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Rheumatology (Oxford, England) Dec 2022A decline in the frequency of AA amyloidosis secondary to RA and infectious diseases has been reported. We aimed to determine the change in the frequency of AA...
OBJECTIVE
A decline in the frequency of AA amyloidosis secondary to RA and infectious diseases has been reported. We aimed to determine the change in the frequency of AA amyloidosis in our Behçet's syndrome (BS) patients and to summarize the clinical characteristics of and outcomes for our patients, and also those identified by a systematic review.
METHODS
We identified patients with amyloidosis in our BS cohort (as well as their clinical and laboratory features, treatment, and outcome) through a chart review. The primary end points were end-stage renal disease and death. The prevalence of AA amyloidosis was estimated separately for patients registered during 1976-2000 and those registered during 2001-2017, in order to determine whether there was any change in the frequency. We searched PubMed and EMBASE for reports on BS patients with AA amyloidosis. Risk of bias was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) tool.
RESULTS
The prevalence of AA amyloidosis was 0.62% (24/3820) in the earlier cohort and declined to 0.054% (3/5590) in the recent cohort. The systematic review revealed 82 cases in 42 publications. The main features of patients were male predominance and a high frequency of vascular involvement. One-third of patients died within 6 months after diagnosis of amyloidosis.
CONCLUSION
The frequency of AA amyloidosis has decreased in patients with BS, which is similar to the decrease observed for AA amyloidosis due to other inflammatory and infectious causes. However, AA amyloidosis is a rare, but potentially fatal complication of BS.
Topics: Humans; Male; Female; Behcet Syndrome; Retrospective Studies; Follow-Up Studies; Amyloidosis
PubMed: 35657376
DOI: 10.1093/rheumatology/keac223 -
Arthritis Care & Research May 2023The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to update the current guidelines, and the objective here was to produce...
OBJECTIVE
The Multinational Interdisciplinary Working Group for Uveitis in Childhood identified the need to update the current guidelines, and the objective here was to produce this document to guide clinicians managing children with juvenile idiopathic arthritis-associated uveitis (JIAU) and idiopathic chronic anterior uveitis (CAU).
METHODS
The group analyzed the literature published between December 2014 and June 2020 after a systematic literature review conducted by 2 clinicians. Pediatric rheumatologists were paired with ophthalmologists to review the eligible 37 publications. The search criteria were selected to reflect those used for the 2018 Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) recommendations, in order to provide an update, rather than a replacement for that publication. The summary of the current evidence for each SHARE recommendation was presented to the expert committee. These recommendations were then discussed and revised during a video consensus meeting on January 22, 2021, with 14 voting participants, using a nominal group technique to reach consensus.
RESULTS
JIAU treatment was extended to include CAU. Fourteen recommendations regarding treatment of JIAU und CAU with >90% agreement were accepted.
CONCLUSION
An update to the previous 2018 SHARE recommendations for the treatment of children with JIAU with the addition of CAU was created using an evidence-based consensus process. This guideline should help support clinicians to care for children and young people with CAU.
Topics: Child; Humans; Adolescent; Arthritis, Juvenile; Uveitis; Europe; Rheumatology; Uveitis, Anterior
PubMed: 35638697
DOI: 10.1002/acr.24963 -
European Journal of Clinical... Aug 2022Behçet disease (BD) is a systemic vasculitis. In addition to the mucocutaneous lesions, the vascular injury of pathophysiology in BD is theoretically correlated with... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Behçet disease (BD) is a systemic vasculitis. In addition to the mucocutaneous lesions, the vascular injury of pathophysiology in BD is theoretically correlated with cardiovascular diseases. This study aimed to elucidate the association of BD with ischaemic heart diseases (IHDs) and stroke.
METHODS
A systematic search of PubMed, Embase, Web of Science and Cochrane Library databases was performed for all relevant observational studies from database inception until 10 July 2021. No language restriction was applied. A random-effects model was used for meta-analysis.
