Abstract
Enthesitis is a characteristic manifestation of spondyloarthropathy (SpA). Historically, Behçet’s syndrome (BS) was classified within SpA. Although they are now classified separately, the association between BS and SpA remains controversial. The concept of MHC-I (major histocompatibility complex class I)-opathy has been proposed based on the overlap in immunopathological mechanisms among diseases associated with human leukocyte antigen (HLA) class I. Enthesitis is a frequent complication in patients with BS who also have acne and arthritis. However, information regarding enthesitis in patients with BS without arthritis (BS-WA) is limited. Herein, we report a case of vascular BS complicated by enthesitis. In this case, heel pain was the dominant symptom at presentation. Laboratory tests revealed chlamydia antibody positivity, leading to a tentative diagnosis of reactive arthritis. Despite treatment, C-reactive protein (CRP) levels remained elevated. Imaging revealed numerous aneurysmal lesions in the large vessels. Based on these findings and other symptoms, patient was diagnosed with vascular BS. He tested positive for HLA-B15 and HLA-B46, which are associated with peripheral SpA. Subsequent remission induction therapy for BS was effective and the patient was discharged without complications. Our case and a literature review suggest that there exists a subgroup of BS-WA with a complication of enthesitis, possibly belonging to the spectrum of MHC-I-opathies. It is important to consider BS as a differential diagnosis in patients presenting with enthesitis and to conduct a precise medical history review regarding the symptoms of BS.
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The authors thank the staff of the Department of Rheumatology, Tohoku University Hospital, for their helpful discussions. We also thank Editage for professional English editing.
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This work was partly supported by JSPS KAKENHI Grant Number 21K08469T and a JCR Grant for Promoting Research for D2T RA.
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Concept and design: IS and TS. Acquisition, analysis, or interpretation of data: All authors. Manuscript drafting: IS. Critical revision of the manuscript for intellectual content: All authors. Statistical analysis: All authors. Obtained funding: TS. All the authors contributed to the manuscript and approved the submitted version. All authors take full responsibility for the integrity and accuracy of all aspects of the work.
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Saito, I., Shirai, T., Sato, H. et al. Enthesitis as an initial presentation of vascular Behçet’s syndrome: a case-based review. Rheumatol Int (2024). https://doi.org/10.1007/s00296-024-05607-3
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DOI: https://doi.org/10.1007/s00296-024-05607-3