scholarly journals Behçet‘s Syndrome Apart From the Triple Symptom Complex: Vascular, Neurologic, Gastrointestinal, and Musculoskeletal Manifestations. A Mini Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Ina Kötter ◽  
Fabian Lötscher
Keyword(s):  
Current Knowledge ◽  
Clinical Manifestations ◽  
Behçet’S Syndrome ◽  
Cutaneous Lesions ◽  
Behçet's Syndrome ◽  
Ocular Manifestations ◽  
Major Vessel ◽  
Musculoskeletal Manifestations ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Behçet‘s Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis—major vessel and life—or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.

scholarly journals New Insights into Behçet’s Syndrome Metabolic Reprogramming: Citrate Pathway Dysregulation

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Anna Santarsiero ◽  
Pietro Leccese ◽  
Paolo Convertini ◽  
Angela Padula ◽  
Paolo Abriola ◽  
...  
Keyword(s):  
Research Effort ◽  
Clinical Manifestations ◽  
Metabolic Reprogramming ◽  
Mrna Levels ◽  
Behçet’S Syndrome ◽  
Healthy Controls ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Pathway Dysregulation ◽  
Behcet's Syndrome

To date, a major research effort on Behçet’s syndrome (BS) has been concentrated on immunological aspects. Little is known about the metabolic reprogramming in BS. Citrate is an intermediary metabolite synthesized in mitochondria, and when transported into the cytosol by the mitochondrial citrate carrier—SLC25A1-encoded protein—it is cleaved into acetyl-CoA and oxaloacetate by ATP citrate lyase (ACLY). In induced macrophages, mitochondrial citrate is necessary for the production of inflammatory mediators. The aim of our study was to evaluate SLC25A1 and ACLY expression levels in BS patients. Following a power analysis undertaken on few random samples, the number of enrolled patients was set. Thirty-nine consecutive BS patients fulfilling ISG criteria, and 21 healthy controls suitable for age and sex were recruited. BS patients were divided into two groups according to the presence (active) or absence (inactive) of clinical manifestations. Real-time PCR experiments were performed on PBMCs to quantify SLC25A1 and ACLY mRNA levels. Data processing through the Kruskal-Wallis test and Dunn’s multiple comparison test as post hoc showed higher SLC25A1 and ACLY mRNA levels in BS patients compared to those in healthy controls. Therefore, SLC25A1 and ACLY upregulation suggests that metabolic reprogramming in BS involves the citrate pathway dysregulation.

scholarly journals Modulation of gut microbiota through nutritional interventions in Behçet's syndrome patients (the MAMBA study): study protocol for a randomized controlled trial

2019 ◽  
Author(s):  
Giuditta PAGLIAI ◽  
Monica DINU ◽  
Claudia FIORILLO ◽  
Matteo BECATTI ◽  
Silvia TURRONI ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Mediterranean Diet ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Processed Meat ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Wide Range ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Background Behçet's syndrome (BS) is a systemic inflammatory disorder of unknown etiology, characterized by a wide range of potential clinical manifestations. Recent evidences suggest that the gut microbiota (GM) in BS shows low biodiversity with a significant depletion in butyrate producers. The aim of the present project is to investigate whether a dietary intervention could ameliorate the clinical manifestations and modulate the GM of patients with BS.Methods This is a randomized, open, cross-over study involving 90 BS patients who will be randomized to follow a 3-months dietary profile with either: lacto-ovo-vegetarian diet (VD), Mediterranean diet (MD) or Mediterranean diet supplemented with butyrate (MD-Bt). The VD will contain inulin and resistant starch-rich foods, eggs and dairy, in addition to plant-based food, but will not contain meat, poultry or fish. The MD will contain all food categories and will provide 2 portions per week of fish and 3 portions per week of fresh and processed meat. The MD-Bt will be similar to the MD but supplemented with 1.8 g/day of oral butyrate. The three different dietary patterns will be isocaloric and related to subject’s nutritional requirements. Anthropometric measurements, body composition, blood and fecal samples will be obtained from each participant at the beginning and at the end of each intervention phase. The primary outcomes will be represented by the change from baseline of the BS gastrointestinal and systemic symptoms. Changes from baseline of GM composition, SCFA production, inflammatory and antioxidant profile will be considered as secondary outcomes.Discussion BS is a rare disease, and, actually, not all the available treatments are target therapies. A supportive treatment based on dietary and lifestyle issues, able to restore immune system homeostasis, could have a high impact on costs sustainability for the treatment of such a chronic and disabling inflammatory condition.

scholarly journals Modulation of gut microbiota through nutritional interventions in Behçet's syndrome patients (the MAMBA study): study protocol for a randomized controlled trial

