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Clinical and Experimental Rheumatology 2015A number of patients with Behçet's disease (BD) associated with myelodysplastic syndrome (MDS) with or without trisomy 8 have been reported. A high frequency of... (Review)
Review
OBJECTIVES
A number of patients with Behçet's disease (BD) associated with myelodysplastic syndrome (MDS) with or without trisomy 8 have been reported. A high frequency of gastrointestinal (GI) involvement was reported in such patients. The aim of this systematic literature review was to delineate whether GI involvement is an inherent feature of BD associated with MDS, whether these patients do actually have BD rather than GI symptoms related to MDS, and whether the presence of trisomy 8 plays a role in the disease expression of BD associated with MDS.
METHODS
A systematic literature review was performed in PubMed using the keywords (Behçet's disease OR Behçet's syndrome) AND (myelodysplastic syndrome OR trisomy 8) until December 2013.
RESULTS
Data from 39 manuscripts that met the inclusion criteria, reporting on 52 patients were analysed. GI involvement was common in reports from both the Far East and non-Far East countries (75% vs. 50.0%, p=0.15). These patients had typical BD manifestations, except for 1 patient who had only oral ulcers and gastrointestinal involvement. The presence of trisomy 8 seems to be associated with an increased frequency of fever (79.5% vs. 33.3%, p=0.005).
CONCLUSIONS
GI involvement seems to be an inherent feature of BD associated with MDS regardless of geographic differences. Despite the increased frequency of GI involvement in these patients, MDS does not seem to modify the clinical expression of gastrointestinal involvement. Presence of trisomy 8 seems to modify the disease expression with an increased frequency of fever.
Topics: Behcet Syndrome; Chromosomes, Human, Pair 8; Gastrointestinal Diseases; Genetic Predisposition to Disease; Humans; Myelodysplastic Syndromes; Phenotype; Prognosis; Risk Factors; Trisomy
PubMed: 25664843
DOI: No ID Found -
Autoimmunity Reviews Mar 2013Thrombosis is frequent in patients with Behçet's disease (BD), although the exact cause remains uncertain. Some single nucleotide polymorphism (SNP) (G1691A in factor V... (Meta-Analysis)
Meta-Analysis Review
Association of allelic variants of factor V Leiden, prothrombin and methylenetetrahydrofolate reductase with thrombosis or ocular involvement in Behçet's disease: a systematic review and meta-analysis.
UNLABELLED
Thrombosis is frequent in patients with Behçet's disease (BD), although the exact cause remains uncertain. Some single nucleotide polymorphism (SNP) (G1691A in factor V gene, also called factor V Leiden [FVL], G20210A in prothrombin gene and C677T in methyltetrahydrofolate reductase [MTHFR] gene) have been associated with thrombosis and ocular involvement in BD with controversial results.
AIM
To assess the effects of FVL, prothrombin and MTHFR SNP variants in patients with BD and thrombosis and ocular involvement by means of a systematic review and meta-analysis.
METHODS
We retrieved studies analyzing the genotype of the above-mentioned polymorphism among patients with BD. A meta-analysis was conducted in a random effects model and calculations of odds ratio (OR) and confidence intervals (CI) were done. Sensitivity analysis and tests for heterogeneity of the results were performed.
RESULTS
27 previous studies analyzed the association of BD and thrombosis with the FVL, prothrombin and MTHFR polymorphisms. A significant association was found between the possession of the AA or GA genotypes of FVL polymorphism among patients with BD and the presence of any thrombosis (OR=2.51; 95% CI: 1.68, 3.74; P<0.00001). In addition, a significant association was found between the possession of the GA or AA genotypes and the presence of BD (OR=2.67; 95% CI: 1.93. 3.72; P<0.00001) when cases with BD and healthy controls were compared. This association was not found when studies from Turkey were excluded. No association was found between prothrombin and MTHFR SNPs and thrombosis in BD, and no association between any SNP and ocular involvement was shown either.
