Clinical and epidemiological features of visual organ damage in systemic connective tissue diseases

2020 ◽  
Vol 1-2 (211-212) ◽  
pp. 67-74
Author(s):  
Akmarzhan Rystanbayeva ◽  
◽  
Aigul Balmukhanova ◽  
Olga Mashkunova ◽  
Kuralay Kaynazarova ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Systemic Vasculitis ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Posterior Uveitis ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

To this date, uveitis associated with connective tissue diseases remain relevant due to serious complications that significantly reduce vision and thus worsen the quality of life of patients. Aim. To study the frequency and prevalence, as well as clinical manifestations of ophthalmopathology in connective tissue diseases. Material and methods. A retrospective and prospective study of 534 patients (144 men and 390 women) with connective tissue diseases was conducted, in which ophthalmological disorders were detected. The patients underwent standard rheumatological examination in the department. And also, ophthalmological examination with the determination of visual acuity, intraocular pressure and the use of biomicroscopy, ophthalmoscopy (or cycloscopy). If necessary and possible, additional studies were performed such as: ultrasound B-scan, optical coherence tomography, perimetry. The study examined the type of common disease, age of onset and gender of the patient. Of particular interest were patients with uveal inflammation, in which the localization and nature of the course of inflammation were evaluated. Results and discussion. The most common diseases with eye damage were rheumatoid arthritis (RA) – 129 (24.1%), systemic lupus erythematosus (SLE) – 92 (17.2%), inflammatory bowel disease (IBD) – 95 (17.8%), systemic scleroderma (SDS) – 54 (10.1%) and spondylarthritis (Spa) – 41 (7.8%). In a small percentage of cases, other connective tissue diseases were diagnosed, such as: mixed connective tissue diseases (MCTS) – 24 (4.5%), Overlap syndrome – 21 (3.9%), systemic vasculitis – 23 (4.3%), Behcet's syndrome -18 (3.4%), Sjogren disease – 14 (2.6%) and juvenile idiopathic arthritis - 7 (1.3%). All ophthalmic disorders were divided into four general groups and distributed as follows: inflammatory 27.7%, degenerative 4.9%, vascular 48.7%, side effects of glucocorticoids 18.7%. Uveitis accounted for 52 (9.7%) of all ophthalmopathology, occurring in connective tissue diseases. Unilateral acute anterior uveitis (AAU) was diagnosed in 13 (25%) patients with ankylosing spondylitis, of which 4 (7.8%) had a relapse in the paired eye. Simultaneously, bilateral AAU was detected in 3 (5.7%) patients with Behcet's syndrome. Chronic, recurrent posterior uveitis was diagnosed in 13 (25%) patients with Behcet's systemic vasculitis. All patients with Behcet's syndrome were male at the age of 31±3.1 years. The uveal process approximately started 3.2±1.16 years after the onset of systemic disease. Generalization of inflammation in all departments of uvea was detected in patients with PSA-5 (9.6%), AC – 3 (5.7%), DS – 2 (3.8%) and SLE – 2 (3.8%). In 5 (9.6%) patients, the process was bilateral. Inflammation of the paired eye by the type of AAU was observed in 1 patient and posterior uveitis - in 2 patients. Conclusions. Ophthalmic disorders in the structure of systemic diseases make up 20.2% and vary widely in their clinical manifestations. Chronic blepharoconjunctivitis, combined with rheumatoid arthritis (46%) is a common inflammation of the accessory structures of the eye. About 20% of patients have complications from general immunosuppressive therapy, leading to impaired visual function. The frequency of uveitis in the structure of ophthalmopathology associated with connective tissue disease is 9.7%. Uveitis associated with ankylosing spondylitis and systemic vasculitis of Behcet occurs in men at a younger age and is 31%. More than half (63%) of patients with uveitis have a severe course with the development of panuveitis and neuropathy. An increase in the thickness of the choroid (up to 2 mm) according to two-dimensional ultrasound is an early diagnostic criterion for the development of uveitis and the underlying disease of connective tissue. Keywords: inflammatory eye diseases, uveitis, connective tissue diseases, spondyloarthritis, Behcet's syndrome, complications.

