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Clinical and Experimental Rheumatology 2019Several epidemiologic studies report on the prevalence of Behçet's syndrome (BS) and demographic and clinical findings in patients from different countries and... (Review)
Review
Several epidemiologic studies report on the prevalence of Behçet's syndrome (BS) and demographic and clinical findings in patients from different countries and ethnicities. Although these studies point out geographic differences in disease course, methodologic differences make it difficult to compare the results of these studies. Recent data suggest that neutrophil extracellular trap levels are elevated in patients with BS, and that it may be a potential therapeutic target for the reduction or prevention of BS-associated thrombotic risk. Details on the mode of functioning of ERAP have been delineated and further epigenetic data reported. Wall thickness of lower extremity veins is increased among BS patients without any apparent clinical involvement. Magnetic resonance (MR) venography and Doppler ultrasonography (USG) were comparable in the diagnosis of chronic deep vein thrombosis, while MR venography is more effective in detecting collateral formations. Results were also collected on some dietary and non-dietary factors in triggering oral ulcers, while smoking seems to have a protective role. With regards to the therapy, it has been demonstrated that endovascular interventions carry the risk of inducing pathergy phenomenon. Apremilast has been convincingly shown to be useful for oral ulcers of BS and classical immunosuppressives are effective as first line therapy in more than half of patients with uveitis. While infliximab and adalimumab seem to be equally effective in the treatment of refractory uveitis of BS, the combination of adalimumab and immunosuppressives appears to be superior to immunosuppressives alone for venous thrombosis of the extremities. In addition, tocilizumab might be an alternative to anti-TNF agents for patients with arterial involvement refractory to immunosuppressives. On the other hand, the place of IL-17 inhibition in the treatment of BS still remains questionable.
Topics: Adalimumab; Behcet Syndrome; Genetic Predisposition to Disease; Humans; Immunosuppressive Agents; Oral Ulcer; Prevalence; Tumor Necrosis Factor-alpha; Uveitis; Venous Thrombosis
PubMed: 31856939
DOI: No ID Found -
Journal of B.U.ON. : Official Journal... 2020
Topics: Behcet Syndrome; Breast Neoplasms; Female; Humans
PubMed: 33099967
DOI: No ID Found -
International Journal of Dermatology 1984Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, intestinal, articular, vascular, urogenital, and neurologic involvement. While classically... (Review)
Review
Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, intestinal, articular, vascular, urogenital, and neurologic involvement. While classically intermittent in its manifestations, the disease can stabilize and become chronic in a given organ system. The diagnosis of Behçet's disease is based on clinical criteria. Recurrent aphthous ulcerations in the mouth, skin lesions, eye lesions, and genital ulcerations must be present during the course of the disease for a diagnosis of Behçet's disease to be made unequivocally. A nonspecific skin hyperreactivity called pathergy is said to be helpful in the diagnosis. There are no pathognomonic laboratory findings, but biopsy usually shows a venulitis. The pathogenesis of the disease is unknown. No virus has been satisfactorily isolated to date. There is evidence of an increased frequency of HLA-B5 and HLA-B12. Humoral and cellular immunity seem to play a major part in the pathogenesis of the various manifestations of the disease. The treatment of Behçet's disease is difficult to evaluate because of the many spontaneous exacerbations and remissions during the clinical course of the disease. Topical corticosteroids for orogenital ulcers and ocular inflammation are helpful. Intralesional injections for affected joints and retrobulbar tissues are useful in some cases. Systemic corticosteroids have appeared to be helpful for all manifestations of the disease. It is customary to use 60 mg of prednisone by mouth daily during acute exacerbations, then to taper as the condition improves. Chlorambucil has been shown to be safe and effective for various manifestations of Behçet's disease; often it is used in combination with corticosteroids.(ABSTRACT TRUNCATED AT 250 WORDS)
Topics: Behcet Syndrome; Eye Diseases; Female; Genital Diseases, Female; Genital Diseases, Male; Humans; Male; Prognosis; Skin Diseases; Stomatitis, Aphthous
PubMed: 6373632
DOI: 10.1111/j.1365-4362.1984.tb05658.x -
Clinical and Experimental Rheumatology 2018New epidemiologic studies from Poland, Jordan, Algeria, Taiwan and Korea highlight the geographic differences in incidence, prevalence and clinical features of Behçet's... (Review)
Review
New epidemiologic studies from Poland, Jordan, Algeria, Taiwan and Korea highlight the geographic differences in incidence, prevalence and clinical features of Behçet's syndrome (BS). A study from Austria comparing clinical manifestations of their BS patients with different countries of origin suggest that environmental factors may be important in the disease phenotype of BS. New genetic association studies dealing with the innate and acquired aspects of BS prevailed during 2017 and novel susceptibility and regulatory factors were described. Common denominators among various disease processes were again highlighted and epigenetic factors were emphasised. "Bagel sign" pattern, a central lesion with hypo-intense core and hyper-intense rim was defined in the spinal MRIs of the patients with neuro-BS especially during the acute attacks of myelopathy. This distinctive pattern suggests venous thrombosis and surrounding oedema in the spinal cord. Pseudotumour cerebri may present with similar clinical presentation to that observed in cerebral venous sinus thrombosis, responds well to immunosuppressive treatment, and could be associated with venous thrombotic relapses. Menstruation and certain food appear to exacerbate skin and mucosa lesions in BS. The EULAR recommendations for the treatment of BS have been updated with 5 new overarching principles and one additional recommendation for surgical management of vascular complications. Infliximab initiated earlier in the course of uveitis yields a better visual outcome. Tapering or stopping of anti-TNF agents seem to be possible when remission has been achieved. Adalimumab appears to be more effective for venous thrombosis than classical immunosuppressives. Oral anticoagulants might not be crucial for cerebral or peripheral venous thrombosis. Transcatheter embolisation of pulmonary aneurysms may be life-saving by providing immediate control of haemoptysis. The results of surgery for pulmonary artery involvement appear to be satisfactory.