CONCLUSIONS
Factor V Leiden could be responsible for some thrombotic events in at least Turkish patients. However, this relationship has to be demonstrated from a pathogenic point of view.
Topics: Alleles; Behcet Syndrome; Eye Diseases; Factor V; Female; Genetic Variation; Humans; Male; Methylenetetrahydrofolate Reductase (NADPH2); Prothrombin; Random Allocation; Thrombosis
PubMed: 23207285
DOI: 10.1016/j.autrev.2012.11.001 -
Acta Reumatologica Portuguesa 2014To analyze the prognostic role of antinuclear antibodies (ANA) for the onset of uveitis in the context of juvenile idiopathic arthritis (JIA), its correlation with... (Review)
Review
AIMS
To analyze the prognostic role of antinuclear antibodies (ANA) for the onset of uveitis in the context of juvenile idiopathic arthritis (JIA), its correlation with uveitis course and severity and its prognostic role for the development of arthritis in children with uveitis.
METHODS
We conducted a systematic review analysis of the literature on the prognostic value of ANA on JIA associated uveitis and its complications. We included series published between January 1990 and December 2011 reporting the prognostic value of ANA positivity on uveitis in consecutive patients diagnosed with JIA.
RESULTS
We identified 246 studies from our search, of which 25 were selected for detailed analysis and only 9 fulfilled the inclusion criteria. Some authors have mentioned that uveitis could preceed arthritis and that ANA positivity might represent a predictive factor for subsequent joint involvement. A chronic course and insidious onset of uveitis are predictors for an association with JIA. Although recognized as a possible predictor of uveitis development, presence of positive ANA does not represent a predictor of severity.
CONCLUSIONS
The presence of ANAs seems to be a risk factor for ocular involvement in patients with JIA. These autoantibodies, however, did not have any correlation with the recurrence of either idiopathic anterior uveitis or JIA-related uveitis and cannot be used as a marker to predict the clinical course of ocular inflammation. Any analysis of the literature is subjected to the limitations of each of the studies under evaluation. A large, prospective population-based study of JIA patients would be certainly ideal.
Topics: Antibodies, Antinuclear; Arthritis, Juvenile; Child; Humans; Prognosis; Severity of Illness Index; Uveitis, Anterior
PubMed: 24879943
DOI: No ID Found -
Orphanet Journal of Rare Diseases Aug 2012Uveitis is an autoimmune disease of the eye that refers to any of a number of intraocular inflammatory conditions. Because it is a rare disease, uveitis is often... (Review)
Review
BACKGROUND
Uveitis is an autoimmune disease of the eye that refers to any of a number of intraocular inflammatory conditions. Because it is a rare disease, uveitis is often overlooked, and the possible associations between uveitis and extra-ocular disease manifestations are not well known. The aim of this study was to characterize uveitis in a large sample of patients and to evaluate the relationship between uveitis and systemic diseases.
METHODS
The present study is a cross-sectional study of a cohort of patients with uveitis. Records from consecutive uveitis patients who were seen by the Uveitis Service in the Department of Ophthalmology at the Medical University of Vienna between 1995 and 2009 were selected from the clinical databases. The cases were classified according to the Standardization of Uveitis Nomenclature Study Group criteria for Uveitis.
RESULTS
Data were available for 2619 patients, of whom 59.9% suffered from anterior, 14.8% from intermediate, 18.3% from posterior and 7.0% from panuveitis. 37.2% of all cases showed an association between uveitis and extra-organ diseases; diseases with primarily arthritic manifestations were seen in 10.1% of all cases, non-infectious systemic diseases (i.e., Behçet´s disease, sarcoidosis or multiple sclerosis) in 8.4% and infectious uveitis in 18.7%. 49.4% of subjects suffering from anterior uveitis tested positively for the HLA-B27 antigen. In posterior uveitis cases 29% were caused by ocular toxoplasmosis and 17.7% by multifocal choroiditis.
