-
Acta Medica Portuguesa Sep 2023Behçet's disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and... (Review)
Review
Behçet's disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and gastrointestinal and neurological involvement. Neuro-Behçet corresponds to nervous system involvement and is one of the most severe complications of Behçet disease. It occurs in 3% to 30% of cases and is categorized into parenchymal (most common) or non-parenchymal disease. The most common manifestation of parenchymal neuro-Behçet is meningoencephalitis with involvement of the brainstem, where patients present with cranial neuropathies, encephalopathy, sensory-motor syndromes, epilepsy, or myelitis. The main non-parenchymal manifestation is cerebral venous thrombosis. Neuro-Behçet has a predominantly subacute course, with remission within weeks, or clinical progression in one third of the cases. The diagnosis is essentially clinical and diagnostic tests help to corroborate the suspicion, distinguish from differential diagnoses, and exclude complications. Brain magnetic resonance imaging allows the identification of acute lesions (hypointense or isointense on T2-weighted and hypointense on T1-weighted sequences) contrast-enhanced, and chronic lesions characterized by non-contrast enhanced small lesions and brainstem atrophy. If non-parenchymal involvement is suspected, cerebral veno-magnetic resonance imaging /computed tomography should be performed. Cerebrospinal fluid shows elevated proteinorachia and pleocytosis in parenchymal and no changes in non-parenchymal neuro-Behçet (except increased opening pressure). Outbursts of parenchymal disease should be treated with high dose intravenous corticosteroid therapy, with subsequent switch to oral corticoids, followed by biologic therapy, usually an anti-TNF. The treatment of cerebral venous thrombosis is controversial and may consist of a combination of corticosteroids and anticoagulation.
Topics: Humans; Behcet Syndrome; Tumor Necrosis Factor Inhibitors; Brain; Magnetic Resonance Imaging; Diagnosis, Differential; Adrenal Cortex Hormones; Venous Thrombosis
PubMed: 37345389
DOI: 10.20344/amp.19734 -
The Korean Journal of Internal Medicine Jan 2016We prepared a cross-cultural adaptation of the Behcet's Syndrome Activity Scale (BSAS) and evaluated its reliability and validity in Korea.
BACKGROUND/AIMS
We prepared a cross-cultural adaptation of the Behcet's Syndrome Activity Scale (BSAS) and evaluated its reliability and validity in Korea.
METHODS
Fifty patients with Behcet's disease (BD) who attended the Rheumatology Clinic of Gachon University Gil Medical Center were included in this study. The first BSAS questionnaire was administered at each clinic visit, and the second questionnaire was completed at home within 24 hours of the visit. A Behcet's Disease Current Activity Form (BDCAF) and a Behcet's Disease Quality of Life (BDQOL) form were also given to patients. The test-retest reliability was analyzed by intraclass correlation coefficients (ICC). To assess the validity, the total BSAS score was compared with the BDCAF score, the patient/physician global assessment, and the BDQOL by Spearman rank correlation.
RESULTS
Twelve males and 38 females were enrolled. The mean age was 48.5 years and the mean disease duration was 6.7 years. Thirty-eight patients (76.0%) returned the questionnaire by mail. For the test-retest reliability, the two assessments were significantly correlated on all 10 items of the BSAS questionnaire (p < 0.05) and the total BSAS score (ICC, 0.925; p < 0.001). The total BSAS score was statistically correlated with the BDQOL, BDCAF, and patient/physician global assessment (p < 0.01).
CONCLUSIONS
The Korean version of BSAS is a reliable and valid instrument to measure BD activity.
Topics: Academic Medical Centers; Adult; Behcet Syndrome; Cost of Illness; Cultural Characteristics; Female; Humans; Male; Middle Aged; Patient Reported Outcome Measures; Predictive Value of Tests; Quality of Life; Reproducibility of Results; Republic of Korea; Severity of Illness Index
PubMed: 26767871
DOI: 10.3904/kjim.2016.31.1.170 -
Arthritis Care & Research Apr 2022An unmet need exists for reliable, validated, and widely-accepted outcome measures for randomized clinical trials in Behçet's syndrome. The Outcome Measures in...
OBJECTIVE
An unmet need exists for reliable, validated, and widely-accepted outcome measures for randomized clinical trials in Behçet's syndrome. The Outcome Measures in Rheumatology (OMERACT) Behçet's Syndrome Working Group, a large, multidisciplinary group of experts in Behçet's syndrome and patients with Behçet's syndrome, had an objective of developing a core set of data-driven outcome measures for use in all clinical trials of Behçet's syndrome.
