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International Journal For Vitamin and... Oct 2020In the present study, the evidence about the association between vitamin D deficiency and Behcet's disease activity was systematically reviewed and meta-analyzed. We... (Meta-Analysis)
Meta-Analysis
In the present study, the evidence about the association between vitamin D deficiency and Behcet's disease activity was systematically reviewed and meta-analyzed. We searched the English and Persian databases of Medline (Ovid), CINHAL, Scopus, Proquest, the Cochrane library and SID, IranDoc, Magiran, Iran Medex for articles published up until May 2018 with the keywords were related to serum vitamin D and active and inactive Behcet's disease in adults. Meta-analysis was done using the CMA software. A total of 138 titles were retrieved and reduced to 80 titles after deletion of duplicates and finally after close assessing of titles and abstracts eight eligible studies including a total of 939 participants were identified for systematic review and meta-analysis. According to the results of the meta-analysis, the pooled effect size of the differences in the serum level of vitamin D in patients with inactive Behçet's Disease and healthy controls was [OR:-0.05; 95% CI:-2.05, 1.94; p = 0.95]. The serum vitamin D level was significantly lower in active patients compared with healthy controls [OR:1.21; 95%CI: -0.12, 2.31; p = 0.03]. The pooled effect size of the differences in the serum level of vitamin D in active and inactive Behçet's Disease was [OR:-0.71; 95%CI: -1.41, -0.007; p = 0.04] There is an association between vitamin D deficiency and active Behçet's Disease. Future studies investigating the association of vitamin D deficiency and Behçet's Disease needs to involve following information: dietary intake of calcium and vitamin D, measuring of sun exposure, report of drug consumption and physical activity level.
Topics: Adult; Behcet Syndrome; Humans; Iran; Vitamin D; Vitamin D Deficiency
PubMed: 30789802
DOI: 10.1024/0300-9831/a000542 -
Minerva Ginecologica Oct 2020Behcet's disease (BD) is a rare inflammatory, multisystemic, autoimmune disorder with unknown origin. BD is included in vasculitic disorders with a more frequent onset...
INTRODUCTION
Behcet's disease (BD) is a rare inflammatory, multisystemic, autoimmune disorder with unknown origin. BD is included in vasculitic disorders with a more frequent onset characterized by oral and genital ulcers associated with eye inflammation. However, BD has several clinical manifestations, and the most fearful complication is thrombotic involvement. BD occurs mainly in women of childbearing age, therefore it is important to identify the potential risks of pregnancy on the mother and fetus.
EVIDENCE ACQUISITION
The aim of our review is to identify, through the study of existing literature, the possible consequences of pregnancy on the course of this disease, the potential risks for the mother and fetus in gestation period and in puerperium, in order to identify a correct pregnancy management in patient affected by BD.
EVIDENCE SYNTHESIS
Currently, there are few studies that have analyzed the consequences of the disease on the course of pregnancy and pregnancy on the activity of the pathology. Some authors believe that pregnancy may worsen the symptoms of the disease, while others may even improve the course. Many authors believe that thromboembolic events are the main problems for which focus attention on these patients, both in pregnancy and in puerperium. Different opinions exist about pregnancy complications and neonatal outcomes, although events such as abortion, intrauterine growth restriction and C-section appear to have a higher incidence in BD patients.
CONCLUSIONS
There are no contraindications for the onset of pregnancy in BD patients. In most cases pregnancy can improve the course of the disease. However, in view of the potential adverse events, a thorough follow-up of the pregnancy is necessary in order to minimize any risks to the mother and fetus.
Topics: Abortion, Spontaneous; Behcet Syndrome; Female; Humans; Infant, Newborn; Postpartum Period; Pregnancy; Pregnancy Complications; Retrospective Studies
PubMed: 32403914
DOI: 10.23736/S0026-4784.20.04564-5 -
Expert Review of Clinical Immunology May 2023Noninfectious uveitis related to systemic inflammatory diseases represents a leading cause of blindness. Anti-TNFα agents are the first-line biologic therapy after...
INTRODUCTION
Noninfectious uveitis related to systemic inflammatory diseases represents a leading cause of blindness. Anti-TNFα agents are the first-line biologic therapy after traditional immunosuppressants, for ocular and systemic involvement. However, some patients fails anti-TNFα agents, due to primary inefficacy, loss of efficacy or adverse events.