CONCLUSION
Ophthalmologists, rheumatologists, infectiologists, neurologists and general practitioners should be familiar with the differential diagnosis of uveitis. A better interdisciplinary approach could help in tailoring of the work-up, earlier diagnosis of co-existing diseases and management of uveitis patients.
Topics: Age of Onset; Cohort Studies; Cross-Sectional Studies; Female; Humans; Male; Rare Diseases; Uveitis
PubMed: 22932001
DOI: 10.1186/1750-1172-7-57 -
Oral Diseases Nov 2011Recurrent aphthous stomatitis (RAS) is the most common idiopathic intraoral ulcerative disease in the USA. Aphthae typically occur in apparently healthy individuals,... (Review)
Review
Recurrent aphthous stomatitis (RAS) is the most common idiopathic intraoral ulcerative disease in the USA. Aphthae typically occur in apparently healthy individuals, although an association with certain systemic diseases has been reported. Despite the unclear etiopathogenesis, new drug trials are continuously conducted in an attempt to reduce pain and dysfunction. We investigated four controversial topics: (1) Is complex aphthosis a mild form of Behçet's disease (BD)? (2) Is periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome a distinct medical entity? (3) Is RAS associated with other systemic diseases [e.g., celiac disease (CD) and B12 deficiency]? (4) Are there any new RAS treatments? Results from extensive literature searches, including a systematic review of RAS trials, suggested the following: (1) Complex aphthosis is not a mild form of BD in North America or Western Europe; (2) Diagnostic criteria for PFAPA have low specificity and the characteristics of the oral ulcers warrant further studies; (3) Oral ulcers may be associated with CD; however, these ulcers may not be RAS; RAS is rarely associated with B12 deficiency; nevertheless, B12 treatment may be beneficial, via mechanisms that warrant further study; (4) Thirty-three controlled trials published in the past 6 years reported some effectiveness, although potential for bias was high.
Topics: Behcet Syndrome; Celiac Disease; Evidence-Based Dentistry; Familial Mediterranean Fever; Humans; Pharyngitis; Sialadenitis; Stomatitis, Aphthous; Syndrome; Vitamin B 12 Deficiency
PubMed: 21812866
DOI: 10.1111/j.1601-0825.2011.01840.x -
Clinical and Experimental Rheumatology Sep 2022The assessment of quality of life (QoL) in Behçet's disease (BD) patients has been a surrogate of disease outcomes, but a wider impact on the patient's lifestyle has... (Review)
Review
OBJECTIVES
The assessment of quality of life (QoL) in Behçet's disease (BD) patients has been a surrogate of disease outcomes, but a wider impact on the patient's lifestyle has not been considered. This systematic review aims to provide an overview of the existing tools specifically adopted to explore the QoL in BD patients.
METHODS
A systematic literature review was conducted using 2 electronic databases, according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. A combination of BD and QoL-related search terms were used. All articles were screened by 3 independent reviewers for title, abstract and full text level. Studies investigating QoL in BD patients were included.
RESULTS
64 papers of 497 records were retained. Data about 7,449 patients with a BD diagnosis and QoL evaluation were collected. 47 different tools to evaluate QoL were detected. The mean number of tools adopted in each study was 2.14±1.34. General QoL and psychological and social impact were investigated in 68.75% and 54.69% respectively. The correlation with disease activity was investigated in 71.86%.
CONCLUSIONS
The assessment of QoL in BD patients may provide a fundamental measurement for health to evaluate the outcome of interventions for BD patients. The adoption of a single validated QoL tool, developed including the BD patient's perspective, may provide an accurate and effective assessment, ensure the comparison within different cohorts, and set standardised values to define QoL level in BD patients.