RESULTS
A total of six observational studies consisting of three cohort studies, two cross-sectional studies and one study with both study designs were adopted in the meta-analysis. The numbers of patients with BD and healthy controls were 9,813 and 41,802, respectively. The pooled analysis demonstrated no significant association between BD and IHD. By contrast, we found that patients with BD had a significantly higher risk of stroke (adjusted hazard ratio, 2.083; 95% confidence interval, 1.339-3.240; p = 0.001) than healthy controls. We observed substantial heterogeneity across studies in few meta-analyses, but no significant publication bias was detected in any of the meta-analyses.
CONCLUSIONS
BD was significantly associated with stroke but not IHD. Physicians should be aware of possible vascular and neurological complications during care of patients with BD.
Topics: Behcet Syndrome; Cross-Sectional Studies; Humans; Myocardial Ischemia; Risk Factors; Stroke
PubMed: 35342952
DOI: 10.1111/eci.13778 -
Ocular Immunology and Inflammation Apr 2023To compare the outcomes of mycophenolate mofetil (MMF) versus methotrexate (MTX) in non-infectious ocular inflammatory disease (NIOID). (Meta-Analysis)
Meta-Analysis
PURPOSE
To compare the outcomes of mycophenolate mofetil (MMF) versus methotrexate (MTX) in non-infectious ocular inflammatory disease (NIOID).
METHODS
The study was performed as per the PRISMA Guidelines. A search identified all studies comparing MMF versus MTX in NIOID. Treatment result and side effects were primary outcomes.
RESULTS
Four studies enrolling 905 patients were identified. There was no significant difference between MMF and MTX groups in overall treatment success (OR = 0.97, P = .96), treatment failure (OR = 0.86, P = .85). MTX showed a significantly improved effect in cases involving posterior uveitis and panuveitis (OR = 0.41, P = .003). In addition, MTX was associated with a faster median time to treatment success and had less side effects when compared to MTX, however this was not significant. For secondary outcomes, no significant difference was found in visual acuity and resolution of macular oedema.
CONCLUSION
MMF is comparable to MTX in the treatment of NIOID.
Topics: Humans; Methotrexate; Immunosuppressive Agents; Inflammation; Mycophenolic Acid; Enzyme Inhibitors; Treatment Outcome; Retrospective Studies
PubMed: 35201968
DOI: 10.1080/09273948.2022.2034166 -
Current Rheumatology Reviews 2022Behcet's disease (BD) is a chronic multisystem inflammatory disease classified as Variable Vessel Vasculitis with unclear etiology. We designed this systematic review... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Behcet's disease (BD) is a chronic multisystem inflammatory disease classified as Variable Vessel Vasculitis with unclear etiology. We designed this systematic review and meta-analysis to evaluate vitamin D status in Behcet's disease patients with this background.
METHODS
We performed this systematic review and meta-analysis according to PRISMA guidelines. We included all observational studies in humans published in English, evaluating the association of 25(OH)D concentrations in Behcet's patients. Two reviewers (HRK and AE) independently searched the databases and screened articles based on their titles and abstracts. A third reviewer resolved all disagreements. We performed analysis using Cochrane Program Review Manager Version 5.3. The protocol for this review was registered on PROSPERO (CRD42020197426).
RESULTS
A total of 341 publications were initially identified according to the search strategy. Finally, 12 publications were included in the meta-analysis. We performed this meta-analysis on 1265 participants from different studies with a sample size ranging from 63 to 224 individuals. In studies comparing active and inactive subgroups of patients with Behcet's disease, we found a significantly lower serum level of vitamin D in patients with Active BD (-0.4; 95% CI: -0.61, -0.25; p<0.001). We found that the serum level of vitamin D in Behcet's disease is significantly higher than in health controls (0.5; 95% CI: 0.15, 0.50; p=0.001).
