J Rheum Dis.  2024 Jul;31(3):178-181. 10.4078/jrd.2023.0069.

Corticosteroid-free adalimumab-cyclophosphamide combination therapy for acute phase neuro-Behçet’s disease: a case report

• Kim JH
• Chung SW
• Lee YJ

• KMID: 2557406
• DOI: http://doi.org/10.4078/jrd.2023.0069

Abstract

Neuro-Behçet's disease (NBD) represents a significant complication of Behçet's syndrome, potentially leading to elevated mortality and disability rates. The standard treatment for parenchymal NBD typically entails administering high-dose corticosteroids to prompt rapid-onset effects, coupled with immunosuppressants to prevent subsequent relapses. A 48-year-old male with NBD presented with progressively worsening dysarthria over 9 months. This patient experienced increased intraocular pressure while using glucocorticoids, which worsened his pre-existing glaucoma. The patient had a prior diagnosis of NBD and presented with progressive dysarthria over a period of nine months, leading to a brain magnetic resonance imaging (MRI) scan. The brain MRI revealed multifocal punctate high signal intensities in the left frontoparietal area, insula, and basal ganglia. Instead of the standard steroid pulse therapy, the patient received adalimumab-cyclophosphamide combination as an alternative induction therapy. Subsequent serial brain MRI scans exhibited no emergence of new lesions, and the patient remained devoid of clinical relapses even after 17 months from the commencement of induction treatment. Adalimumab-cyclophosphamide combination could be used as a corticosteroid-free induction strategy for NBD. Further investigations are warranted to establish the most suitable combination regimen.

Keyword

Behçet syndrome; Vasculitis; Cyclophosphamide; Adalimumab; Central nervous system

Figure

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  Figure 1 Serial brain magnetic resonance imaging. (A) T2-weighted, fluid-attenuated inversion recovery (FLAIR) images show temporal changes in high signal intensities in the left pre- and post-central gyrus and parietal lobe. (B) Pre-treatment (at 1 month) T1-weighted fat-suppressed contrast-enhanced coronal images show multifocal punctate hyperintense lesions in the left frontoparietal and basal ganglia regions. The intensities disappeared after the adalimumab-cyclophosphamide combination therapy (at 3 and 10 months).

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  Figure 2 The clinical course of the patient. AZA: azathioprine, PDS: prednisolone, MPD: methylprednisolone, ADA: adalimumab, CPM: cyclophosphamide, MMF: mycophenolate mofetil, Lt: left, Rt: right, MRI: magnetic resonance imaging.

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