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.

scholarly journals Clinical Manifestations and Diagnosis of Behçet’s Syndrome

2021 ◽  
pp. 94-104
Author(s):  
Sara Beça ◽  
Gerard Espinosa
Keyword(s):  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Disease Assessment ◽  
Significant Heterogeneity ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Clinical Presentations ◽  
Wide Range ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Behçet’s syndrome (BS) is a systemic vasculitis with a wide range of clinical presentations and disease courses. It may involve the mucosa, skin, joints, vessels, eyes, and nervous and gastrointestinal systems. These organ involvements may present alone or co-exist in the same patient. Three main clusters of commonly co-existing manifestations were recognised and are currently called disease phenotypes. There is a significant heterogeneity among patients regarding demographic features and clinical expression of the disease that hinders a standardised disease assessment and a generalised use of diagnostic criteria. Additionally, BS is not associated with pathognomonic laboratory or histopathology features; therefore, the diagnosis is mainly based on the clinical manifestations. The purpose of this narrative review of the literature is to provide a description of the most common or typical clinical features of BS, summarise the major phenotypes of BS, and address the diagnosis strategy of this syndrome.

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 Pathogenesis of Behçet's Syndrome: Genetic, Environmental and Immunological Factors

2021 ◽  
Vol 8 ◽  
Author(s):  
Irene Mattioli ◽  
Alessandra Bettiol ◽  
Güher Saruhan-Direskeneli ◽  
Haner Direskeneli ◽  
Giacomo Emmi
Keyword(s):  
Systemic Vasculitis ◽  
Strong Association ◽  
Current Evidence ◽  
Behçet’S Syndrome ◽  
Predisposing Factor ◽  
Behçet's Syndrome ◽  
Wide Range ◽  
Behcet’S Syndrome ◽  
Immunological Factors ◽  
Behcet's Syndrome

Behçet's syndrome (BS) is a rare systemic vasculitis, characterized by a wide range of different clinical involvements and unpredictable phases of recurrence and remission. BS can be described as a multifactorial disease with an incompletely known etiopathogenesis; in fact, though presenting some peculiar features, such as its typical geographic distribution and the strong association with the well-known genetic predisposing factor HLA-B*51, the cause behind the onset and progression of the disease remains currently not fully understood. Besides genetic HLA and non-HLA predisposing associations and epigenetic influence, environmental factors also play an important role in the pathogenesis of the disease, and among these, infectious agents (both bacterial and viral) and specific microbiome alterations are considered of particular relevance in BS pathogenesis. BS has been included for decades among autoimmune diseases, in light of evidence showing T- and B-cell aberrant responses. However, because of recurrent mucocutaneous lesions and episodes of inflammation without antigen-specific T-cell or autoantibody responses, BS has also been classified among autoinflammatory disorders. Nevertheless, differently from autoinflammatory diseases, BS mildly responds to therapies targeting IL-1, its onset is not usually in childhood, and has high neutrophilic vasculitic involvement. Finally, given the association with HLA class I alleles, similar to spondyloarthropathies, the concept of BS as a major histocompatibility complex (MHC) I -opathy has been introduced. Understanding the complex etiopathogenesis of BS is essential to identify modifiable risk factors of BS occurrence or exacerbation and to develop targeted therapies. This review summarizes current evidence on the main genetic, environmental and immunological factors contributing to BS development.

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.

Behçet: the syndrome

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii101-iii107 ◽  
Author(s):  
Alessandra Bettiol ◽  
Domenico Prisco ◽  
Giacomo Emmi
Keyword(s):  
Systemic Vasculitis ◽  
Current Evidence ◽  
Patient Specific ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Disease Phenotypes ◽  
Genetic Features ◽  
Distinct Disease ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Behçet’s syndrome (BS) is a systemic vasculitis characterized by a relapsing and remitting course. It can involve the skin, mucosa, joints, vessels (arteries and/or veins), eyes, and nervous and gastrointestinal systems, and so is referred to as a syndrome rather than as a unique and nosologically distinct condition. These involvements may present alone or co-exist in the same patient. Although all the possible combinations of the above-mentioned manifestations may occur, clusters of commonly co-existing involvements (also referred to as ‘disease phenotypes’) have been suggested, namely ‘mucocutaneous and articular’, ‘peripheral vascular and extra-parenchymal neurological’ and ‘parenchymal neurological and ocular’ phenotypes have been described. Patient-specific demographic and genetic features have been described as positively or negatively associated with specific disease phenotypes. This review will focus on the different clinical features of Behçet’s syndrome, summarizing current evidence on the distinct disease manifestations as well as the major phenotypes.

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