METHODS
The core domain set was developed through a comprehensive, iterative, multistage project that included a systematic review, a focus group meeting and qualitative patient interviews, a survey among experts in Behçet's syndrome, a Delphi exercise involving both patients and physician experts in Behçet's syndrome, and use of the data, insight, and feedback generated by these processes to develop a final core domain set.
RESULTS
All steps were completed and domains were delineated across the organ systems involved in this disease. Since trials in Behçet's syndrome often focus on specific manifestations and not on the disease in its entirety, the final proposed core set includes 5 domains mandatory for study in all trials in Behçet's syndrome (disease activity, new organ involvement, quality of life, adverse events, and death) with additional subdomains mandatory for study of specific organ-systems. The final core set was endorsed at the 2018 OMERACT meeting.
CONCLUSION
The core set of domains in Behçet's syndrome provides the foundation through which the international research community, including clinical investigators, patients, the biopharmaceutical industry, and government regulatory bodies can harmonize the study of this complex disease, compare findings across studies, and advance development of effective therapies.
Topics: Behcet Syndrome; Focus Groups; Humans; Outcome Assessment, Health Care; Quality of Life; Rheumatology
PubMed: 33202101
DOI: 10.1002/acr.24511 -
Pediatric Rheumatology Online Journal Feb 2016BD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown... (Review)
Review
BD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early. An international expert consensus has recently proposed new classification criteria for children with BD. The clinical spectrum of BD is heterogeneous and influenced by gender, ethnicity and country of residence. Young males have the worst prognosis with significantly more frequent neurological, ocular and vascular involvement. BD treatment is aimed at alleviating inflammation. Among all drugs, TNFα inhibitors have become a standard to control severe ocular, neurological and digestive system involvement.
Topics: Behcet Syndrome; Child; Disease Management; Global Health; Humans; Morbidity
PubMed: 26887984
DOI: 10.1186/s12969-016-0070-z -
Postgraduate Medical Journal Oct 2000Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital... (Review)
Review
Behçet's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.
Topics: Adult; Autoimmune Diseases; Behcet Syndrome; Brain Diseases; Diagnosis, Differential; Female; Genital Diseases, Female; Genital Diseases, Male; Humans; Infant; Infant, Newborn; Male; Oral Ulcer; Pregnancy; Skin Ulcer; Ulcer; Uveitis; Vasculitis; Venules
PubMed: 11009577
DOI: 10.1136/pmj.76.900.629 -
Acta Reumatologica Portuguesa 2006Behçet syndrome (BS) is a multisystem vasculitis characterized by skin and mucosa lesions and musculoskeletal, ocular, gastrointestinal, neurological and major vessel... (Review)
Review
Behçet syndrome (BS) is a multisystem vasculitis characterized by skin and mucosa lesions and musculoskeletal, ocular, gastrointestinal, neurological and major vessel involvement. It is seen mainly in the Mediterranean basin, Middle East and the Far East. The disease runs a more severe course among young males and the severity diminishes with age. This review describes the management of this disease, which should be individualized and varies according to site and gender.
Topics: Behcet Syndrome; Humans
PubMed: 17058358
DOI: No ID Found -
Clinical and Experimental Rheumatology 2021
Topics: Behcet Syndrome; Case-Control Studies; Humans
PubMed: 34596024
DOI: 10.55563/clinexprheumatol/x453f7 -
Clinical and Experimental Rheumatology 2016Several articles highlighting the epidemiology, pathogenesis, clinical features, treatment modalities and disease assessment of Behçet's syndrome (BS) have been... (Review)
Review
Several articles highlighting the epidemiology, pathogenesis, clinical features, treatment modalities and disease assessment of Behçet's syndrome (BS) have been published during the last year. Clinical and radiological features of lower extremity deep vein thrombosis due to BS can be quite different than those found in thrombosis due to other causes; additionally, frequency of post-thrombotic syndrome is significantly increased in BS. Some clinical and colonoscopic features are useful in differentiating BS from Crohn's disease. Barkhof criteria may be helpful in differentiating neurologic involvement due to BS from multiple sclerosis. Anatomical localization of papulopustular lesions but not histology has been found to be helpful in differentiating papulopustular lesions of BS from those found in acne vulgaris. Several studies looked at the ovarian reserve with contradicting results. A population-based cohort study found higher risk of hematological malignancies only among female BS patients living in Taiwan. The role of genetic factors and environment is discussed and both autoimmune and autoinflammatory features are underlined in the pathogenesis of BS. New data on the epistatic interactions between ERAP and HLA B51 is available and information on the microbiome have started to appear. New uncontrolled data suggest beneficial effects of anti-TNFs for refractory extra-ocular complications of BS such as pulmonary artery, gastrointestinal and central nervous system involvement. Uncontrolled studies suggest promising results with interleukin-1 inhibition but gevokizumab, a humanised anti IL-1β antibody, failed to meet the primary endpoint of time to first ocular exacerbation in a phase III trial. The debate on anticoagulation continues with new observational data.
