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Annals of Hematology Jun 2020The association between myelodysplastic syndrome (MDS) and Behçet syndrome (BS) is recognized for over 25 years. High frequency of trisomy 8 and intestinal ulcers are...
The association between myelodysplastic syndrome (MDS) and Behçet syndrome (BS) is recognized for over 25 years. High frequency of trisomy 8 and intestinal ulcers are striking features of this association. There are no recommendations for how these patients should be treated. A systematic literature review was performed in PubMed using the keyword combination "(((((intestinal) OR gastrointestinal) OR ulcer) OR Behcet*)) AND ((myelodysplastic syndrome) OR MDS)" in March 2019. Our aim was to gain insight regarding clinical responses to individual treatment modalities. A recent case was also presented and included in the analysis. Data from 41 articles reporting on a total of 53 patients carried adequate information to assess treatment responses. Glucocorticoids provided benefit in 23 of 43 patients. Azacitidine, decitabine, thalidomide, and cyclosporine contributed to a clinical improvement in 4/6, 2/3, 3/4, and 5/8 patients respectively. Hematopoietic stem cell transplantation was successful in 9 of 13 patients. With the use of TNF inhibitors, azathioprine, and mesalamine derivatives, clinical improvement was observed in 3/11, 0/4, and 6/18 patients respectively. Patients with MDS and BS-like features who are resistant to glucocorticoids have so far benefited more from treatment approaches directed at MDS, rather than the immunosuppressive agents used for BS.
Topics: Aged; Behcet Syndrome; Diagnosis, Differential; Humans; Immunosuppressive Agents; Male; Myelodysplastic Syndromes; Treatment Outcome
PubMed: 32140893
DOI: 10.1007/s00277-020-03951-5 -
PloS One 2020Behçet's disease (BD) is a multifactorial systemic inflammatory disease of unknown aetiology characterised by several clinical manifestations including vascular... (Meta-Analysis)
Meta-Analysis
Behçet's disease (BD) is a multifactorial systemic inflammatory disease of unknown aetiology characterised by several clinical manifestations including vascular involvements (i.e., both arterial and venous thrombosis). Antiphospholipid antibodies (aPLs)-including anticardiolipin (aCL), anti-β2-glycoprotein I (β2-GPI) antibodies and lupus anticoagulant (LA) are detected in systemic autoimmune diseases which contribute to thrombosis. The aim of this systematic review and meta-analysis was to evaluate the prevalence of aPLs in patients with BD as compared to controls. A protocol was registered in PROSPERO (Registration No. CRD42018088125) and a systematic literature search was conducted through PubMed, Web of Science, Embase, Scopus and ScienceDirect databases. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated using random-effects model. Quality assessment was carried out by using the modified 9-star Newcastle-Ottawa Scale (NOS). Publication bias was evaluated via visualisation of contour- enhanced and trim and fill funnel plots along with Begg's and Egger's tests. We included ten case-control studies (a total of 999 participants from 380 BD patients and 619 controls) based on the inclusion criteria. The prevalence of aCL (OR: 12.10, 95% CI: 5.15-28.41, p<0.00001) and anti-β2-GPI antibodies (OR: 23.57, 95% CI: 1.31-423.63, p = 0.03) were statistically significant, however, the prevalence of LA was not significant (OR: 13.77, 95% CI: 0.65-293.59, p = 0.09). The results remained statistically significant from different sensitivity analyses which represented the robustness of this meta-analysis. According to the NOS, 50.0% of the studies were considered as of high methodological quality (low risk of bias). No significant publication bias was detected from contour-enhanced and trim and fill funnel plots or Begg's and Egger's tests. This meta-analysis established that there is a significantly high prevalence of aPLs (i.e., aCL and anti-β2-GPI antibodies) in patients with BD when compared to controls.
Topics: Antibodies, Anticardiolipin; Antibodies, Antiphospholipid; Behcet Syndrome; Humans; Prevalence; beta 2-Glycoprotein I
PubMed: 31929597
DOI: 10.1371/journal.pone.0227836 -
International Ophthalmology May 2020To review the multimodal imaging patterns of posterior syphilitic uveitis.
PURPOSE
To review the multimodal imaging patterns of posterior syphilitic uveitis.
METHODS
A systematic review.
RESULTS
The percentage of syphilis has started to increase again: The World Health Organization has reported 12 million new cases of syphilis each year. In addition, syphilis was responsible for 0.3% of deaths globally in 2002. Eye manifestations happen prevalently in secondary and tertiary stages of syphilis, even though ocular involvement can occur in all stages. Syphilis has the nickname: "the great imitator" since it has no unique clinical presentation, even though posterior uveitis is considered the most common form. Syphilis is known as "the great imitator," making its diagnosis in the presence of posterior uveitis particularly challenging as it presents similarly to other ocular conditions such as acute retinal necrosis. However, with the advent of multimodal imaging some particular patterns of pre-retinal, retinal, retinochoroidal and optic nerve involvement from syphilis can be identified to guide the diagnosis and the laboratory workup.
