Use of infliximab and other biologics in <scp>B</scp>ehçet disease

Li, J.; Fernando, SL; Herkes, Geoffrey

doi:10.1111/imj.12297

Cited by 4 publications

(2 citation statements)

References 16 publications

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“…In light of the increasing evidence of elevated levels of proinflammatory cytokines, including TNF-α, in the pathogenesis of BD, these agents have become more popular as they may directly inhibit the development and evolution of the disease. Review of the current literature reveals numerous case reports describing successful use of infliximab in refractory and aggressive cases, maintenance of remission in chronic disease as well as in new-onset NBD, 38,39 often used in combination with a disease-modifying therapy.…”

Section: Treatmentmentioning

confidence: 99%

Neuro-Behçet Disease and Autoinflammatory Disorders

2014

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Misregulation of innate immunity leads to autoinflammation. Behçet disease is an autoinflammatory condition involving recurrent attacks of inflammation in skin, eyes, joints, and even the nervous system. The etiology may involve vascular inflammation. Central nervous system involvement in neuro-Behçet disease (NBD) comes in the form of parenchymal NBD or nonparenchymal NBD. The parenchymal form has a predilection for the brainstem, diencephalon and cerebral hemispheres, and represents a meningoencephalitis thought to be related to small vessel vasculitis. Cerebral venous sinus thrombosis, arising from a vasculitic process of large veins, comprises the majority of vascular NBD cases. The rarer monogenetic autoinflammatory syndromes are characterized by periodic fever, and typically present in the pediatric population. Neurologic involvement in these syndromes typically presents in the form of an aseptic meningitis. Treatment of autoinflammatory disorders involves immune modulation with corticosteroids, disease-modifying antirheumatic medications, and increasingly antibodies targeting cytokines like tumor necrosis factor α and interleukin 1.

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Section: Treatmentmentioning

confidence: 99%

Neuro-Behçet Disease and Autoinflammatory Disorders

2014

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“…As its clinical course is heterogeneous, pharmacotherapy is variable depending on the organ involvement and severity of the disease (2). Treatment is based largely on anecdotal case reports, case series, and scarce randomized clinical trials (3,4).…”

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confidence: 99%

An Incomplete Form of Childhood Behçet's Disease Treated with Infliximab

Caporuscio

Pranteda

Nisticò

et al. 2014

Int J Immunopathol Pharmacol

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Behçet's disease (BD) is a multi-systemic vasculitis characterized by the possible presence of cutaneous, ocular, articular and neurological manifestations. In this report, we examine the case of a fifteen-year old boy with an incomplete form of juvenile Behçet's disease which began with joint involvement and developed into a complete form only after several years. The patient showed a rapid response to anti-TNF-alpha (infliximab) with an improvement of mucocutaneous lesions (oral and genital ulcers, pseudofolliculitis) and arthritis.

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Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

Kidd

2023

Pract Neurol

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Neurological involvement in Behçet’s syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur. Psychiatric syndromes may arise with brain and brainstem involvement, and cognitive disorders relate to the brain disease, to circulating inflammatory factors, and to fatigue and despondency. Eighty per cent of cases begin with a relapsing disease course, of whom 70% have only one attack, and 30% have a progressive disease course either from onset or following an initially relapsing course. Venous thrombosis leading to intracranial hypertension and cerebral venous infarction is less common and caused by inflammation in affected veins and a circulating prothrombotic state. Arterial involvement is rare and relates to an arteritis affecting large-sized and medium-sized vessels within the brain leading to infarction, subarachnoid and parenchymal haemorrhage, aneurysm formation and arterial dissection. There is a newly recognised disorder of cerebral cortical hypoperfusion. Cranial neuropathy, peripheral neuropathy and myositis are rare. There has been significant progress in understanding the pathophysiology and treatment of the systemic disease, leading to improved outcomes, but there has been no randomised trial of treatment in the neurological disorder.

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Use of infliximab and other biologics in Behçet disease

Cited by 4 publications

References 16 publications

Neuro-Behçet Disease and Autoinflammatory Disorders

Neuro-Behçet Disease and Autoinflammatory Disorders

An Incomplete Form of Childhood Behçet's Disease Treated with Infliximab

Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

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