Topics: Animals; Behcet Syndrome; Disease Progression; Genetic Predisposition to Disease; Humans; Immunosuppressive Agents; Phenotype; Risk Factors; Treatment Outcome
PubMed: 30582516
DOI: No ID Found -
Clinical and Experimental Rheumatology Sep 2022Behçet's syndrome (BS) has been reported with cardiovascular involvement. It's still unclear that BS is associated with the increased risk of ischaemic heart disease... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Behçet's syndrome (BS) has been reported with cardiovascular involvement. It's still unclear that BS is associated with the increased risk of ischaemic heart disease (IHD). We aimed to conduct a meta-analysis concerning the incidence of IHD in BS and identify the relationship between IHD and BS.
METHODS
We performed a comprehensive literature search based on PubMed and Embase databases up to 7 July, 2021. Incidence of IHD was calculated by metaproportion. Pooled risk ratio and 95% confidence interval (CI) were calculated using a random-effect, generic inverse variance method of DerSimonian and Laird.
RESULTS
Four studies with 9237 patients with IHD in BS and 40353 controls were identified and included in our meta-analysis. The pooled risk ratio of IHD in patients with BS was 1.30 and achieved statistical significance (95% CI 1.04-1.64). The statistical heterogeneity was low with an I2 of 39% (p=0.18).
CONCLUSIONS
In this meta-analysis the presence of BS was associated with an increased risk of IHD. Prospective researches should be done to determine the pathophysiological and prognostic implications of increased IHD in BS.
Topics: Behcet Syndrome; Coronary Artery Disease; Humans; Incidence; Myocardial Ischemia; Prospective Studies; Risk Factors
PubMed: 35699061
DOI: 10.55563/clinexprheumatol/1shqmz -
Frontiers in Immunology 2023Behcet's disease (BD) is a chronic multi-systemic disease characterized by relapsing-remitting oral ulcers, genital ulcers, ocular inflammatory involvements, and... (Review)
Review
Behcet's disease (BD) is a chronic multi-systemic disease characterized by relapsing-remitting oral ulcers, genital ulcers, ocular inflammatory involvements, and numerous other systemic features. Ocular involvements are quite common in BD and may cause severe tissue damage and potentially blindness. Even though the pathogenesis of BD remains ambiguous, growing evidences have shown that genetic factors, environmental triggers and immunological abnormalities play significant roles in its development and progression. Novel biotherapies targeting IFN-γ, TNF-α and interleukins have been used in recent years. In this review, we mainly pay attention to the ocular involvement of BD, and discuss the current understanding of mechanisms and advances in therapeutic approaches, especially novel biologics. Finally, we discuss the management in patients with pregnancy.
Topics: Humans; Behcet Syndrome; Eye; Inflammation; Biological Factors; Interleukins
PubMed: 37841268
DOI: 10.3389/fimmu.2023.1206959 -
Internal and Emergency Medicine Apr 2023This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet's syndrome (BS), their association with other disease manifestations, response... (Clinical Trial)
Clinical Trial
This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet's syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet's Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0-4). Colchicine was inefficacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p = 0.46) or the concomitant therapy (p = 0.30 for cDMARDs, p = 1.00 for glucocorticoids); cDMARDs and bDMARDs were inefficacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefficacy (p = 0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively affects response to biologic therapies. ClinicalTrials.gov Identifier: NCT05200715 (registered on December 18, 2021).