CONCLUSION
This review highlights the various patterns of pre-retinal precipitates, multifocal retinitis, retinochoroiditis (confluent and placoid) and optic neuritis caused by syphilis, the appropriate laboratory work to be obtained and the treatment to be initiated.
Topics: Anti-Bacterial Agents; Eye Infections, Bacterial; Humans; Multimodal Imaging; Prognosis; Syphilis; Uvea; Uveitis, Posterior; Visual Acuity
PubMed: 31927680
DOI: 10.1007/s10792-020-01285-9 -
Journal of Ophthalmic Inflammation and... Jan 2020This study aims to determine if the intravitreal dexamethasone implant (DEX implant, Ozurdex; Allergan, Inc., Irvine, California) is effective for treating intermediate,...
BACKGROUND
This study aims to determine if the intravitreal dexamethasone implant (DEX implant, Ozurdex; Allergan, Inc., Irvine, California) is effective for treating intermediate, posterior, and panuveitis as a monotherapy or adjunctive treatment to systemic immunomodulatory therapies.
METHODS
A systematic review using MEDLINE, EMBASE, and PubMed database searches was conducted with the Oxford Centre for Evidence-based Medicine Levels of Evidence criteria to select publications. Available background information and patient data from each study was tabulated. Outcomes studied were central retinal thickness (CRT), best corrected visual acuity, intraocular inflammation (anterior chamber cells, vitreous haze), number of patients with prior and concomitant immunomodulatory treatments, intraocular pressure (IOP) elevation (≥ 25 mmHg), and other adverse effects associated with the implant.
RESULTS
One hundred ninety-five (61.51%) patients had previous immunomodulatory treatment while 232 (64.8%) were treated with concomitant immunomodulatory therapy with the DEX implant. CRT decreased by an average of 198.65 μm (42.74%). Visual acuity improved to an average of 0.451 (logMAR) or 20/57 (Snellen) which is a 43.11% improvement from baseline. One hundred seventy-three (59%) of eyes were quiescent at the end of the trials, of which 40 (13.7%) previously inflamed eyes became quiescent. Elevated IOP occurred in 91 (20.6%). The most common adverse events were cataract/posterior subcapsular opacities in 47 (11.03%) patients and conjunctival hemorrhage in 24 (5.44%) patients.
CONCLUSIONS
The DEX implant is an effective medication for the treatment of posterior segment uveitis, uveitic macular edema, and results in improved visual acuity. Development of elevated IOP and cataract should be closely monitored as they are tangible risks associated with the DEX implant. This study was not able to determine whether the DEX implant was more effective as a monotherapy or as an adjunctive therapy to systemic immunomodulatory treatment.
PubMed: 31925591
DOI: 10.1186/s12348-019-0189-4 -
Clinical and Experimental Rheumatology 2019Systemic therapy aimed at suppressing the diffuse inflammation in the vessel wall is the major treatment modality for venous thrombosis in Behçet's syndrome (BS)....
OBJECTIVES
Systemic therapy aimed at suppressing the diffuse inflammation in the vessel wall is the major treatment modality for venous thrombosis in Behçet's syndrome (BS). Endovascular and/or surgical interventions are also used. We here report five patients who were referred to our clinic after having such interventions and also present a literature review to assess the outcome of invasive procedures for venous thrombosis in BS.
METHODS
Our patients were presented and a literature search for endovascular and/or surgical interventions in Pub-Med was performed. Recanalisation, reocclusion or other complications were assessed as outcomes.
RESULTS
Five BS patients with lower extremity thrombosis were referred to our clinic with post thrombotic syndrome due to incomplete recanalisation or infectious complication after endovascular interventions. Twenty-one articles reporting on 36 patients were found suitable for review. There were totally 21 lower extremity venous intervention cases, 14 of which had failure such as complication, reocclusion or incomplete recanalisation. Reocclusions occurred in 10 patients and reinterventions to 8 of them could restore flow only in 4 cases. Ileal infarct and vena cava wall-duodenal perforation were major complications. Invasive procedures of 8 abdominal thrombosis cases resulted with death due to ileus in one patient, and reocclusion in another. Seven of the 12 upper extremity/superior vena cava thrombosis cases resulted with reocclusions.
