Skip to main content
SpringerLink
Log in

Enthesitis as an initial presentation of vascular Behçet’s syndrome: a case-based review

  • Case Based Review
  • Published:
Rheumatology International Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

Data availability

The data underlying this article will be shared upon reasonable request from the corresponding author.

References

  1. Yazici H, Seyahi E, Hatemi G, Yazici Y (2018) Behçet syndrome: a contemporary view. Nat Rev Rheumatol 14:107–119. https://doi.org/10.1038/nrrheum.2017.208

    Article  CAS  PubMed  Google Scholar 

  2. Bettiol A, Prisco D, Emmi G (2020) Behçet: the syndrome. Rheumatol (United Kingdom) 59:iii101–iii107. https://doi.org/10.1093/rheumatology/kez626

    Article  Google Scholar 

  3. Yazici H, Ugurlu S, Seyahi E (2012) Behçet syndrome: is it one condition? Clin Rev Allergy Immunol 43:275–280. https://doi.org/10.1007/s12016-012-8319-x

    Article  CAS  PubMed  Google Scholar 

  4. Diri E, Mat C, Hamuryudan V et al (2001) Papulopustular skin lesions are seen more frequently in patients with Behçet’s syndrome who have arthritis: a controlled and masked study. Ann Rheum Dis 60:1074–1076. https://doi.org/10.1136/ard.60.11.1074

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Hatemi G, Bahar H, Uysal S et al (2004) The pustular skin lesions in Behçet’s syndrome are not sterile. Ann Rheum Dis 63:1450. https://doi.org/10.1136/ard.2003.017467

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Çalgüneri M, Kiraz S, Ertenli I et al (1996) The effect of prophylactic penicillin treatment on the course of arthritis episodes in patients with Behçet’s disease: a randomized clinical trial. Arthritis Rheum 39:2062–2065. https://doi.org/10.1002/art.1780391216

    Article  PubMed  Google Scholar 

  7. Çalgüneri M, Ertenli I, Kim S et al (1996) Effect of prophylactic benzathine penicillin on mucocutaneous symptoms of behçet’s disease. Dermatology 192:125–128. https://doi.org/10.1159/000246336

    Article  PubMed  Google Scholar 

  8. Hatemi G, Fresko I, Tascilar K, Yazici H (2008) Increased enthesopathy among Behçet’s syndrome patients with acne and arthritis: an ultrasonography study. Arthritis Rheum 58:1539–1545. https://doi.org/10.1002/art.23450

    Article  PubMed  Google Scholar 

  9. Bekaryssova D, Joshi M, Gupta L et al (2022) Knowledge and perceptions of reactive arthritis diagnosis and management among healthcare workers during the COVID-19 pandemic: online survey. J Korean Med Sci 37:e355. https://doi.org/10.3346/jkms.2022.37.e355

    Article  PubMed  PubMed Central  Google Scholar 

  10. Baker H, Amaral JK, Schoen RT (2024) Management of postinfectious inflammatory arthritis. Curr Opin Rheumatol https://doi.org/10.1097/BOR.0000000000001009

    Article  PubMed  Google Scholar 

  11. Davatchi F, Assaad-Khalil S, Calamia KT et al (2014) The international criteria for Behçet’s disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 28:338–347. https://doi.org/10.1111/jdv.12107

    Article  Google Scholar 

  12. Hatemi G, Christensen R, Bang D et al (2018) 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis 77:808–818. https://doi.org/10.1136/annrheumdis-2018-213225

    Article  PubMed  Google Scholar 

  13. Caporn N, Higgs ER, Dieppe PA, Watt L (1983) Arthritis in Behcet’s syndrome. Br J Radiol 56:87–91. https://doi.org/10.1259/0007-1285-56-662-87

    Article  CAS  PubMed  Google Scholar 

  14. Olivieri I, Gemignani G, Braccini G, Pasero G (1990) Behçet’s syndrome and spondyloarthritis. Br J Rheumatol 29:409–410. https://doi.org/10.1093/rheumatology/29.5.409

    Article  CAS  PubMed  Google Scholar 

  15. Chang HK, Lee DH, Jung SM et al (2002) The comparison between Behçet’s disease and spondyloarthritides: does Behçet’s disease belong to the spondyloarthropathy complex? J Korean Med Sci 17:524–529. https://doi.org/10.3346/jkms.2002.17.4.524

    Article  PubMed  PubMed Central  Google Scholar 

  16. Düzgün N, Ateş A (2003) Erosive arthritis in a patient with Behçet’s disease. Rheumatol Int 23:265–267. https://doi.org/10.1007/s00296-003-0302-7

    Article  PubMed  Google Scholar 

  17. Takahashi A, Takeda I, Kanno T et al (2004) Achilles tendonitis in a patient with Behçet’s disease. Mod Rheumatol 14:485–487. https://doi.org/10.1007/s10165-004-0344-z

    Article  PubMed  Google Scholar 

  18. Ozkan F, Cetin GY, Bakan B et al (2012) Sonographic evaluation of subclinical entheseal involvement in patients with Behçet disease. Am J Roentgenol 199:W723–W729. https://doi.org/10.2214/AJR.12.8576

