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When it looks like Behçet's syndrome but is something else

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When it looks like Behçet's syndrome but is something else. / Lötscher, Fabian; Kerstens, F; Krusche, M; Ruffer, N; Kötter, I; Turkstra, F; EULAR studygroup MHC-I-opathies.

In: RHEUMATOLOGY, Vol. 62, No. 11, 02.11.2023, p. 3654-3661.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Lötscher, F, Kerstens, F, Krusche, M, Ruffer, N, Kötter, I, Turkstra, F & EULAR studygroup MHC-I-opathies 2023, 'When it looks like Behçet's syndrome but is something else', RHEUMATOLOGY, vol. 62, no. 11, pp. 3654-3661. https://doi.org/10.1093/rheumatology/kead101

APA

Lötscher, F., Kerstens, F., Krusche, M., Ruffer, N., Kötter, I., Turkstra, F., & EULAR studygroup MHC-I-opathies (2023). When it looks like Behçet's syndrome but is something else. RHEUMATOLOGY, 62(11), 3654-3661. https://doi.org/10.1093/rheumatology/kead101

Vancouver

Lötscher F, Kerstens F, Krusche M, Ruffer N, Kötter I, Turkstra F et al. When it looks like Behçet's syndrome but is something else. RHEUMATOLOGY. 2023 Nov 2;62(11):3654-3661. https://doi.org/10.1093/rheumatology/kead101

Bibtex

@article{4fb6258241d34463841cb41f6bc587b1,
title = "When it looks like Beh{\c c}et's syndrome but is something else",
abstract = "OBJECTIVE: To investigate the differential diagnostic spectrum in patients with suspected Beh{\c c}et's syndrome (BS) in low prevalence regions. In addition, the number of patients fulfilling the ICBD criteria despite not having BS was evaluated.METHODS: This retrospective analysis was performed in two referral centres for BS. Patients with confirmed BS (clinical diagnosis with fulfilment of ISG criteria or a score of ≥5 points in the ICBD criteria) were excluded. The remaining patients were divided into 11 differential diagnosis categories. If no definitive alternative diagnosis could be established, patients were termed 'probable BS' in case of (i) relapsing orogenital aphthosis in the absence of other causes and either HLA-B51 positivity, or origin from an endemic area or presence of an additional typical BS symptom that is not part of the classification criteria, or (ii) with 3-4 points scored in the ICBD criteria.RESULTS: In total 202 patients were included and categorized as follows: 58 patients (28.7%) as 'probable BS', 57 (28.2%) skin disease, 26 (12.9%) chronic pain syndrome, 14 (6.9%) eye disease, 11 (5.4%) spondyloarthropathy, 9 (4.5%) gastrointestinal disease, 7 (3.5%) neurological disease, 4 (2%) arthritis, 3 (1.5%) auto-inflammation, 3 (1.5%) connective tissue disease and 10 (5.0%) miscellaneous disease. HLA-B51 was positive in 55/132 (41.7%); 75/202 (37.1%) of the patients fulfilled the ICBD criteria.CONCLUSION: In a low disease prevalence setting, the straightforward application of the ICBD criteria may lead to overdiagnosis of BS. The differential diagnosis of BS is enormously broad. Clinicians should be aware that HLA-B51 positivity is still not considered as a diagnostic feature in BS.",
keywords = "Behcet Syndrome/diagnosis, Diagnosis, Differential, HLA-B51 Antigen, Humans, Retrospective Studies, Stomatitis, Aphthous",
author = "Fabian L{\"o}tscher and F Kerstens and M Krusche and N Ruffer and I K{\"o}tter and F Turkstra and {EULAR studygroup MHC-I-opathies}",
year = "2023",
month = nov,
day = "2",
doi = "10.1093/rheumatology/kead101",
language = "English",
volume = "62",
pages = "3654--3661",
journal = "RHEUMATOLOGY",
issn = "1462-0324",
publisher = "Oxford University Press",
number = "11",

