome

Wrist Drop as a Manifestation of Behçet's Disease

<p>Behçet’s Disease in Children: Diagnostic and Management Challenges</p>

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