AREAS COVERED
This systematic review summarizes evidence on the efficacy and safety of non-anti-TNFα biologics in adult patients with noninfectious uveitis associated with systemic inflammatory diseases. The systematic review of PubMed and Embase yielded 3663 records, from which 16 studies were included (13 non-controlled, 3 controlled trials). Most studies focused on Behçet's syndrome (BS) and juvenile idiopathic arthritis (JIA) and assessed the efficacy of tocilizumab ( = 11), rituximab ( = 3), secukinumab ( = 1), or anakinra/canakinumab ( = 1). A body of evidence supports the use of tocilizumab BS and JIA-associated uveitis, for improving visual acuity, reducing central macular thickness, inducing ocular remission, and sparing corticosteroids. Preliminary data suggest that rituximab may represent a valid alternative, particularly in JIA, while anakinra/canakinumab might play a role in BS-associated uveitis. The role of secukinumab appears limited.
EXPERT OPINION
Current evidence encourages investigations on the efficacy and safety of non-anti-TNFα agents in noninfectious non-idiopathic uveitis.
Topics: Adult; Humans; Arthritis, Juvenile; Behcet Syndrome; Biological Factors; Immunosuppressive Agents; Interleukin 1 Receptor Antagonist Protein; Rituximab; Uveitis; Tumor Necrosis Factor-alpha
PubMed: 36939549
DOI: 10.1080/1744666X.2023.2193687 -
International Journal of Molecular... Nov 2023Behçet's disease (BD) is a complex, recurring inflammatory disorder with autoinflammatory and autoimmune components. This comprehensive review aims to explore BD's... (Review)
Review
Behçet's disease (BD) is a complex, recurring inflammatory disorder with autoinflammatory and autoimmune components. This comprehensive review aims to explore BD's pathogenesis, focusing on established genetic factors. Studies reveal that is the primary genetic risk factor, but non-HLA genes (, , ), as well as innate immunity genes (, , ), also contribute. Genome-wide studies emphasize the significance of and HLA-I epistasis. These variants influence antigen presentation, enzymatic activity, and HLA-I peptidomes, potentially leading to distinct autoimmune responses. We conducted a systematic review of the literature to identify studies exploring the association between and BD and further highlighted the roles of innate and adaptive immunity in BD. Dysregulations in Th1/Th2 and Th17/Th1 ratios, heightened clonal cytotoxic (CD8+) T cells, and reduced T regulatory cells characterize BD's complex immune responses. Various immune cell types (neutrophils, γδ T cells, natural killer cells) further contribute by releasing cytokines (IL-17, IL-8, GM-CSF) that enhance neutrophil activation and mediate interactions between innate and adaptive immunity. In summary, this review advances our understanding of BD pathogenesis while acknowledging the research limitations. Further exploration of genetic interactions, immune dysregulation, and immune cell roles is crucial. Future studies may unveil novel diagnostic and therapeutic strategies, offering improved management for this complex disease.
Topics: Humans; Behcet Syndrome; Antigen Presentation; Genetic Predisposition to Disease; HLA-B Antigens; Risk Factors; Aminopeptidases; Minor Histocompatibility Antigens
PubMed: 38003572
DOI: 10.3390/ijms242216382 -
Autoimmunity Reviews Mar 2022Vasculitis are severe systemic autoimmune diseases which may involve different organs and systems. Conversely, muscles do not represent an organ commonly involved by... (Review)
Review
Vasculitis are severe systemic autoimmune diseases which may involve different organs and systems. Conversely, muscles do not represent an organ commonly involved by systemic vasculitis and myositis is not include among any classification or diagnostic criterion of vasculitis. In this regard, we aimed to review the literature in order to report all the available evidence concerning the inflammatory involvement of muscle in patients affected by systemic vasculitis. We collected a total of 108 papers, for a sum of 395 patients affected by muscle vasculitis. Most of them suffered from medium and small vessels vasculitis (mainly polyarteritis nodosa and ANCA-associated vasculitis) or from vasculitis secondary to rheumatoid arthritis. Conversely, muscle involvement in case of large vessel vasculitis occurred seldom, while only few papers reported such occurrence in Kawasaki or Behçet's disease. Histological findings may differ, but the most common ones displayed a necrotizing vasculitis of perimysium vessels, while granulomatous vasculitis was assessed only in case of ANCA-associated vasculitis patients. Creatine kinase were usually within normal range, seldom elevated, while imaging findings were generally undistinguishable from the ones found in idiopathic inflammatory myopathies: magnetic resonance imaging displays signal hyperintensity in T2 and STIR scans, while few data exist for positron emission tomography. The presentation of the disease may be fearsome and severe, sometimes life-threatening, but an overall good response to conventional immunosuppressants and/or glucocorticoids has been reported.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Arteritis; Behcet Syndrome; Humans; Muscles; Polyarteritis Nodosa
PubMed: 34971804
DOI: 10.1016/j.autrev.2021.103029 -
Clinical and Experimental Rheumatology 2021Children and adults may develop Behçet's disease (BD), often with ocular involvement such as uveitis. This study aimed to determine the prevalence and type of ocular... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
Children and adults may develop Behçet's disease (BD), often with ocular involvement such as uveitis. This study aimed to determine the prevalence and type of ocular manifestations in childhood and adult BD.