CONCLUSION
We demonstrated that the existing evidence is consistent with the hypothesis that an increased serum level of vitamin D would be associated with a substantially lower risk of active Behcet's disease.
Topics: Behcet Syndrome; Humans; Vasculitis; Vitamin D
PubMed: 35184713
DOI: 10.2174/1573397118666220218112841 -
Graefe's Archive For Clinical and... Jul 2022We conducted a systematic review to evaluate the outcome of macular hole (MH) treatment in eyes with uveitis. (Review)
Review
OBJECTIVE
We conducted a systematic review to evaluate the outcome of macular hole (MH) treatment in eyes with uveitis.
DATA SOURCE
We searched PubMed and Embase databases from inception through August 15, 2021.
STUDY SELECTION
We included eyes with MHs secondary to uveitis that were managed medically or underwent pars plana vitrectomy (PPV). We excluded eyes with idiopathic MH and those secondary to causes other than uveitis.
RESULTS
Of 27 articles, we identified 86 eyes with MH secondary to uveitis that received either conservative medical treatment alone or PPV with adequate follow-up. The mean (± SD) age of patients included in this review was 46.6 (± 16.8) years; 60.5% were males. The most common etiology of uveitis was Behçet's disease (34.6%) and toxoplasmosis (19.7%). The most common anatomical location of uveitis was posterior (59.3%) followed by panuveitis (35.2%). The mean (± SD) baseline LogMAR vision was 1.1 (± 0.5). Conservative medical treatment was employed in 34.9%, while PPV was performed in 65.1% of eyes. Overall, the mean (SD) LogMAR vision improved from 1.1 (± 0.5) at baseline to 0.7 (± 0.5) after treatment. Inflammation-related MHs were closed in 40% of eyes after conservative therapy and in 87.5% of eyes after PPV. Visual improvement occurred in most eyes (83.9%) that had successful closure of their MH.
CONCLUSIONS
Visual improvement occurs in most eyes that had successful closure of their inflammation-related MH. Conservative medical control of uveitis may lead to closure of inflammation-related MHs and is an important step prior to surgery, if required. Surgical intervention for inflammation-related MHs is associated with good functional and anatomical results.
Topics: Adult; Female; Humans; Inflammation; Male; Middle Aged; Retinal Perforations; Retrospective Studies; Treatment Outcome; Uveitis; Visual Acuity; Vitrectomy
PubMed: 35175410
DOI: 10.1007/s00417-022-05590-0 -
Yonsei Medical Journal Feb 2022Intestinal Behcet's disease (BD) is a systemic autoimmune disease for which treatment options are limited. As a prospective therapeutic strategy for intestinal BD,... (Meta-Analysis)
Meta-Analysis
PURPOSE
Intestinal Behcet's disease (BD) is a systemic autoimmune disease for which treatment options are limited. As a prospective therapeutic strategy for intestinal BD, anti-tumor necrosis factor-alpha (anti-TNF-α) agents have received increasing attention. In this study, we conducted a systematic review and meta-analysis to evaluate the efficacy and safety of anti-TNF-α agents for patients with intestinal BD.
MATERIALS AND METHODS
We searched PubMed, Embase, and Cochrane Library databases up to July 1, 2021 and articles that met the eligibility criteria were further assessed. Pooled rates were synthesized by a randomized effects model using Stata software.
RESULTS
Eleven clinical trials covering 671 patients with intestinal BD were included. According to compositive data, the pooled rate for remission was 39% [95% confidence interval (CI) 26-52] in patients receiving anti-TNF-α agents. Intestinal symptoms were cured in 70% (95% CI 53-84) of the patients, and the rate for endoscopic healing was 65% (95% CI 52-78). Corticosteroid discontinuation was achieved in 43% (95% CI 28-58) of the patients, and the dose reduction of corticosteroid was 20.43 mg (95% CI 13.4-27.46). There were 239 adverse events and 80 serious adverse events during follow-up.
