Recommendations for the Management of Neuro-Behcet’s Disease By the Japanese National Research Committee for Behcet’s Disease

Hirotoshi Kikuchi¹, Tetsuji Sawada², Masato Okada³, Mitsuhiro Takeno⁴, Masataka Kuwana⁴, Yoshiaki Ishigatsubo⁵, Izumi Kawachi⁶, Hideki Mochizuki⁷, Susumu Kusunoki⁸ and Shunsei Hirohata⁹, ¹Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, Japan, ²Rheumatology, Tokyo Medical University, Shinjuku Tokyo, Japan, ³Immuno-Rheumatology Center, St. Luke’s International Hospital, Tokyo, Japan, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ⁵Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, Yokohama, Japan, ⁶Department of Neurology, Niigata University Medical and Dental Hospital, Niigata, Japan, ⁷Department of Neurology, Osaka University Graduate School of Medicine, Osaka, Japan, ⁸Department of Neurology, Kinki University School of Medicine, Osaka, Japan, ⁹Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Kanagawa, Japan

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Behcet's syndrome, central nervous system involvement, guidelines and management

Session Information

Date: Tuesday, November 7, 2017

Session Title: Vasculitis Poster III: Other Vasculitis Syndromes

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Central nervous system involvement is one of the most serious complications in Behçet’s disease (BD). This condition is referred to as neuro-Behçet’s disease (NB) and can be classified into acute type (ANB) and chronic progressive type (CPNB) based upon differences in the clinical course and responses to corticosteroid treatment. Diagnostic criteria were generated in 2013 based on a multicenter clinical survey performed by the Behçet’s Disease Research Committee of the Ministry of Health, Labor and Welfare of the Japanese Government. Although ‘Guidelines for Treatment of NB’ was also proposed based on the survey, it is still preliminary. The aim of the current study is to develop evidence-based recommendations for the management of NB supplemented by expert opinions where necessary.

Methods: First, clinical questions (CQs) on NB were extracted from a literature search for problem areas and related keywords, and draft CQs and a flow chart were prepared. The expert committee, a task force of the research subcommittee for NB, consisted of 7 board-certified rheumatologists (one was also a board-certified neurologist) and 3 board-certified neurologists. A systematic literature search was performed using Medline and the Japan Medical Abstract Society databases from 1997 to 2016. A total of 15 initial CQs were generated. These yielded the final recommendations developed from 3 blind Delphi rounds, in which the rate of agreement scores on CQs (range 1[strongly disagree]-5[strongly agree]) was determined though voting by the whole committee.

Results: Thirteen recommendations were developed for the management of NB (general 1, ANB 7, CPNB 5). The strength of each recommendation was established based on the evidence level as well as rate of agreement. There was excellent concordance between the level of agreement of rheumatologists and that of neurologists. Based on these recommendations, a flow chart was established for the management for ANB and CPNB (Figure).

Conclusion: The recommendations generated in this study are mainly based not only on expert opinions but on the results of uncontrolled evidence from open trials and retrospective cohort studies. Nevertheless, such guidelines that can be used for international studies would be needed, for which verification by further properly designed controlled clinical trials is required.

Disclosure: H. Kikuchi, Mitsubishi Tanabe Pharma, 2; T. Sawada, Mitsubishi Tanabe Pharma, 2; M. Okada, Mitsubishi Tanabe Pharma, 2; M. Takeno, Mitsubishi Tanabe Pharma, 2; M. Kuwana, Mitsubishi Tanabe Pharma, 2; Y. Ishigatsubo, Mitsubishi Tanabe Pharma, 2; I. Kawachi, None; H. Mochizuki, None; S. Kusunoki, None; S. Hirohata, Mitsubishi Tanabe Pharma, 2.

To cite this abstract in AMA style:

Kikuchi H, Sawada T, Okada M, Takeno M, Kuwana M, Ishigatsubo Y, Kawachi I, Mochizuki H, Kusunoki S, Hirohata S. Recommendations for the Management of Neuro-Behcet’s Disease By the Japanese National Research Committee for Behcet’s Disease [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/recommendations-for-the-management-of-neuro-behcets-disease-by-the-japanese-national-research-committee-for-behcets-disease/. Accessed October 1, 2022.

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