2020 ◽  
Author(s):  
Giuditta PAGLIAI ◽  
Monica DINU ◽  
Claudia FIORILLO ◽  
Matteo BECATTI ◽  
Silvia TURRONI ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Mediterranean Diet ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Processed Meat ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Wide Range ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Background: Behçet's syndrome (BS) is a systemic inflammatory disorder of unknown etiology, characterized by a wide range of potential clinical manifestations. Recent evidences suggest that the gut microbiota (GM) in BS shows low biodiversity with a significant depletion in butyrate producers. The aim of the present project is to investigate whether a dietary intervention could ameliorate the clinical manifestations and modulate the GM of individuals with BS. Methods: This is a randomized, open, cross-over study involving 90 BS individuals who will be randomized to follow a 3-months dietary profile with either: lacto-ovo-vegetarian diet (VD), Mediterranean diet (MD) or Mediterranean diet supplemented with butyrate (MD-Bt). The VD will contain inulin and resistant starch-rich foods, eggs and dairy, in addition to plant-based food, but will not contain meat, poultry or fish. The MD will contain all food categories and will provide 2 portions per week of fish and 3 portions per week of fresh and processed meat. The MD-Bt will be similar to the MD but supplemented with 1.8 g/day of oral butyrate. The three different dietary patterns will be isocaloric and related to participants’ nutritional requirements. Anthropometric measurements, body composition, blood and fecal samples will be obtained from each participant at the beginning and at the end of each intervention phase. The primary outcomes will be represented by the change from baseline of the BS gastrointestinal and systemic symptoms. Changes from baseline of GM composition, SCFA production, inflammatory and antioxidant profile will be considered as secondary outcomes. Discussion: BS is a rare disease, and, actually, not all the available treatments are target therapies. A supportive treatment based on dietary and lifestyle issues, able to restore immune system homeostasis, could have a high impact on costs sustainability for the treatment of such a chronic and disabling inflammatory condition.

About Behcet's syndrome

1998 ◽  
Vol 79 (3) ◽  
pp. 214-215
Author(s):  
A. P. Suvorov ◽  
V. F. Orkin ◽  
A. I. Zavyalov ◽  
A. L. Bakulev ◽  
E. V. Rumyantseva ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Clinical Manifestations ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Symptom Complex ◽  
Ulcerative Lesions ◽  
Behcet's Syndrome

More than half a century ago, the Turkish dermatologist Huluzi Behcet first described a kind of symptom complex, consisting of a combination of ulcerative lesions of the oral cavity, genitals and the choroid. Further study of the disease showed that its clinical manifestations are much more diverse.

scholarly journals Neutrophil-mediated mechanisms in non-vascular Behçet’s syndrome

2021 ◽  
Author(s):  
Alessandra Bettiol ◽  
MATTEO BECATTI ◽  
Elena Slvestri ◽  
Flavia Rita Argento ◽  
Eleonora Fini ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Gastrointestinal Symptoms ◽  
Inactive Disease ◽  
Behçet’S Syndrome ◽  
Ros Production ◽  
Behçet's Syndrome ◽  
Intracellular Ros ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Objective: Behçet’s syndrome (BS) is a systemic vasculitis with several clinical manifestations. Neutrophils hyperactivation mediates vascular BS involvement, via a massive production of reactive oxygen species (ROS) and the release of neutrophil extracellular traps (NETs). We investigated neutrophil-mediated mechanisms of damage in non-vascular BS manifestations and explored in vitro the effects of colchicine in counteracting these mechanisms. Methods: NETs and intracellular ROS production was assessed in blood samples from 80 BS patients (46 with active non-vascular BS, 34 with inactive disease) and 80 healthy controls. Moreover, isolated neutrophils were incubated for 1 hour with an oxidating agent (2,2′-azobis (2-amidinopropane) dihydrochloride; 250nM), and the ability of pure colchicine pre-treatment (100ng/ml) to counteract oxidation-induced damage was assessed. Results: Patients with active non-vascular BS had remarkably increased NET levels [21.2 (IQR 18.3-25.9) mU/ml] compared to patients with inactive disease [16.8 (13.3-20.2) mU/ml] and to controls [7.1 (5.1-8.7) mU/ml], p<0.001]. Also, intracellular ROS tended to be increased in active BS, although not significantly. In active non-vascular BS, NETs correlated with neutrophils ROS production (p<0.001) and were particularly increased in patients with active mucosal (p<0.001), articular (p=0.004), and gastrointestinal symptoms (p=0.006). On isolated neutrophils, colchicine significantly reduced oxidation-induced NET production and cell apoptosis, though not via an antioxidant activity. Conclusion: Neutrophil-mediated mechanisms might be directly involved in non-vascular BS, and NETs, more than ROS, might drive the pathogenesis of mucosal, articular and intestinal manifestations. Colchicine might be effective to counteract neutrophils-mediated damage in BS, although further studies are needed.

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