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Frontiers in Immunology 2019Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and... (Review)
Review
Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.
Topics: Behcet Syndrome; Blood Vessels; Cartilage, Articular; Combined Modality Therapy; Disease Management; Humans; Mucous Membrane; Phenotype
PubMed: 31921115
DOI: 10.3389/fimmu.2019.02830 -
Revista Espanola de Enfermedades... Jul 2023A 36-year-old man was admitted to the emergency department due to a 30-day history of abdominal distention and epigastralgia. He had described a non-intentional 10kg...
A 36-year-old man was admitted to the emergency department due to a 30-day history of abdominal distention and epigastralgia. He had described a non-intentional 10kg weight loss, dry cough, and fever 6 months before his admission. He had a history of tobacco and cocaine abuse and reported recurrent oral and genital ulcers. Physical examination showed an extensive area of venous collateral circulation on the abdominal wall, hepatomegaly, signs of a moderate ascites, and lower limb edema. Liver and renal function tests were normal. The ascitic fluid analysis did not show an inflammatory or infectious pattern. Upper flexible endoscopy revealed esophageal fine-caliber varices and colonoscopy showed an isolated terminal ileal ulcer. Abdominal imaging revealed hepatomegaly, voluminous ascites, and thrombosis of hepatic veins, inferior and superior vena cava (Figure 1). Infections and coagulation or lymphoproliferative disorders were excluded. Thereafter, the diagnosis of Budd-Chiari Syndrome in Behçet disease was established and immunosuppression treatment was started with good initial clinical evolution.
Topics: Male; Humans; Adult; Budd-Chiari Syndrome; Behcet Syndrome; Ascites; Hepatomegaly; Vena Cava, Superior; Vena Cava, Inferior
PubMed: 36263811
DOI: 10.17235/reed.2022.9267/2022 -
Orphanet Journal of Rare Diseases Feb 2021Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal... (Review)
Review
Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with remissions and exacerbations. It is a multifactorial disease, and several triggering factors including oral cavity infections and viruses may induce inflammatory attacks in genetically susceptible individuals. BD vasculitis involves different vessel types and sizes of the vascular tree with mixed-cellular perivascular infiltrates and is often complicated by recurrent thrombosis, particularly in the venous compartment. Several new therapeutic modalities with different mechanisms of action have been studied in patients with BD. A substantial amount of new data have been published on the management of BD, especially with biologics, over the last years. These important therapeutic advances in BD have led us to propose French recommendations for the management of Behçet's disease [Protocole National de Diagnostic et de Soins de la maladie de Behçet (PNDS)]. These recommendations are divided into two parts: (1) the diagnostic process and initial assessment; (2) the therapeutic management. Thirty key points summarize the essence of the recommendations. We highlighted the main differential diagnosis of BD according to the type of clinical involvement; the role of genetics is also discussed, and we indicate the clinical presentations that must lead to the search for a genetic cause.
Topics: Behcet Syndrome; Diagnosis, Differential; Genetic Predisposition to Disease; Humans; Vasculitis
PubMed: 33622338
DOI: 10.1186/s13023-020-01620-4 -
Behçet's Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review.Medicina (Kaunas, Lithuania) Mar 2024Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital... (Review)
Review
Behçet's disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital ulcers, skin lesions, and uveitis, though it can affect multiple organ systems. Diagnosis is primarily clinical due to the lack of a definitive diagnostic test, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies involve corticosteroids, immunosuppressive agents, and, increasingly, biological therapies. Behçet's disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress in understanding its clinical characteristics and treatment approaches, gaps remain in our understanding of its pathogenesis. Future research is needed to elucidate the disease's pathophysiology and optimize treatment strategies.
Topics: Humans; Adrenal Cortex Hormones; Behcet Syndrome; Immunosuppressive Agents
PubMed: 38674208
DOI: 10.3390/medicina60040562 -
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 -
Ophthalmology. Retina Feb 2023
Topics: Humans; COVID-19; Retinal Diseases; Scotoma; White Dot Syndromes
PubMed: 36216223
DOI: 10.1016/j.oret.2022.09.005 -
Indian Journal of Ophthalmology Dec 2022To evaluate efficacy of topical interferon alfa-2b (IFN) in the treatment of uveitic macular edema (UME).
PURPOSE
To evaluate efficacy of topical interferon alfa-2b (IFN) in the treatment of uveitic macular edema (UME).
METHODS
This is a prospective, interventional case study of patients with UME. Injection IFN was reconstituted into eye drops and a four times/day (QID) application was prescribed. Central macular thickness (CMT) on optical coherence tomography (OCT) scan was evaluated. Improvement in CMT by ≥50 μm from the baseline was studied in eyes with presenting CMT ≥400 μm.
RESULTS
Twenty eyes of 20 patients with UME were studied: anterior uveitis (n = 3), anterior + intermediate uveitis (n = 5), posterior uveitis (n = 3), retinal vasculitis (n = 3), and panuveitis (n = 6). Mean CMT at the presentation was 423.3 μm (range: 270-604 μm), which improved at 1 month (n = 16), 2 months (n = 10), and ≥3 months (n = 11) follow-up, to 415.3 μm (range: 247-579 μm) (P = 0.411), 364.4 μm (range: 258-566 μm) (P = 0.099), 344 μm (range: 258-484 μm) (P = 0.001), respectively. Twelve eyes of 12 patients had presenting CMT ≥400 μm. In these cases, decrease in CMT by ≥50 μm was seen in 4/10, 4/5, and 5/6 eyes at 1 and 2 months and ≥3 months follow-up. Mean follow-up was 4 months (range: 1-17 months). Complete resolution of UME was seen only in three eyes. No ocular or systemic side effects were observed.
