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Characteristics and phenotype heterogeneity in late-onset Behçet’s syndrome: a cohort from a referral center in China

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Abstract

Objectives

To evaluate the demographics, clinical aspects, and major organ involvement of patients with late-onset Behçet’s syndrome (BS) in a tertiary center in China.

Methods

We conducted a cross-sectional study of consecutive BS patients in Huadong Hospital of Fudan University from September 2012 to January 2020. We compared clinical variables between patients with disease onset before and after 40 years of age. The relative risks (RRs) of clinical variables were calculated between the two age groups. Moreover, a hierarchical cluster analysis was conducted according to 29 variables to determine homogeneous subgroups in patients with late-onset BS.

Results

We enrolled 152 late-onset BS patients, with a median age at onset of 47 years (interquartile range, IQR: 43–52 years). There is a higher prevalence of intestinal ulcers in late-onset BS than in early-onset BS (RR 1.47), but a lower prevalence of ocular involvements (RR 0.54) and folliculitis (RR 0.46). Female sex was associated with genital ulcers, erythema nodosum, and arthritis. Four clusters (C1–C4) were formed. C1 (n = 71), the largest cluster, was defined as the mucocutaneous group, C2 (n = 20) as the arthritis group, C3 (n = 39) as the gastrointestinal group, in which all patients presented with intestinal lesions, and five cases with esophageal ulcers. In C4 (n = 22), showing a mixture of uveitis and vascular lesions, 15 patients presented with uveitis and 8 had vascular lesions, and 1 case had central nervous system lesions.

Conclusion

Four phenotype clusters were identified. Patients with skin lesions comprised the largest cluster, while gastrointestinal, panuveitis, and cardiovascular clusters are the most commonly involved organs in late-onset BS patients.

Key Points

• Our analysis demonstrated the phenotype discrepancy between early and late onset groups.

• Four phenotype clusters were identified, with gastrointestinal, panuveitis and cardiovascular clusters representing commonly involved organs.

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Data availability

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Abbreviations

BS:

Behçet’s syndrome

CHN:

the Chinese version of Behçet’s disease criteria created by Cheng and Zhang

CNS:

central neurological involvement

CT:

computed tomography

GIBS:

gastrointestinal Behçet’s syndrome

ICBD:

International Criteria for BS

IQR:

Interquartile range

ISG:

International Study Group criteria

JPN:

Diagnostic criteria of the Behçet’s disease research committee of Japan (1987 revision)

MDS:

myelodysplastic syndrome

RR:

relative risk

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Funding

This work is supported by the National Natural Science Foundation of China (81871276) and the Clinical Science Innovation Program of Shenkang Hospital Development Center (SHDC12017129).

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Authors and Affiliations

  1. Department of Rheumatology and Immunology, Shanghai Key Laboratory of Clinical Geriatric Medicine, Huadong Hospital, Research Center on Aging and Medicine, Huadong Hospital, Fudan University, No. 221 west Yan’an Road Shanghai, 200040, Shanghai, People’s Republic of China

    Jun Zou, Dan Luo, Yan Shen & Jian-long Guan

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  1. Jun Zou
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  2. Dan Luo
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  3. Yan Shen
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  4. Jian-long Guan
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Contributions

J-LG designed the study and reviewed the manuscript. D L and Y S carried out the data collection. J Z analyzed the data, wrote the manuscript. J-F L carried out the data analysis. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Jian-long Guan.

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Ethics approval and consent to participate

The current study was approved by the Ethics Committee of Huadong Hospital, Fudan University, and was conducted under strict accordance with the Declaration of Helsinki, Good Clinical Practice guidelines, and all applicable laws and regulations in China.

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All subjects gave consent to participate in the study.

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The authors declare that they have no competing interest.

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Zou, J., Luo, D., Shen, Y. et al. Characteristics and phenotype heterogeneity in late-onset Behçet’s syndrome: a cohort from a referral center in China. Clin Rheumatol 40, 2319–2326 (2021). https://doi.org/10.1007/s10067-020-05536-z

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