    Article  Google Scholar 

  19. Elbarky EM, Hussien MI, Elgazzar NM et al (2021) Serum growth differentiation factor-15 (GDF-15) level in Behcet’s disease patients: relation to clinical characteristics, musculoskeletal ultrasound findings and disease activity. Egypt Rheumatologist 43:261–266. https://doi.org/10.1016/j.ejr.2021.05.002

    Article  CAS  Google Scholar 

  20. Topaloğlu M, Kaymaz S, Karasu U et al (2021) Magnetic resonance evaluation of sacroiliac joint involvement in Behçet’s disease: a cross-sectional study. Ulusal Romatoloji Dergisi 13:12–17. https://doi.org/10.4274/raed.galenos.2021.07830

    Article  Google Scholar 

  21. Hanai S, Ogawa Y, Honobe-Tabuchi A et al (2023) Unusual enthesitis in a patient with Behçet’s syndrome. Clin Rheumatol 42:1721–1722. https://doi.org/10.1007/s10067-023-06549-0

    Article  PubMed  Google Scholar 

  22. Göğebakan H, Akbaş N (2023) The correlation with enthesitis and the severity of the disease in Behçet’s syndrome. Arch Basic Clin Res 5(1):171–177. https://doi.org/10.5152/ABCR.2023.224066

    Article  Google Scholar 

  23. Tascilar K, Melikoglu M, Ugurlu S et al (2014) Vascular involvement in behceţ’s syndrome: aretrospective analysis of associations and the time course. Rheumatol (United Kingdom) 53:2018–2021. https://doi.org/10.1093/rheumatology/keu233

    Article  CAS  Google Scholar 

  24. Bettiol A, Alibaz-Oner F, Direskeneli H et al (2023) Vascular Behçet syndrome: from pathogenesis to treatment. Nat Rev Rheumatol 19:111–126. https://doi.org/10.1038/s41584-022-00880-7

    Article  CAS  PubMed  Google Scholar 

  25. Saadoun D, Wechsler B, Desseaux K et al (2010) Mortality in Behçet’s disease. Arthritis Rheum 62:2806–2812. https://doi.org/10.1002/art.27568

    Article  CAS  PubMed  Google Scholar 

  26. Schett G, Lories RJ, D’Agostino MA et al (2017) Enthesitis: from pathophysiology to treatment. Nat Rev Rheumatol 13:731–741. https://doi.org/10.1038/nrrheum.2017.188

    Article  CAS  PubMed  Google Scholar 

  27. Dougados M, Baeten D (2011) Spondyloarthritis. Lancet 377:2127–2137. https://doi.org/10.1016/S0140-6736(11)60071-8

    Article  PubMed  Google Scholar 

  28. Moll JMH, Haslock I, Macrae IF, Wright V (1974) Associations between ankylosing spondylitis, psoriatic arthritis, reiter’s disease, the intestinal arthropathies, and Behcet’s syndrome. Med (United States) 53:343–364. https://doi.org/10.1097/00005792-197409000-00002

    Article  CAS  Google Scholar 

  29. McGonagle D, Aydin SZ, Gül A et al (2015) MHC-I-opathy’-unified concept for spondyloarthritis and Behçet disease. Nat Rev Rheumatol 11:731–740. https://doi.org/10.1038/nrrheum.2015.147

    Article  CAS  PubMed  Google Scholar 

  30. Syrbe U, Baraliakos X (2018) Spondylarthritiden [Spondyloarthritis]. Z Rheumatol 77:783–788. https://doi.org/10.1007/s00393-018-0475-9

    Article  CAS  PubMed  Google Scholar 

  31. van der Houwen TB, van Hagen PM, van Laar JAM (2022) Immunopathogenesis of Behçet’s disease and treatment modalities. Semin Arthritis Rheum 52:151956. https://doi.org/10.1016/j.semarthrit.2022.151956

    Article  CAS  PubMed  Google Scholar 

  32. İlgen U (2022) Clusters in Behçet’s syndrome. Arthritis Res Ther 24:242. https://doi.org/10.1186/s13075-022-02937-0

    Article  PubMed  PubMed Central  Google Scholar 

  33. Wu X, Wu J, Li X et al (2021) The clinical characteristics of other HLA-B types in Chinese ankylosing spondylitis patients. Front Med (Lausanne) 7:568790. https://doi.org/10.3389/fmed.2020.568790

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

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.

Funding

This work was partly supported by JSPS KAKENHI Grant Number 21K08469T and a JCR Grant for Promoting Research for D2T RA.

Author information

Authors and Affiliations

  1. Department of Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Miyagi, Japan

    Isso Saito, Tsuyoshi Shirai, Hiroko Sato, Tomonori Ishii & Hiroshi Fujii

Authors
  1. Isso Saito
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tsuyoshi Shirai
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Hiroko Sato
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Tomonori Ishii
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hiroshi Fujii
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

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.

Corresponding author

Correspondence to Tsuyoshi Shirai.

Ethics declarations

Ethics approval

The study protocol complied with the principles of the Declaration of Helsinki and was approved by the Ethics Committee of Tohoku University Graduate School of Medicine (reference number:2022-1-775).

Compliance with ethical standards

Written informed consent was obtained from the patient for the publication of this case report.

Conflict of interest

The authors declare that they have no conflicts of interest.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Supplementary Material 1

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00296-024-05607-3

Keywords

Search

Navigation