On-line First

J Rheum Dis

Published online February 2, 2024

© Korean College of Rheumatology

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

Ji Hyoun Kim, M.D.1 , Sang Wan Chung, M.D., Ph.D.2 , Yun Jong Lee, M.D., Ph.D.3,4

1Division of Rheumatology, Department of Internal Medicine, Chungbuk National University Hospital, Cheongju, 2Division of Rheumatology, Department of Internal Medicine, School of Medicine, Kyung Hee University, Seoul, 3Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, 4Department of Medical Device Development, Seoul National University Graduate School, Seongnam, Korea

Correspondence to : Yun Jong Lee, https://orcid.org/0000-0001-7615-8611
Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, 82 Gumi-ro, 173beon-gil, Bundang-gu, Seongnam 13620, Korea. E-mail: yn35@snu.ac.kr

Received: October 13, 2023; Revised: December 15, 2023; Accepted: December 26, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords Behçet syndrome, Vasculitis, Cyclophosphamide, Adalimumab, Central nervous system

Article

On-line First

J Rheum Dis

Published online February 2, 2024

Copyright © Korean College of Rheumatology.

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

Ji Hyoun Kim, M.D.1 , Sang Wan Chung, M.D., Ph.D.2 , Yun Jong Lee, M.D., Ph.D.3,4

1Division of Rheumatology, Department of Internal Medicine, Chungbuk National University Hospital, Cheongju, 2Division of Rheumatology, Department of Internal Medicine, School of Medicine, Kyung Hee University, Seoul, 3Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, 4Department of Medical Device Development, Seoul National University Graduate School, Seongnam, Korea

Correspondence to:Yun Jong Lee, https://orcid.org/0000-0001-7615-8611
Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, 82 Gumi-ro, 173beon-gil, Bundang-gu, Seongnam 13620, Korea. E-mail: yn35@snu.ac.kr

Received: October 13, 2023; Revised: December 15, 2023; Accepted: December 26, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Behç,et syndrome, Vasculitis, Cyclophosphamide, Adalimumab, Central nervous system

JRD
Apr 01, 2024 Vol.31 No.2, pp. 108~96
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Schematic diagram for mechanism of botanical drugs and ingredients for health functional food for treatment of OA. OA: osteoarthritis, IL: interleukin, TNF-α: tumor necrosis factor-alpha, PGE2: prostaglandin E2, MMP: matrix metalloproteinase, VAS: visual analogue scale. (J Rheum Dis 2024;31:68-78)

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