CONCLUSION
Topical IFN therapy in QID doses is safe but may have limited role in UME. Long-term therapy may improve its efficacy. Larger studies with dose modification, combination with other drugs, and with homogeneous uveitis population are recommended.
Topics: Humans; Macular Edema; Prospective Studies; Uveitis; Eye; Uveitis, Anterior
PubMed: 36453344
DOI: 10.4103/ijo.IJO_1450_22 -
Ophthalmology Jun 2021To evaluate long-term efficacy and safety of extended treatment with adalimumab in patients with noninfectious intermediate, posterior, or panuveitis. (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
To evaluate long-term efficacy and safety of extended treatment with adalimumab in patients with noninfectious intermediate, posterior, or panuveitis.
DESIGN
Open-label, multicenter, phase 3 extension study (VISUAL III).
PARTICIPANTS
Adults who had completed a randomized, placebo-controlled phase 3 parent trial (VISUAL I or II) without treatment failure (inactive uveitis) or who discontinued the study after meeting treatment failure criteria (active uveitis).
METHODS
Patients received subcutaneous adalimumab 40 mg every other week. Data were collected for ≤ 362 weeks. Adverse events (AEs) were recorded until 70 days after the last dose.
MAIN OUTCOME MEASURES
Long-term safety and quiescence; other efficacy variables included inflammatory lesions, anterior chamber cell and vitreous haze grade, macular edema, visual acuity, and dose of uveitis-related systemic corticosteroids.
RESULTS
At study entry, 67% of patients (283/424) showed active uveitis and 33% (141/424) showed inactive uveitis; 60 patients subsequently met exclusion criteria, and 364 were included in the intention-to-treat analysis. Efficacy variables were analyzed through week 150, when approximately 50% of patients (214/424) remained in the study. Patients showing quiescence increased from 34% (122/364) at week 0 to 85% (153/180) at week 150. Corticosteroid-free quiescence was achieved by 54% (66/123) and 89% (51/57) of patients with active or inactive uveitis at study entry. Mean daily dose of systemic corticosteroids was reduced from 9.4 ± 17.1 mg/day at week 0 (n = 359) to 1.5 ± 3.9 mg/day at week 150 (n = 181). The percentage of patients who achieved other efficacy variables increased over time for those with active uveitis at study entry and was maintained for those with inactive uveitis. The most frequently reported treatment-emergent AEs of special interest were infections (n = 275; 79 events/100 patient-years [PY]); AEs and serious AEs occurred at a rate of 396 events/100 PY and 15 events/100 PY, respectively.
CONCLUSIONS
Long-term treatment with adalimumab led to quiescence and reduced corticosteroid use for patients who entered VISUAL III with active uveitis and led to maintenance of quiescence for those with inactive uveitis. AEs were comparable with those reported in the parent trials and consistent with the known safety profile of adalimumab.
Topics: Adalimumab; Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Injections, Subcutaneous; Male; Middle Aged; Panuveitis; Retrospective Studies; Time Factors; Treatment Outcome; Uveitis, Intermediate; Uveitis, Posterior; Visual Acuity; Young Adult
PubMed: 33157077
DOI: 10.1016/j.ophtha.2020.10.036 -
The Israel Medical Association Journal... Nov 2022Behçet's disease (BD) is a chronic vasculitic multi-systemic disease of unknown etiology. BD is characterized by recurrent attacks of oral aphthae, genital ulcers, and...
BACKGROUND
Behçet's disease (BD) is a chronic vasculitic multi-systemic disease of unknown etiology. BD is characterized by recurrent attacks of oral aphthae, genital ulcers, and uveitis. BD is a multisystemic disorder and as such it may provoke various psychiatric manifestations, including depression.
OBJECTIVES
To evaluate the association between BD and depression, adjusting for established risk factors for depression.
METHODS
We executed a cross-sectional study based on the Clalit Health Services database, the largest healthcare organization in Israel, serving over 4.4 million members. For this study 873 BD patients were detected and matched with 4369 controls by age and sex.
RESULTS
The rate of depression was higher among the BD patients compared with the control group (9.39% vs 5.49%, respectively, odds ratio [OR] 1.79, 95% confidence interval [95%CI] 1.37-2.31, P < 0.001). An association between BD and depression was also observed on multivariable analysis (OR 1.83, 95%CI 1.39-2.39, P < 0.001). When stratifying the data, according to established risk factors, the association between BD and depression was prominent in the youngest age group (18-39 years of age), low and high socioeconomical status, and non-smokers.
CONCLUSIONS
Establishing the association between BD and depression should influence the attitude and the treatment of BD patients, as this relationship requires a more holistic approach and a multidisciplinary treatment regimen for all patient needs.
Topics: Humans; Adolescent; Young Adult; Adult; Behcet Syndrome; Cross-Sectional Studies; Depression; Stomatitis, Aphthous; Uveitis
PubMed: 36436039
DOI: No ID Found -
Clinical and Experimental Rheumatology 2020Ocular involvement in Behçet's syndrome still represents a challenge for both rheumatologists and ophthalmologists; over the past 20 years the availability of new... (Review)
Review
Ocular involvement in Behçet's syndrome still represents a challenge for both rheumatologists and ophthalmologists; over the past 20 years the availability of new diagnostic tools and the concomitant introduction of biologic drugs led to a significant improvement in the management of these patients. The lack of uniform definitions and the diversity of the outcome measures still represent an obstacle for the prompt and correct management of ocular manifestations. The aim of the present review is to summarise the current evidences related to correct diagnosis and proper management of patients with Behçet's syndrome and ocular involvement.
Topics: Behcet Syndrome; Humans
PubMed: 33253088
DOI: No ID Found