Phenotypes in Behçet’s syndrome

Seyahi, Emire

doi:10.1007/s11739-019-02046-y

Cited by 100 publications

(72 citation statements)

References 74 publications

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“…During the course of the disease, BS can involve the mucosa, skin, or eyes, vessels, nervous and gastrointestinal system [ 22 , 23 , 46 ]. Several cluster and association studies reported clusters of common overlapping involvements.…”

Section: Discussionmentioning

confidence: 99%

“…The clinical course seems to follow four major clusters: 1) oral and genital ulcers, erythema nodosum 2) superficial and deep vein thrombosis 3) uveitis 4) papulopustuler skin lesions and joint involvement [ 2 ]. Due to this heterogeneous spectrum, BS can hardly be considered as a uniform disease, moreover different pathogenic mechanisms may be present in each cluster [ 22 , 23 ].…”

Section: Introductionmentioning

confidence: 99%

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Succinivibrionaceae is dominant family in fecal microbiota of Behçet’s Syndrome patients with uveitis

et al. 2020

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Behçet’s Syndrome (BS) is a multisystem vasculitis with various clinical manifestations. Pathogenesis is unclear, but studies have shown genetic factors, innate immunity and autoinflammation to have an important role in the disease course. Diversity in the microbial community of gut microbiota may significantly contribute to the activation of the innate immune system. The clinical features of BS present themselves in clusters and each cluster may be a consequence of different disease mechanisms. For this reason we aimed to investigate the gut microbiota of BS patients with uveitis. In addition to healthy controls, we have aimed to compare the gut microbiota of BS with that of Familial Mediterranean Fever (FMF) and Crohn’s Disease (CD) as both diseases have innate and autoinflammatory features in their pathogenesis. Seven patients with BS, 12 patients with FMF, 9 patients with CD and 16 healthy controls (HC) were included in the study. Total genomic DNAs were isolated from fecal samples of the patients. Partial 16S rRNA gene was sequenced using the PGM Ion Torrent (Thermo Fisher Scientific, Waltham, MA, USA) for microbiota analysis. Statistical analysis showed that significant differences were detected on the microbial community of four groups. Succinivibrionaceae is dominant and the signature family, whereas Bacteroides was absent in BS patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Succinivibrionaceae is dominant family in fecal microbiota of Behçet’s Syndrome patients with uveitis

et al. 2020

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“…Another critical issue for patients with late-onset BS is differential diagnosis. BS is not a single disease, but a multi-systemic disorder [24]. From our cohort, we identi ed six patients with myelodysplastic syndrome presenting as a Behçet's like disease, three patients were at late-onset age.…”

Section: Discussionmentioning

confidence: 99%

Phenotype heterogeneity in Late-onset Behçet’s syndrome: A cohort from a referral center in China

Zou¹,

Luo²,

Luo³

et al. 2020

Preprint

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Objectives To elaluate the demographic, clinical and major organ involvement of patients with late-onset Behçet’s syndrome (BS) in a tertiary centre in China.Methods It was a cross-sectional study of consecutive BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020, we identified 152 late-onset BS patients diagnosed after age of 40 years. We compared clinical variables between patients with disease onset age before and after 40 years. The relative risks (RRs) of clinical variables were calculated between two age groups. Moreover, a hierarchical cluster analysis was conducted according to twenty-nine variables to determine homogeneous subgroups of patients.Results Late-onset BS patients had a higher incidence of intestinal ulcer (late-onset : early-onset, RR 1.47), but a lower incidence of ocular involvement (RR 0.54) and folliculitis (RR 0.46). Female was associated with genital ulcer, erythema nodosum, and arthritis. Four clusters (C1–C4) were formed. C1 (n = 71) the largest cluster, defined as mucocutaneous group. C2 (n = 20), the arthritis group, C3 (n = 39), the gastrointestinal type, all patients presented with intestinal lesion, and five cases with esophageal ulcer. In C4 (n = 22), showing a mixture of uveitis and vascular group, fifteen patients presented with uveitis and eight had vascular lesion, and one cases had central nervous system lesions.Conclusion This is the first cluster analysis to be carried out in a cohort of late-onset BS patients in China. The significantly lower prevalence of folliculitis in late onset BS and the overall milder disease course suggest that testosterone status may influence disease activity.

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“…clusters of co-existing involvements) were described in different populations based on cluster analysis and association studies. In her review manuscript, Seyahi reported the main "disease phenotypes", namely the "mucocutaneous and articular", the "vascular" the "neurological and ocular" and the "gastrointestinal" ones [44].…”

Section: Clinical Manifestations and Phenotypesmentioning

confidence: 99%

Behçet’s syndrome: focus on pathogenetic background, clinical phenotypes and specific treatments

Emmi

Prisco

2019

Intern Emerg Med

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Phenotypes in Behçet’s syndrome

Cited by 100 publications

References 74 publications

Succinivibrionaceae is dominant family in fecal microbiota of Behçet’s Syndrome patients with uveitis

Succinivibrionaceae is dominant family in fecal microbiota of Behçet’s Syndrome patients with uveitis

Phenotype heterogeneity in Late-onset Behçet’s syndrome: A cohort from a referral center in China

Behçet’s syndrome: focus on pathogenetic background, clinical phenotypes and specific treatments

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