}

RIS

TY - JOUR

T1 - When it looks like Behçet's syndrome but is something else

AU - Lötscher, Fabian

AU - Kerstens, F

AU - Krusche, M

AU - Ruffer, N

AU - Kötter, I

AU - Turkstra, F

AU - EULAR studygroup MHC-I-opathies

PY - 2023/11/2

Y1 - 2023/11/2

N2 - OBJECTIVE: To investigate the differential diagnostic spectrum in patients with suspected Behçet's syndrome (BS) in low prevalence regions. In addition, the number of patients fulfilling the ICBD criteria despite not having BS was evaluated.METHODS: This retrospective analysis was performed in two referral centres for BS. Patients with confirmed BS (clinical diagnosis with fulfilment of ISG criteria or a score of ≥5 points in the ICBD criteria) were excluded. The remaining patients were divided into 11 differential diagnosis categories. If no definitive alternative diagnosis could be established, patients were termed 'probable BS' in case of (i) relapsing orogenital aphthosis in the absence of other causes and either HLA-B51 positivity, or origin from an endemic area or presence of an additional typical BS symptom that is not part of the classification criteria, or (ii) with 3-4 points scored in the ICBD criteria.RESULTS: In total 202 patients were included and categorized as follows: 58 patients (28.7%) as 'probable BS', 57 (28.2%) skin disease, 26 (12.9%) chronic pain syndrome, 14 (6.9%) eye disease, 11 (5.4%) spondyloarthropathy, 9 (4.5%) gastrointestinal disease, 7 (3.5%) neurological disease, 4 (2%) arthritis, 3 (1.5%) auto-inflammation, 3 (1.5%) connective tissue disease and 10 (5.0%) miscellaneous disease. HLA-B51 was positive in 55/132 (41.7%); 75/202 (37.1%) of the patients fulfilled the ICBD criteria.CONCLUSION: In a low disease prevalence setting, the straightforward application of the ICBD criteria may lead to overdiagnosis of BS. The differential diagnosis of BS is enormously broad. Clinicians should be aware that HLA-B51 positivity is still not considered as a diagnostic feature in BS.

AB - OBJECTIVE: To investigate the differential diagnostic spectrum in patients with suspected Behçet's syndrome (BS) in low prevalence regions. In addition, the number of patients fulfilling the ICBD criteria despite not having BS was evaluated.METHODS: This retrospective analysis was performed in two referral centres for BS. Patients with confirmed BS (clinical diagnosis with fulfilment of ISG criteria or a score of ≥5 points in the ICBD criteria) were excluded. The remaining patients were divided into 11 differential diagnosis categories. If no definitive alternative diagnosis could be established, patients were termed 'probable BS' in case of (i) relapsing orogenital aphthosis in the absence of other causes and either HLA-B51 positivity, or origin from an endemic area or presence of an additional typical BS symptom that is not part of the classification criteria, or (ii) with 3-4 points scored in the ICBD criteria.RESULTS: In total 202 patients were included and categorized as follows: 58 patients (28.7%) as 'probable BS', 57 (28.2%) skin disease, 26 (12.9%) chronic pain syndrome, 14 (6.9%) eye disease, 11 (5.4%) spondyloarthropathy, 9 (4.5%) gastrointestinal disease, 7 (3.5%) neurological disease, 4 (2%) arthritis, 3 (1.5%) auto-inflammation, 3 (1.5%) connective tissue disease and 10 (5.0%) miscellaneous disease. HLA-B51 was positive in 55/132 (41.7%); 75/202 (37.1%) of the patients fulfilled the ICBD criteria.CONCLUSION: In a low disease prevalence setting, the straightforward application of the ICBD criteria may lead to overdiagnosis of BS. The differential diagnosis of BS is enormously broad. Clinicians should be aware that HLA-B51 positivity is still not considered as a diagnostic feature in BS.

KW - Behcet Syndrome/diagnosis

KW - Diagnosis, Differential

KW - HLA-B51 Antigen

KW - Humans

KW - Retrospective Studies

KW - Stomatitis, Aphthous

U2 - 10.1093/rheumatology/kead101

DO - 10.1093/rheumatology/kead101

M3 - SCORING: Journal article

C2 - 36864623

VL - 62

SP - 3654

EP - 3661

JO - RHEUMATOLOGY

JF - RHEUMATOLOGY

SN - 1462-0324

IS - 11

ER -