Topics: Behcet Syndrome; Humans; Quality of Life
PubMed: 36106544
DOI: 10.55563/clinexprheumatol/sian1b -
The Cochrane Database of Systematic... 2000To determine the effects of available pharmacological interventions in treating the different clinical features of Behcet's syndrome. (Review)
Review
OBJECTIVES
To determine the effects of available pharmacological interventions in treating the different clinical features of Behcet's syndrome.
SEARCH STRATEGY
We searched the Cochrane Musculoskeletal Group's trials register, the Cochrane Controlled Trials Register, and Medline up to January 1998. The computer search was complemented by a hand search of all bibliographic references from the reference lists of included trials. Principal investigators were contacted to seek unpublished literature. All languages were included.
SELECTION CRITERIA
Studies were eligible if they fulfilled all of the four following criteria: 1. Randomized controlled trials, single or double-blind; 2. Participants were patients with Behcet's Syndrome as defined by the International Study Group, 1990 (Int Study Group, 1990); 3. Interventions included any pharmacological therapy compared to placebo or some other pharmacological intervention for the treatment of Behcet's syndrome. 4. Outcome measures included active ocular inflammatory processes, arthritis, mucocutaneous manifestations (oral ulcer, genital ulcer, erythema nodosum), laboratory changes and major events such as adverse effects and death.
DATA COLLECTION AND ANALYSIS
The 32 potentially relevant references were assessed by two independent reviewers (MA, AS) according to the inclusion criteria. Ten trials fit the inclusion criteria and were included in this review. From the 10 included trials, data were independently extracted by the same two observers and crosschecked. The quality of the included trials was assessed independently by two observers (MA, AS) using a validated scale (Jadad 1996). For dichotomous measures, the treatment effect for each trial was calculated using a fixed effect model [Peto model (Petitti 1994)]. The weighted mean differences were based, if available, on end-of-trial results. The analysis was conducted separately for each different intervention. Since the trials could not be pooled it was not possible to carry out a sensitivity analysis by quality scores or a subgroup analysis by drug dosages. Because of this lack of comparability across trials and the small number of trials, we could not conduct a heterogeneity test or a funnel plot.
MAIN RESULTS
Ten trials and 679 patients were included. The main results were the lack of efficacy of some of the classic treatments for Behcet's syndrome, including colchicine, cyclophosphamide and steroids for eye involvement, azapropazone and colchicine for arthritis and acyclovir, colchicine and topical interpheron for aphthas. The results confirm the protective effects of cyclosporine and azathioprine for eye involvement and benzathine-penicillin for arthritis.
REVIEWER'S CONCLUSIONS
We conclude that further randomized, placebo-controlled, double-blind trials should be carried out to compare cyclosporine, azathioprine and benzathine-penicillin versus placebo in order to make the results generalizable and comparable.
Topics: Acyclovir; Anti-Infective Agents; Apazone; Azathioprine; Behcet Syndrome; Colchicine; Cyclosporine; Humans; Immunosuppressive Agents; Interferon-alpha; Penicillins
PubMed: 10796413
DOI: 10.1002/14651858.CD001084 -
Ophthalmology May 2024Sympathetic ophthalmia (SO) is a sight-threatening granulomatous panuveitis caused by a sensitizing event. Primary enucleation or primary evisceration, versus primary... (Meta-Analysis)
Meta-Analysis Review
TOPIC
Sympathetic ophthalmia (SO) is a sight-threatening granulomatous panuveitis caused by a sensitizing event. Primary enucleation or primary evisceration, versus primary repair, as a risk management strategy after open-globe injury (OGI) remains controversial.
CLINICAL RELEVANCE
This systematic review was conducted to report the incidence of SO after primary repair compared with that of after primary enucleation or primary evisceration. This enabled the reporting of an estimated number needed to treat.
METHODS
Five journal databases were searched. This review was registered with International Prospective Register of Systematic Reviews (identifier, CRD42021262616). Searches were carried out on June 29, 2021, and were updated on December 10, 2022. Prospective or retrospective studies that reported outcomes (including SO or lack of SO) in a patient population who underwent either primary repair and primary enucleation or primary evisceration were included. A systematic review and meta-analysis were carried out in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Random effects modelling was used to estimate pooled SO rates and absolute risk reduction (ARR).