Topics: Animals; Anticoagulants; Behcet Syndrome; Female; Genetic Markers; Genetic Predisposition to Disease; Humans; Immunosuppressive Agents; Inflammation Mediators; Male; Phenotype; Predictive Value of Tests; Risk Factors; Severity of Illness Index; Treatment Outcome
PubMed: 27791958
DOI: No ID Found -
Turkish Journal of Ophthalmology Aug 2021To analyze and compare sociodemographic features between Behçet uveitis and other non-infectious uveitis.
OBJECTIVES
To analyze and compare sociodemographic features between Behçet uveitis and other non-infectious uveitis.
MATERIALS AND METHODS
The data of adults with non-infectious uveitis in the nationwide uveitis database were analyzed and the sociodemographic features of patients with and without Behçet disease were compared.
RESULTS
This study included data of 4,978 eyes of 3,363 patients from 33 centers. The mean age at presentation was 38.7±13.3 (17-87) years. The mean age was 34.3±10.5 years in the Behçet uveitis group and 41.1±14.0 years in the other non-infectious uveitis group (p<0.001). Male predominance was seen in the Behçet uveitis group (67.7% vs. 32.3%) while female patients were more common in the other non-infectious uveitis group (54.4% vs. 45.6%, p<0.001). Regarding education level, the proportion of patients with low education was higher in the Behçet uveitis group than the other non-infectious uveitis group (49.6% vs. 43.4% in males, p=0.004; 61.5% vs. 59.2% in females, p=0.021). Having a low-income job or being currently unemployed, indicators of poor income, were more frequent in the Behçet uveitis group than in the other non-infectious uveitis group (32.0% vs. 22.8%, p<0.001). In the comparison of places of residence, the proportion of patients who lived in cities with low gross national product was 37.0% in the Behçet uveitis group and 31.1% in the other non-infectious uveitis group (p<0.001).
CONCLUSION
Patients with Behçet disease had lower education level and socioeconomic status than patients with other non-infectious uveitis entities.
Topics: Adult; Behcet Syndrome; Educational Status; Female; Humans; Male; Uveitis; Young Adult
PubMed: 34461696
DOI: 10.4274/tjo.galenos.2020.28485 -
Acta Obstetricia Et Gynecologica... Oct 2005Behçet's disease (BD) is a multisystem inflammatory chronic disorder, which is characterized by relapsing oral and genital ulceration and iridocyclitis. While being of... (Review)
Review
BACKGROUND
Behçet's disease (BD) is a multisystem inflammatory chronic disorder, which is characterized by relapsing oral and genital ulceration and iridocyclitis. While being of unknown etiology, vasculitic changes of possible autoimmune origin are common to all involved organs, and thrombotic complications, which may adversely affect gestation, are frequently seen. Very little is known to date about the reciprocal influence of BD and pregnancy. We have undertaken to explore the mutual effect of BD and pregnancy with emphasis on maternal and fetal complications.
METHODS
In this case-control study, we have evaluated pregnancies that occurred in women suffering from BD, who were treated in our institution during the last 25 years. All records were reviewed, and data were confirmed by a telephone interview and compared with a matched control group. To review the current knowledge, a Medline search together with a manual search of selected articles was performed.
RESULTS
Thirty-one Behçet's patients who had 135 pregnancies were studied. Remissions were significantly more frequent during both pregnancy and postpartum periods, while exacerbations were observed only in one-sixth of the patients (P < 0.001). Pregnancy complications (P < 0.001), cesarean section (P < 0.001), and miscarriage (P < 0.02) rates, however, were significantly higher in the study group.
CONCLUSIONS
Our study suggests that pregnancy does not have a deleterious effect on the course of BD and may possibly ameliorate its course. However, it seems that BD may adversely affect pregnancy. The miscarriage rate was higher, and the pregnancy complications and cesarean section rates were significantly elevated.
Topics: Abortion, Spontaneous; Adult; Behcet Syndrome; Case-Control Studies; Cesarean Section; Female; Humans; Israel; Pregnancy; Pregnancy Complications
PubMed: 16167908
DOI: 10.1111/j.0001-6349.2005.00761.x