Case Report: Repeated Low-Dose Rituximab Treatment Is Effective in Relapsing Neuro Behçet's Disease

Zhao, Chao; Li, Chuan; Duan, Feng-ju; Yan, Qi; Zhang, Zhuo; Du, Ying; Zhang, Wei

doi:10.3389/fneur.2021.595984

Cited by 10 publications

(5 citation statements)

References 27 publications

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“…Anti-CD20 significantly reduced the number of gadolinium-enhancing lesions, the number of relapses in RRMS patients [16] and led to the expansion of a rare regulatory B cell population [63]. Interestingly, rituximab was also reported as efficient in severe ocular manifestations of Behçet's disease [64] and two cases of neuro-Behçetpatients presenting pseudotumoral lesions and one with relapsing NBD [65,66]. No studies exploring the underlying causes of rituximab efficiency in these rare cases of NBD were performed.…”

Section: Discussionmentioning

confidence: 99%

B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease

Maghrebi

Belghith

Jeridi

et al. 2022

Cells

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Remitting-RelapsingMultiple Sclerosis (RRMS) and Neuro-Behçet Disease (NBD) are two chronic neuroinflammatory disorders leading to neurological damage. Herein, we investigated in these patients the IL-10-producing cells during the early stages of these disorders. Cellular and molecular investigations were carried out on treatment naive patients suffering from RRMS and NBD recruited at the first episode of clinical relapse. Our findings demonstrate that CSF-B cells from NBD patients, but not RRMS, are the major source of intrathecal IL-10 as compared to T-CD4 cells. Moreover, we showed a lower expression of TGF-β and IL35, in the CSF cells of NBD patients as compared to the control group. Specific in vitro CpG stimulation of peripheral blood B cells from NBD patients resulted in a concomitant early mRNA expression of IL6 and IL10 but was limited to IL10 for RRMS patients. Furthermore, mRNA expression of IL-6 and IL-10 receptors was assessed and intriguingly IL6ST receptor subunit was significantly lower in NBD CSF, but not RRMS while IL10RB was increased in both. Deciphering the role of increased IL-10-producing B cells and IL10RB despite relapsing disease as well as the discordant expression of IL6 and IL6ST may pave the way for a better understanding of the pathophysiology of these neuro-inflammatory disorders.

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Section: Discussionmentioning

confidence: 99%

B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease

Maghrebi

Belghith

Jeridi

et al. 2022

Cells

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“…The approach is different and variable depending on the course of the disease. In the treatment of neuro-Behçet's disease, glucocorticoids are used initially and during further treatment, depending on the course of the disease, as well as azathioprine, methotrexate, cyclophosphamide, TNF-alpha inhibitors and rituximab (9). Therapeutic success is varied in terms of improvement of clinical condition, remission and disease recurrence.…”

Section: Prikaz Bolesnikamentioning

confidence: 99%

“…pristup je različit i varijabilan ovisno o tijeku bolesti. Za neuro oblik Behçetove bolesti koriste se glukokortikoidi u početku i tijekom daljnjeg liječenja, ovisno o tijeku bolesti, te azatioprin, metotreksat, ciklofosfamid, TNF alfa inhibitori i rituksimab (9). Terapijski uspjesi su različiti u pogledu kliničkog poboljšanja, remisije i recidiva bolesti.…”

Section: Introductionunclassified

CHALLENGES IN THE TREATMENT OF NEURO-BEHÇET’S DISEASE — a case report

2023

Reumatizam

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Behçet's disease is a multisystem disease of insufficiently known etiology, with genetics playing a significant role in its development, featuring an increased frequency of the HLA-B51 gene in the genome of patients, but it can sometimes also be associated with the presence of certain strains of bacteria. pathogenetically, this disease is considered to be small-vessel vasculitis. The disease most often manifests itself in oral and genital ulcerations with frequent uveitis and skin changes, and, in very rare cases, there are neurological manifestations, which occur in less than 10 percent of patients that suffer from this disease, and in that case we call it the neuro-Behçet's disease. The treatment of this disease depends on the clinical condition and the treatment approach is individual for each patient. The greatest challenge is the treatment of neuro-Behçet's disease due to the severity of the clinical condition and frequent inadequate response to therapy. Neuro-Behçet's disease is treated with corticosteroids, various immunosuppressants and biological therapy -e.g., TNF-alpha inhibitors as the latest form of therapy with the best results. This paper intends to present the case of a patient with neurological manifestations of the disease, the challenges that we faced during the treatment, the complications that occurred, and the resulting changes in therapy that were sometimes necessary.

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“…It is possible that, in addition to IL-10, B cells present in the CSF produce other cytokines that play a proinflammatory role, i.e., TNF-α, IL-6, and GM-CSF. Remarkably, B cell depletion therapy was reported to be effective in severe ocular manifestations of Behçet’s disease [ 52 ], in two cases of neuro-Behçet disease presenting pseudo-tumoral lesions [ 53 ], and in one case with relapsing NBD [ 54 ]. It is tempting to speculate that B cells represent a promising therapeutic target in patients with NBD.…”

Section: Potential Therapeutic Avenues For Autoinflammatory Disordersmentioning

confidence: 99%

B Cells at the Cross-Roads of Autoimmune Diseases and Auto-Inflammatory Syndromes

Zouali

2022

Cells

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Whereas autoimmune diseases are mediated primarily by T and B cells, auto-inflammatory syndromes (AIFS) involve natural killer cells, macrophages, mast cells, dendritic cells, different granulocyte subsets and complement components. In contrast to autoimmune diseases, the immune response of patients with AIFS is not associated with a breakdown of immune tolerance to self-antigens. Focusing on B lymphocyte subsets, this article offers a fresh perspective on the multiple cross-talks between both branches of innate and adaptive immunity in mounting coordinated signals that lead to AIFS. By virtue of their potential to play a role in adaptive immunity and to exert innate-like functions, B cells can be involved in both promoting inflammation and mitigating auto-inflammation in disorders that include mevalonate kinase deficiency syndrome, Kawasaki syndrome, inflammatory bone disorders, Schnitzler syndrome, Neuro-Behçet’s disease, and neuromyelitis optica spectrum disorder. Since there is a significant overlap between the pathogenic trajectories that culminate in autoimmune diseases, or AIFS, a more detailed understanding of their respective roles in the development of inflammation could lead to designing novel therapeutic avenues.

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Case Report: Repeated Low-Dose Rituximab Treatment Is Effective in Relapsing Neuro Behçet's Disease

Cited by 10 publications

References 27 publications

B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease

B Cells Specific CpG Induces High IL-10 and IL-6 Expression In Vitro in Neuro-Behçet’s Disease

CHALLENGES IN THE TREATMENT OF NEURO-BEHÇET’S DISEASE — a case report

B Cells at the Cross-Roads of Autoimmune Diseases and Auto-Inflammatory Syndromes

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