METHODS
Medline, Web of Science and Cochrane databases were searched from inception to October 5, 2018 to identify publications related to Behçet's disease comprising minimum twenty patients and providing the frequency of ocular manifestations (OC). Random effects models were used to combine the prevalence of OC in adults and children with BD. Heterogeneity was evaluated using I2.
RESULTS
The search resulted in 3129 articles, of which 51 were included in meta-analysis. OCs were slightly more frequent in childhood onset BD with the mean [95% Confidence Interval] frequency of 45 [34-56%] compared to 36 [29-43%] in adults, however, this difference was not statistically significant (p=0.198). In both children and adults, posterior uveitis (children 27% vs. adults 25%, and retinal vasculitis in adults 16%) was the most common ocular manifestation, followed by anterior uveitis (children 18% vs. adults 23%). When comparing the distribution of OC in Behcet's in adults, there was geographic variation where OC were higher in Turkey and the Middle East 42%, followed by Europe and North America (36%), North Africa 26% and East Asia 25% but not significantly (p=0.27).
CONCLUSIONS
Ocular manifestations, predominantly uveitis; are common in BD. Ocular manifestations are not proportionately more frequent in adults with BD along the ancient Silk Road.
Topics: Adult; Behcet Syndrome; Child; Humans; Prevalence; Retinal Vasculitis; Uveitis; Uveitis, Posterior
PubMed: 34596037
DOI: 10.55563/clinexprheumatol/pt60bc -
Rheumatology International Jan 2023Behçet's Disease (BD) can be correlated with sleep impairment and fatigue, resulting in low quality of life (QoL); however, a comprehensive evaluation of this issue is... (Review)
Review
Behçet's Disease (BD) can be correlated with sleep impairment and fatigue, resulting in low quality of life (QoL); however, a comprehensive evaluation of this issue is still missing. We performed a systematic literature review (SLR) of existing evidence in literature regarding sleep quality in BD. Fifteen papers were included in the SLR. Two domains were mainly considered: global sleep characteristics (i) and the identification of specific sleep disorders (ii) in BD patients. From our analysis, it was found that patients affected by BD scored significantly higher Pittsburgh Sleep Quality Index (PSQI) compared to controls. Four papers out of 15 (27%) studied the relationship between sleep disturbance in BD and disease activity and with regards to disease activity measures, BD-Current Activity Form was adopted in all papers, followed by Behçet's Disease Severity (BDS) score, genital ulcer severity score and oral ulcer severity score. Poor sleep quality showed a positive correlation with active disease in 3 out of 4 studies. Six papers reported significant differences between BD patients with and without sleep disturbances regarding specific disease manifestations. Notably, arthritis and genital ulcers were found to be more severe when the PSQI score increased. Our work demonstrated lower quality of sleep in BD patients when compared to the general population, both as altered sleep parameters and higher incidence of specific sleep disorders. A global clinical patient evaluation should thereby include sleep assessment through the creation and adoption of disease-specific and accessible tests.
Topics: Humans; Behcet Syndrome; Quality of Life; Sleep Quality; Sleep; Sleep Wake Disorders
PubMed: 36194239
DOI: 10.1007/s00296-022-05218-w -
The Journal of International Medical... May 2023To study the effectiveness of infliximab for the treatment of refractory central neuro-Behçet's disease. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To study the effectiveness of infliximab for the treatment of refractory central neuro-Behçet's disease.
METHODS
In this systematic review and meta-analysis, the research question was designed using the 'Population, Intervention, Comparator, and Outcomes' (PICO) model and the search methodology was developed according to the PRISMA statement. The study was registered on PROSPERO. Web of Science, PubMed, and Cochrane Library databases were searched for articles published in English between January 2000 and January 2020. Data were analysed using Meta-Essentials software, version 10.12. Treatment effect size was determined by a random effects model. Interstudy heterogeneity was explored using I statistics. Cumulative meta-analysis was conducted to assess the temporal trend for accumulating evidence.
RESULTS
Twenty-one studies, comprising 64 patients (mean age, 38 .21 years and mean disease duration, 84.76 months) were included. Effect-size analysis showed that 93.7% of the treated patients in the analysis were responders to infliximab therapy (95% confidence interval 0.88, 0.993). There was no significant inter-study heterogeneity (I = 0%). Cumulative analysis showed accumulating evidence favoring increasing effectiveness over the last 20 years.