CONCLUSIONS
Endovascular and surgical interventions seemed to be unsuccessful because of recurrent infectious and vascular complications in 22 (53.6%) of 41 patients with venous thrombosis. The indication of these procedures is controversial. Their economic burden on the healthcare system must be considered.
Topics: Behcet Syndrome; Combined Modality Therapy; Humans; Thrombolytic Therapy; Thrombosis; Treatment Outcome; Venous Thrombosis
PubMed: 31856938
DOI: No ID Found -
Immunologic Research Oct 2019The aim of this study was to determine whether interleukin-10 (IL-10) polymorphisms are associated with susceptibility to Behcet's disease (BD). A meta-analysis was... (Meta-Analysis)
Meta-Analysis
The aim of this study was to determine whether interleukin-10 (IL-10) polymorphisms are associated with susceptibility to Behcet's disease (BD). A meta-analysis was conducted, examining the associations between the IL-10 - 1082 G/A, - 819 C/T, and - 592 C/A polymorphisms and BD in various ethnic groups. Fourteen studies involving 5992 patients and 8966 controls were considered in the meta-analysis. Meta-analysis of the IL-10 - 1082 G/A polymorphism showed no association between BD and the - 1082 G allele (odds ratio (OR) = 0.951, 95% confidence interval (CI) = 0.790-1.146, p = 0.601). Meta-analysis of the IL-10 - 819 C/T polymorphism revealed a significant association between BD and the - 819 C allele (OR = 0.751, 95% CI = 0.684-0.825, p < 0.001) in all study subjects. Stratification by ethnicity indicated a significant association between the - 819 C allele and BD in Turkish (OR = 0.779, 95% CI = 0.709-0.856, p < 0.001) and Asian (OR = 0.676, 95% CI = 0.631-0.725, p < 0.001), but not in Middle Eastern populations. Meta-analysis of the - 592 C allele showed an association with BD in all study subjects (OR = 0.792, 95% CI = 0.662-0.948, p = 0.011) Stratification by ethnicity indicated a significant association between the - 592 C allele and BD in Asian populations (OR = 0.656, 95% CI = 0.512-0.841, p = 0.001). This meta-analysis showed that the IL-10 - 819 C/T and - 592 C/A polymorphisms are associated with BD susceptibility, especially in Asian population.
Topics: Alleles; Asian People; Behcet Syndrome; Female; Genetic Predisposition to Disease; Humans; Interleukin-10; Male; Polymorphism, Single Nucleotide
PubMed: 31721090
DOI: 10.1007/s12026-019-09102-x -
Medicine Nov 2019Several studies have reported the association of Behcet disease (BD) with the risk of diverse kinds of cancers. However, its association is controversial. Therefore, we... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Several studies have reported the association of Behcet disease (BD) with the risk of diverse kinds of cancers. However, its association is controversial. Therefore, we conducted a bioinformatics-analysis to explore any possible association.
METHODS
We obtained relevant findings published before October 2018 through literature survey of the PubMed, EMBASE, and Web of Science databases. STATA 12.0 software was used for statistical analysis.
RESULTS
After screening, the meta-analysis comprised 5 studies. We observed a significant positive association between BD and enhanced malignancy risk (pooled relative risk [RR], 1.19; 95% confidence interval [CI]: 1.09-1.30), especially for hematological cancer (pooled RR, 2.58; 95% CI: 1.61-3.55) and thyroid cancer (pooled RR, 1.25; 95% CI: 1.04-1.47). However, high heterogeneity was also observed in the results (I = 81.3%). Subgroup analysis indicated that female BD patients from Korean population are at highest predisposition to overall malignancy. Besides, publication bias was not observed with our choice of surveys.
CONCLUSION
We conclude that patients suffering from BD have an overall increased risk for malignancy. Greater numbers of exhaustive temporal studies are essential for definitive inferences.
Topics: Behcet Syndrome; Computational Biology; Female; Hematologic Neoplasms; Humans; Male; Neoplasms; Risk; Risk Factors; Thyroid Neoplasms
PubMed: 31689818
DOI: 10.1097/MD.0000000000017735 -
The British Journal of Ophthalmology Aug 2020Biologics are increasingly used in management of Behçet's Disease (BD) including ocular BD, but the evidence base is limited, mostly from studies of uveitis and BD...
BACKGROUND
Biologics are increasingly used in management of Behçet's Disease (BD) including ocular BD, but the evidence base is limited, mostly from studies of uveitis and BD manifestations.
OBJECTIVE
To review the evidence base for biologics in the treatment of ocular BD.
METHODS
Systematic literature search was made using exploded key words-Behçet's, ocular, biologics in MEDLINE, Cochrane library, Database of Abstracts Reviews and Effects, Clinical Trials.gov, Science Direct and Google Scholar. There was no limitation on region, language or date (Search updated 16th October 2018). Literature retrieval was restricted to randomised controlled trials (RCTs) of biologics.