RESULTS
Eight studies reporting SO as an outcome were included in total. The included studies contained 7500 patients and 7635 OGIs. In total, 7620 OGIs met the criteria for inclusion in this analysis; SO developed in 21 patients with OGI. When all included studies were pooled, the estimated SO rate was 0.12% (95% confidence interval [CI], 0.00%-0.25%) after OGI. Of 779 patients who underwent primary enucleation or primary evisceration, no SO cases were reported, resulting in a pooled SO estimate of 0.05% (95% CI, 0.00%-0.21%). For primary repair, the pooled estimate of SO rate was 0.15% (95% CI, 0.00%-0.33%). The ARR using a random effects model was -0.0010 (in favour of eye removal; 95% CI, -0.0031 [in favor of eye removal] to 0.0011 [in favor of primary repair]). Grading of Recommendations, Assessment, Development, and Evaluations analysis highlighted a low certainty of evidence because the included studies were observational, and a risk of bias resulted from missing data.
DISCUSSION
Based on the available data, no evidence exists that primary enucleation or primary evisceration reduce the risk of secondary SO.
FINANCIAL DISCLOSURE(S)
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
Topics: Humans; Ophthalmia, Sympathetic; Retrospective Studies; Eye Enucleation; Eye Injuries; Eye Evisceration
PubMed: 38086434
DOI: 10.1016/j.ophtha.2023.12.006 -
Rheumatology (Oxford, England) May 2012To investigate comprehensively the relationships between Behçet's disease (BD) clinical features and HLA-B51 or HLA-B5 (HLA-B51/B5) status using meta-analyses. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To investigate comprehensively the relationships between Behçet's disease (BD) clinical features and HLA-B51 or HLA-B5 (HLA-B51/B5) status using meta-analyses.
METHODS
Relevant publications were identified by a systematic literature search. Eligible studies had to provide frequencies for one or more BD characteristics according to HLA-B51/B5 status. Pooled relative risks (RRs) were calculated by random-effects meta-analysis for those BD characteristics for which five or more relevant studies were identified. Between-study variability was assessed with I(2) and Q-statistics, and modelled using meta-regression.
RESULTS
Among the 859 publications evaluated, 72 (representing 74 study populations) met eligibility criteria. Pooled RRs (95% CIs) of the association of HLA-B51/B5 with the 14 analysed clinical characteristics were male sex 1.14 (1.05, 1.23); eye involvement 1.13 (1.06, 1.21); genital ulcers 1.07 (1.01, 1.14); skin involvement 1.10 (1.03, 1.16); erythema nodosum 1.11 (0.96, 1.29); pseudofolliculitis 1.07 (0.93, 1.23); positive pathergy test 1.05 (0.94, 1.17); joint involvement 0.94 (0.86, 1.04); neurological involvement 0.95 (0.71, 1.27); gastrointestinal involvement 0.70 (0.52, 0.94); thrombophlebitis 1.17 (0.77, 1.76); vascular involvement 1.00 (0.68, 1.47); chest involvement 1.55 (0.75, 3.20) and orchiepididymitis 1.13 (0.59, 2.15). For most of the analysed outcomes, between-study heterogeneity was low or absent and most of the meta-regression models were statistically non-significant.
CONCLUSION
The results of these meta-analyses showed that, in BD, HLA-B51/B5 carriage predominates in males and is associated with moderately higher prevalences of genital ulcers, ocular and skin manifestations, and a decreased prevalence of gastrointestinal involvement.
Topics: Behcet Syndrome; Genotype; HLA-B Antigens; HLA-B51 Antigen; Humans; Male
PubMed: 22240504
DOI: 10.1093/rheumatology/ker428 -
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