CONCLUSION
Infliximab showed considerable therapeutic effectiveness in the treatment of refractory neuro-Behçet's disease.
Topics: Humans; Adult; Infliximab; Behcet Syndrome; Antibodies, Monoclonal; Tumor Necrosis Factor-alpha; Necrosis
PubMed: 37203384
DOI: 10.1177/03000605231169895 -
Clinical Ophthalmology (Auckland, N.Z.) 2020To systematically review the characteristics of patients with endogenous tuberculous (TB) endophthalmitis and panophthalmitis in an effort to help clinicians with...
PURPOSE
To systematically review the characteristics of patients with endogenous tuberculous (TB) endophthalmitis and panophthalmitis in an effort to help clinicians with diagnosis and treatment.
PATIENTS AND METHODS
We conducted a systematic literature search in MEDLINE/PubMed, EMBASE and Web of Science from inception to August 2020. References and abstracts were screened independently by two authors. Included studies were case reports and case series reporting endogenous TB endophthalmitis and panophthalmitis secondary to complex (MTBC). Available-case analysis was employed to handle missing data.
RESULTS
A total of 1343 articles were found using the search strategy. Following abstract screening, 51 articles were selected for full text-review, from which 26 were deemed eligible for inclusion in the study. Forty-four cases from 26 articles were included in the quantitative analysis. The median age of presentation was 29.5 (range: 1 to 81), and 11/44 patients (25.0%) were pediatric. Immunosuppression was seen in 9/36 cases (25.0%). Most patients (24/38, 63.2%) had no prior history of tuberculosis. Systemic symptoms were absent in half of the patients (16/32, 50.0%). Visual acuity was poor, with 23/27 cases (85.2%) being 20/200 or worse at presentation. Poor organ and visual outcomes were reported: 36/43 cases (83.7%) resulted in enucleation/evisceration or exenteration. Intraocular tumors were suspected in 5/34 cases (14.7%). Pulmonary tuberculosis was seen in 15/35 cases (42.8%), and miliary tuberculosis was seen in 7/35 cases (20.0%). The earliest source of TB diagnosis was through histopathologic specimen after eye removal in 32/44 cases (72.7%), vitreous specimen in 6/44 cases (13.6%) and aqueous specimen in 3/44 cases (6.8%).
CONCLUSION
TB endophthalmitis is a rare and sight-threatening manifestation of ocular tuberculosis. It can occur in apparently healthy individuals and can mimic intraocular tumors and other infectious etiologies. Diagnosis remains a significant challenge, which, often delayed, leads to profound visual loss. Early detection and treatment of intraocular tuberculosis may be associated with better ocular and systemic outcomes.
PubMed: 33116360
DOI: 10.2147/OPTH.S265521 -
International Braz J Urol : Official... 2016Improved targeted therapies for rheumatic diseases were developed recently resulting in a better prognosis for affected patients. Nowadays, patients are living longer... (Review)
Review
BACKGROUND
Improved targeted therapies for rheumatic diseases were developed recently resulting in a better prognosis for affected patients. Nowadays, patients are living longer and with improved quality of life, including fertility potential. These patients are affected by impaired reproductive function and the causes are often multifactorial related to particularities of each disease. This review highlights how rheumatic diseases and their management affect testicular function and male fertility.
MATERIALS AND METHODS
A systematic review of literature of all published data after 1970 was conducted. Data was collected about fertility abnormalities in male patients with systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, ankylosing spondylitis, Behçet disease and gout. Two independent researchers carried out the search in online databases.
RESULTS
A total of 19 articles were included addressing the following diseases: 7 systemic lupus erythematosus, 6 Behçet disease, 4 ankylosing spondylitis, 2 rheumatoid arthritis, 2 dermatomyositis and one gout. Systemic lupus erythematosus clearly affects gonadal function impairing spermatogenesis mainly due to antisperm antibodies and cyclophosphamide therapy. Behçet disease, gout and ankylosing spondylitis patients, including those under anti-TNF therapy in the latter disease, do not seem to have reduced fertility whereas in dermatomyositis, the fertility potential is hampered by disease activity and by alkylating agents. Data regarding rheumatoid arthritis is scarce, gonadal dysfunction observed as consequence of disease activity and antisperm antibodies.
CONCLUSIONS
Reduced fertility potential is not uncommon. Its frequency and severity vary among the different rheumatic diseases. Permanent infertility is rare and often associated with alkylating agent therapy.
Topics: Alkylating Agents; Behcet Syndrome; Dermatomyositis; Gout; Humans; Infertility, Male; Lupus Erythematosus, Systemic; Male; Rheumatic Diseases; Spondylitis, Ankylosing
PubMed: 27120778
DOI: 10.1590/S1677-5538.IBJU.2014.0595