RESULTS
Of 237 papers retrieved, eight met the inclusion criteria. RCTs on interferon alpha 2a (INF-α 2a), adalimumab, secukinumab, gevokizumab, rituximab and daclizumab were retrieved (two for adalimumab and gevokizumab). The outcome measures were not met for secukinumab, daclizumab and gevokizumab. Rituximab and INF-α 2a showed promising preliminary results but sufficiently powered RCTs are needed to provide adequate evidence of efficacy. The RCTs on adalimumab did not evaluate efficacy for BD uveitis specifically, hence are of limited value for this review.
CONCLUSION
Some biologics show promise in treating BD uveitis, but more RCTs are needed for firm conclusions about efficacy. A phase IV study or, registry of adalimumab could provide data on its efficacy in BD uveitis compared to other forms.
Topics: Adalimumab; Antibodies, Monoclonal, Humanized; Behcet Syndrome; Biological Factors; Biological Therapy; Daclizumab; Humans; Interferon-alpha; Rituximab; Uveitis
PubMed: 31676596
DOI: 10.1136/bjophthalmol-2019-314154 -
Rheumatology International Jan 2020Behçet's syndrome (BS) is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin...
Behçet's syndrome (BS) is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis; articular, vascular, gastroenteric and neurological involvement may also occur. The multi-organ involvement and the wide range of clinical spectrum make the diagnosis of BS challenging. As other systemic chronic diseases, BS can affect all aspects of patients' life, including sexual dysfunction (SD). So far, SD has been deeply studied among patients affected by many rheumatic diseases; however, little is known about the prevalence and the characteristics of SD among BS patients. The present work was aimed at providing a systematic literature review of the literature published on SD and BS. We carried out a systematic search in PubMed and Scopus based on controlled terms (MeSH) and keywords to identify literature data on SD in BS. The systematic search was independently performed by two clinicians based on the controlled medical subject heading terms for Behcet syndrome and SD. Systematic database search identified 32 records. Screening by title and abstract was then conducted and a total of 10 articles were eligible for full text assessment, five studies explored SD in male patients with BS, 3 studies in females and 2 works reported data on both genders. Globally, the systematic literature review results have underlined that SD seems frequent in BS patients. Both female and male patients experienced a significant correlation between SD and depression. The studies investigating sexuality in BS seem to demonstrate that in these patients SD may be mainly related to depression rather than to active organic manifestations.
Topics: Behcet Syndrome; Erectile Dysfunction; Female; Humans; Male; Sexual Dysfunction, Physiological; Sexual Dysfunctions, Psychological
PubMed: 31595309
DOI: 10.1007/s00296-019-04455-w -
Neuropsychology Review Dec 2019Behçet's disease (BD) is a vascular, inflammatory multisystem disorder with neuro-Behçet's (NBD) diagnosed in a subset of patients with neurological manifestations....
Behçet's disease (BD) is a vascular, inflammatory multisystem disorder with neuro-Behçet's (NBD) diagnosed in a subset of patients with neurological manifestations. The objective of this review was to determine whether neurocognitive dysfunction is observed in BD, in which neurocognitive domains, and whether there are differences in rates of dysfunction observed between BD and NBD groups. Studies of any methodology were included that reported results from standardized neurocognitive assessment measures in participants with BD or NBD. Twelve group comparison studies met the criteria for inclusion in the review (totalling 284 BD and 157 NBD participants), as well as 17 case study/series papers (11 BD, 35 NBD). Issues with blinding, incomplete data reporting and selective reporting bias were found across the group and case study/series papers, as well as inadequate statistical adjustment for multiple comparisons in the group studies, and the lack of the use of appropriate norms or adjustment for premorbid ability in the case series/studies papers. These quality issues impacted on the conclusions that could be drawn from the current literature. Neurocognitive dysfunction was found in NBD compared to health controls (HC) in a higher proportion of results across studies, than in comparisons between BD and HC groups. The domains in which neurocognitive attenuation was most often reported were visual spatial ability, working memory and acquired knowledge, with more than 25% of these results showing significantly lower functioning in both the BD and NBD groups compared to HC. More than 25% of the processing speed and long-term memory encoding and retrieval results were also lower for the NBD group, compared to HC. Group comparisons between NBD and multiple sclerosis participants indicated few significant differences in neurocognitive test results. The majority of case study/series participants were found to have some degree of attenuated neurocognitive functioning, as defined by case study/series authors.
Topics: Behcet Syndrome; Brain; Humans; Mental Status and Dementia Tests
PubMed: 31494834
DOI: 10.1007/s11065-019-09416-5