CONCLUSION
Our study indicated that anti-TNF-α agents may serve as an effective treatment with acceptable safety for patients with intestinal BD. However, more robust evidence from randomized controlled trials is urgently needed to assess the long-term efficacy and safety of anti-TNF-α agents for those patients.
Topics: Behcet Syndrome; Humans; Intestines; Treatment Outcome; Tumor Necrosis Factor Inhibitors
PubMed: 35083900
DOI: 10.3349/ymj.2022.63.2.148 -
Scientific Reports Jan 2022This meta-analysis aimed to analyze retinal microvasculature features in eyes with Behçet's disease (BD) using optical coherence tomography angiography (OCTA).... (Meta-Analysis)
Meta-Analysis
This meta-analysis aimed to analyze retinal microvasculature features in eyes with Behçet's disease (BD) using optical coherence tomography angiography (OCTA). Electronic databases, including PubMed, Web of Science, Embase, and Cochrane Library, were comprehensively searched for published studies comparing retinal microvasculature characteristics between eyes with BD and controls. Continuous variables were calculated using the mean difference (MD) with 95% confidence interval (CI). Review Manager software (version 5.30) was used to conduct statistical analysis. A total of 13 eligible studies involving 599 eyes with BD and 622 control eyes were included in the meta-analysis. The pooled results showed that the macular whole enface superficial and deep vessel density (VD) values measured by OCTA were significantly lower in eyes with BD than in control eyes (superficial VD: MD = - 3.05, P < 0.00001; deep VD: MD = - 4.05, P = 0.0004). The foveal superficial and deep VD values were also significantly lower in the BD group than in the control group (superficial VD: MD = - 1.50, P = 0.009; deep VD: MD = - 4.25, - = 0.03). Similarly, the analysis revealed a significant reduction in the parafoveal superficial and deep VD in eyes with BD than in control eyes (superficial VD: MD = - 3.68, P < 0.00001; deep VD: MD = - 4.95, P = 0.0007). In addition, the superficial and deep foveal avascular zones (FAZs) were significantly larger in patients with BD than in controls (superficial FAZ: MD = 0.06, P = 0.02; deep FAZ: MD = 0.12, P = 0.03). The present meta-analysis found that macular whole enface VD, foveal VD, and parafoveal VD were lower in eyes with BD, and the FAZ was larger in patients with BD. The findings suggest that OCTA can assist clinicians in diagnosing and monitoring the status of patients with BD.
Topics: Adult; Behcet Syndrome; Female; Fovea Centralis; Humans; Macula Lutea; Macular Edema; Male; Microvessels; Middle Aged; Retinal Vasculitis; Retinal Vessels; Tomography, Optical Coherence
PubMed: 35031636
DOI: 10.1038/s41598-021-04730-6 -
Thrombosis and Haemostasis Jul 2022To evaluate the relevance of plasma homocysteine (HC) in Behcet's disease (BD) and its clinical manifestations. (Meta-Analysis)
Meta-Analysis
AIM
To evaluate the relevance of plasma homocysteine (HC) in Behcet's disease (BD) and its clinical manifestations.
METHODS
Systematic review of EMBASE and PubMed databases according to PRISMA guidelines from inception to July 2021; random-effects meta-analyses for continuous outcomes.
RESULTS
The search strategy retrieved 48 case-control (2,669 BD and 2,245 control participants) and 5 cohort studies (708 BD participants). Plasma HC was higher in BD than in controls ( < 0.0001) with wide heterogeneity ( = 89.7%) that remained unchanged after sensitivity analysis according to year of article publication, age of BD participants, study size, study quality, method of HC determination, and male/female ratio >1.5; some pooled ethnicities explained a small part of the heterogeneity ( = 16.3%). Active BD participants had higher HC than inactive ones ( < 0.0001), with moderate heterogeneity = 49.2%) that disappeared after removal of an outlier study with very high disease activity. BD participants with any vascular involvement had higher HC than those without ( < 0.0001) with wide heterogeneity ( = 89.7%); subgroup analysis on venous thrombosis only changed neither effect size ( < 0.0001) nor heterogeneity ( = 72.7%). BD participants with ocular involvement had higher HC than those without ( < 0.0001) with moderate heterogeneity ( = 40.3%).