Topics: Child; Humans; Arthritis; Behcet Syndrome; Myalgia; Registries; Ulcer
PubMed: 36881285
DOI: 10.1007/s11739-023-03215-w -
Pediatric Rheumatology Online Journal Apr 2017Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated, inflammatory disorders affecting many different systems (joints, skin, eyes,... (Review)
Review
Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated, inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal and biliary tracts). Both disorders have fluctuating courses and when gastrointestinal symptoms are prevalent, differential diagnosis can be difficult. BD involves the gastrointestinal tract in 10-15% of cases with localized lesions in the ileocecal region. The clinical picture is heterogeneous with various clusters of disease expression. CD is a chronic inflammatory disorder, which can affect any part of the intestinal tract, as well as extra-intestinal tissue. Factors that contribute towards the pathogenesis of both disease include the host's genetic profile, and immune system, and environmental factors such as the gut microbiota. The aim of this manuscript is to provide a narrative review of clinical features of BD and CD, highlighting the importance of differential diagnosis and therapeutic approach, especially in the presence of gastrointestinal involvement. A comprehensive search of published literature using the Pubmed ( http://www.ncbi.nlm.nih.gov/pubmed/ ) database was carried out to identify all articles published in English from 1999 to October 2016, using 4 key terms: "Behçet Disease", "Intestinal Behçet's Disease", "Crohn's Disease" and" Inflammatory Bowel Disease".
Topics: Antirheumatic Agents; Behcet Syndrome; Crohn Disease; Diagnosis, Differential; Digestive System Surgical Procedures; Endoscopy, Gastrointestinal; Gastrointestinal Diseases; Gout Suppressants; Humans; Immunosuppressive Agents
PubMed: 28427473
DOI: 10.1186/s12969-017-0162-4 -
Clinical and Experimental Rheumatology 2017A meta-analysis showed that methodological differences in prevalence studies such as a sample survey design or census design may be responsible for some of the variance... (Review)
Review
A meta-analysis showed that methodological differences in prevalence studies such as a sample survey design or census design may be responsible for some of the variance in BS prevalence reported across countries, in addition to a true geographic variation. Efforts towards developing a data driven core set of outcome measures for clinical trials is continuing. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualising diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in Behçet's uveitis. Haemoptysis could also be due to bronchial artery enlargement in BS patients with pulmonary artery involvement and can be effectively treated with embolisation. Recent studies shed light on the link between immune system and thrombosis: fibrin clots seemed to be structurally different and plasmin resistant in BS. Newer genetic associations using immunochip were determined, but HLA-B51 is still the principal genetic link. Various studies on micro-RNA's, important molecules of immune regulation were published and discussed. Anti-TNF agents are still the key biologics for the treatment of various manifestations of BS. Two Phase III trials enrolling a small number of BS patients have shown the efficacy of adalimumab in the treatment of non-infectious, non-anterior uveitis. Interferon-alpha was found to induce long-lasting drug free remissions in a retrospective study. Small observational studies with non-TNF biologics such as ustekinumab, anakinra and canakinumab report beneficial results which await confirmation with further studies.
Topics: Behcet Syndrome; Biological Products; Disease Progression; Genetic Predisposition to Disease; Humans; Immunosuppressive Agents; Multimodal Imaging; Predictive Value of Tests; Prevalence; Risk Factors; Treatment Outcome; Tumor Necrosis Factor-alpha
PubMed: 28980900
DOI: No ID Found -
La Tunisie MedicaleBehçet's disease is a chronic vasulitis. The aphtous stomatitis is present in nearly the totality of patients having this disease.
INTRODUCTION
Behçet's disease is a chronic vasulitis. The aphtous stomatitis is present in nearly the totality of patients having this disease.
AIM
The purpose of this study was to evaluate the oral health of Behcet's disease (BD) patients and then compare it with healthy controls.
METHODS
Twenty patients with BD and 20 healthy subjects were included in this cross-sectional study conducted at the military hospital of Bizerta from 01/10/2017 to 31/05/2019. The level of plaque were recorded using the plaque index (PI). The gingival index (GI) was used to evaluate the gingival inflammation. The depths of the sulci/pockets were probed with a periodontal probe. The clinical attachment loss (CAL) was measured from the cementoenamel junction to the sulcus base. The caries status was scored by using DMFT introduced by Klein and Palmer.The diagnosis of oral lesions was performed using the visual tactile examination.
RESULTS
The two groups were age, sexe, and socioeconomic level matched. The percentage of oral ulcers was observed to be higher in BD patients compared with healthy controls (40% Vs 5%, p0.01). Statistical significant differences in the two groups' PI (1,19±0,38 Vs 0,86±0,37 ; p=0,011) and GI (1,10±0,30 Vs 0,75±0,36 ; p=0,03) were observed. The probing depth, the CAL and the DMF/T showed no associations with the BD.
CONCLUSION
Our results showed that gingival health is worse in BD patients. Therfore, the dentist must play an important part in the management of BD and the motivation for the oral hygiene.
Topics: Behcet Syndrome; Cross-Sectional Studies; Humans; Oral Health; Periodontal Index
PubMed: 36155901
DOI: No ID Found