Behçet's syndrome patients exhibit specific microbiome signature

Consolandi, Clarissa; Turroni, Silvia; Emmi, Giacomo; Severgnini, Marco; Fiori, Jessica; Peano, Clelia; Biagi, Elena; Grassi, Alessia; Rampelli, Simone; Silvestri, Elena; Centanni, Manuela; Cianchi, Fabio; Gotti, Roberto; Emmi, Lorenzo; Brigidi, Patrizia; Bizzaro, Nicola; Bellis, Gianluca De; Prisco, Domenico; Candela, Marco; D’Elios, Mario Milco

doi:10.1016/j.autrev.2014.11.009

Cited by 199 publications

(156 citation statements)

References 45 publications

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“…Triggering infectious factors are supposed to participate in the outbreak of BD in genetically predisposed subjects [19], and even a peculiar dysbiosis of the gut microbiota might be involved in the differentiation of T-regulatory cells of BD patients [20]. On the other hand, the role of infections in PFAPA syndrome has not yet been elucidated, and many authors question the existence of unambiguous microbiologic etiology [21].…”

Section: Discussionmentioning

confidence: 99%

PFAPA syndrome and Behçet’s disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists

et al. 2015

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The pediatric syndrome characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) and adult Behçet's disease share some clinical manifestations and are both polygenic autoinflammatory disorders with interleukin-1β showing to play a pivotal role. However, the diagnosis is mostly clinical and we hypothesize that specific criteria may be addressed differently by different physicians. To determine the diagnostic variability, we compared the answers of 80 patients with a definite diagnosis of Behçet's disease (age 42.1 ± 13.7 years) obtained by separate telephone interviews conducted by a rheumatologist, a pediatrician, and an internist working largely in the field of autoinflammatory disorders. Questions were related to the age of symptom onset, the occurrence of recurrent fevers during childhood, and the association with oral aphthosis, cervical adenitis and/or pharyngitis, previous treatments, possible growth impairment, the time lapse between PFAPA-like symptoms and the onset of Behçet's disease, and the occurrence of Behçet-related manifestation during childhood. The rheumatologist identified 30 % of patients with Behçet's disease fulfilling PFAPA syndrome diagnostic criteria, compared to the pediatrician and the internist identifying 10 and 7.5 %, respectively. Most of the patients suffered from recurrent oral aphthosis in childhood also without fever (50, 39, and 48 % with each interviewer), yet no patient fulfilled the Behçet's disease diagnostic criteria. Our data suggest that physician awareness and expertise are central to the diagnosis of autoinflammatory disorders through an accurate collection of the medical history.

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Section: Discussionmentioning

confidence: 99%

PFAPA syndrome and Behçet’s disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists

et al. 2015

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“…It may be that only very small numbers of certain pathogenic strains are required to cause disease in susceptible individuals, allowing them to be overlooked when hundreds of microbes are detected. Or, it may be that bacterial diversity actually confers vascular protection, and perhaps it is a loss of important commensals that promotes disease susceptibility, as is seen in inflammatory bowel disease [5] and Behcet's disease [75]. The increasingly frequent identification of microbes within healthy vessels supports this latter notion and raises the possibility that microbes may be a 'normal' fixture in our anatomy [49 && ].…”

Section: Interpretation Of Findings and Next Stepsmentioning

confidence: 99%

Evidence for a vascular microbiome and its role in vessel health and disease

Clifford

Hoffman²

2015

Current Opinion in Rheumatology

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“…They found that Haemophilus parainfluenza was the most abundant species in BD, whereas Alloprevotella rava and species in the Leptotrichia genus were less abundant. Another study by Consolandi et al [42] showed gut microbiota dysbiosis and decreased production of butyrate in patients with BD compared to healthy controls. Finally, the oral microbial flora have also been implicated in the pathogenesis of the disease, with Streptococcus and streptococcal infections being the most common in the oral cavity of BD patients with unhygienic oral conditions [43].…”

Section: Microbiome Involvementmentioning

confidence: 96%

Is Behçet's disease a ‘class 1-opathy’? The role of HLA-B*51 in the pathogenesis of Behçet's disease

Giza

Koftori

Chen

et al. 2017

Clinical and Experimental Immunology

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Summary The association between carriage of the human leucocyte antigen (HLA)-B*51 allele and development of Behçet's disease (BD) has been known since the early 1970s, but the exact mechanisms responsible for its role in pathogenesis remain much-debated. In an effort to explain the disease process, it has been suggested that BD constitutes one of a newly termed group of diseases, the ‘MHC-I-opathies’. Other MHC-I-opathies include ankylosing spondylitis and HLA-B*27-associated spondyloarthropathies and HLA-C*0602-associated skin psoriasis. Recent work analysing the peptidome of HLA-B*51 suggests that altered peptide presentation by HLA-B*51 is vital to the disease process. In this review, we argue that immune receptor interactions with HLA-B*51 or the HLA-B*51-peptide complex could lead to development of inflammation in BD. The evidence for CD8+ T cell involvement is weak, and based on emerging studies it seems more likely that natural killer (NK) or other cell interactions, perhaps mediated by leucocyte immunoglobulin-like receptor (LILR) or killer immunoglobulin-like receptor (KIR) receptors, are culpable in pathogenesis. HLA misfolding leading directly to inflammation is another hypothesis for BD pathogenesis that deserves greater investigation. Ultimately, greater understanding of HLA-B*51's unique role in BD will probably lead to improved development of therapeutic strategies.

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Behçet's syndrome patients exhibit specific microbiome signature

Abstract: Altogether, our results indicate that both a peculiar dysbiosis of the gut microbiota and a significant decrease of butyrate production are present in patients with Behçet syndrome.

Cited by 199 publications

References 45 publications

PFAPA syndrome and Behçet’s disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists

PFAPA syndrome and Behçet’s disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists

Evidence for a vascular microbiome and its role in vessel health and disease

Is Behçet's disease a ‘class 1-opathy’? The role of HLA-B*51 in the pathogenesis of Behçet's disease

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