CONCLUSION
Although causality cannot be inferred, the consistency of the elevation of plasma HC in BD, particularly in patients with active disease, with vascular and ocular involvement suggests an intrinsic involvement of HC in these clinical manifestations.
Topics: Behcet Syndrome; Case-Control Studies; Female; Homocysteine; Humans; Male; Venous Thrombosis
PubMed: 34996122
DOI: 10.1055/s-0041-1740637 -
Seminars in Arthritis and Rheumatism Feb 2022Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is characterized by overlapping features of relapsing polychondritis (RP) and Behcet's disease (BD). To...
OBJECTIVE
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is characterized by overlapping features of relapsing polychondritis (RP) and Behcet's disease (BD). To date, no studies have defined the clinical spectrum of disease in a cohort of patients with MAGIC syndrome.
METHODS
Adult patients within an ongoing prospective, observational cohort study in RP were clinically assessed for MAGIC syndrome. A systematic review was conducted to identify additional cases of MAGIC syndrome by searching four databases: PubMed (US National Library of Medicine), Embase (Elsevier), Scopus (Elsevier) and Web of Science: Core Collection (Clarivate Analytics). The inclusion criteria used were: [1] patients of any age or gender who were diagnosed with MAGIC syndrome, or both RP and BD; [2] case report or case series study; [3] published from 1985 - July 2020; and [4] in English language. Risk of bias was assessed using a checklist developed by the authors and based on the Consensus-based Clinical Case Reporting (CARE) Guidelines. Search results screening, article inclusion, data extraction and risk of bais assessment was performed independently by two investigators. Clinical characteristics, particularly BD-related features, were compared between patients with MAGIC syndrome and cases of non-MAGIC RP. The performance characteristics of different criteria to classify MAGIC syndrome were also evaluated.
RESULTS
Out of 96 patients with RP, 13 (14%) patients were diagnosed with MAGIC syndrome. For the systematic review, 380 articles were retrieved of which 90 were screened at title and abstract levels. Of these screened, 60 were excluded and 30 proceeded to full text review where an additional 8 were excluded. Twenty-two articles were included in our review and from which 27 additional cases of MAGIC syndrome were identified. Pooling all 40 cases together and comparing them with non-MAGIC RP, there was a significantly higher prevalence of ocular involvement (28% vs 4%, p<0.01), cutaneous involvement (35% vs 1%, p<0.01), GI involvement (23% vs 4%, p<0.01), and CNS involvement (8% vs 0, p = 0.04) in MAGIC syndrome. A higher prevalence of aortitis (23% vs 1%, p<0.01), Raynaud's phenomenon (54% vs 11%, p<0.01), and elevated anti-collagen II antibodies (50% vs 9%, p = 0.04) were observed in MAGIC syndrome. Fulfillment of either McAdam's or Damiani's Criteria for RP plus the International Criteria for Behçet's Disease had excellent sensitivity (98%) to classify cases of MAGIC syndrome.
CONCLUSION
A substantial proportion of patients with RP can be clinically diagnosed with MAGIC syndrome. These patients have features of RP, BD, and other unique features including aortitis, Raynaud's phenomenon and elevated anti-collagen II antibodies.
Topics: Adult; Behcet Syndrome; Cartilage; Genitalia; Humans; Mouth; Observational Studies as Topic; Prospective Studies; Ulcer; United States
PubMed: 34972595
DOI: 10.1016/j.semarthrit.2021.10.007