Novel genetic variants of HLA gene associated with Thai Behcet's disease (BD) patients using next generation sequencing technology.

Behçet's disease (BD) manifests as an autoimmune disorder featuring recurrent ulcers and multi-organ involvement, influenced by genetic factors associated with both HLA and non-HLA genes, including TNF-α and ERAP1. The study investigated the susceptible alleles of both Class I and II molecules of the HLA gene in 56 Thai BD patients and 192 healthy controls through next-generation sequencing using a PacBio kit. The study assessed 56 BD patients, primarily females (58.9%), revealing diverse manifestations including ocular (41.1%), vascular (35.7%), skin (55.4%), CNS (5.4%), and GI system (10.7%) involvement. This study found associations between BD and HLA-A*26:01:01 (OR 3.285, 95% CI 1.135-9.504, P-value 0.028), HLA-B*39:01:01 (OR 6.176, 95% CI 1.428-26.712, P-value 0.015), HLA-B*51:01:01 (OR 3.033, 95% CI 1.135-8.103, P-value 0.027), HLA-B*51:01:02 (OR 6.176, 95% CI 1.428-26.712, P-value 0.015), HLA-C*14:02:01 (OR 3.485, 95% CI 1.339-9.065, P-value 0.01), HLA-DRB1*14:54:01 (OR 1.924, 95% CI 1.051-3.522, P-value 0.034), and HLA-DQB1*05:03:01 (OR 3.00, 95% CI 1.323-6.798, P-value 0.008). However, after Bonferroni correction none of these alleles were found to be associated with BD. In haplotype analysis, we found a strong linkage disequilibrium in HLA-B*51:01:01, HLA-C*14:02:01 (P-value 0.0, Pc-value 0.02). Regarding the phenotype, a significant association was found between HLA-DRB1*14:54:01 (OR 11.67, 95% CI 2.86-47.57, P-value 0.001) and BD with ocular involvement, apart from this, no distinct phenotype-HLA association was documented. In summary, our study identifies specific HLA associations in BD. Although limited by a small sample size, we acknowledge the need for further investigation into HLA relationships with CNS, GI, and neurological phenotypes in the Thai population.

Development and validation of a risk prediction model for valve regurgitation in Behçet's disease.

BACKGROUND: In Behçet's disease (BD), mild-to-severe valvular regurgitation (VR) poses a serious complication that contributes significantly to heart failure and eventually death. The accurate prediction of VR is crucial in the early stages of BD subjects for improved prognosis. Accordingly, this study aimed to develop a nomogram that can detect VR early in the course of BD. METHODS: One hundred seventy-two patients diagnosed with Behçet's disease (BD) were conducted to assess cardiac valve regurgitation as the primary outcome. The severity of regurgitation was classified as mild, moderate, or severe. The parameters related to the diagnostic criteria were used to develop model 1. The combination of stepAIC, best subset, and random forest approaches was employed to identify the independent predictors of VR and thus establish model 2 and create a nomogram for predicting the probability of VR in BD. Receiver operating characteristics (ROC) and decision curve analysis (DCA) were used to evaluate the model performance. RESULTS: Thirty-four patients experienced mild-to-severe VR events. Model 2 was established using five variables, including arterial involvement, sex, age at hospitalization, mean arterial pressure, and skin lesions. In comparison with model 1 (0.635, 95% CI: 0.512-0.757), the ROC of model 2 (0.879, 95% CI: 0.793-0.966) was improved significantly. DCA suggested that model 2 was more feasible and clinically applicable than model 1. CONCLUSION: A predictive model and a nomogram for predicting the VR of patients with Behçet's disease were developed. The good performance of this model can help us identify potential high-risk groups for heart failure. Key Points • In this study, the predictors of VR in BD were evaluated, and a risk prediction model was developed for the early prediction of the occurrence of VR in patients with BD. • The VR prediction model included the following indexes: arterial involvement, sex, age at hospitalization, mean arterial pressure, and skin lesions. • The risk model that we developed was better and more optimized than the models built with diagnostic criteria parameters, and visualizing and personalizing the model, a nomogram, provided clinicians with an easy and intuitive tool for practical prediction.

Upper gastrointestinal involvement of Behçet's disease in Japan: endoscopic findings and clinical features.

AIM: Behçet's disease (BD) can involve any gastrointestinal (GI) tract site. We analyzed the characteristics, risk factors, and treatment responses to upper GI (UGI) involvement in patients with BD. METHODS: This retrospective cohort study analyzed UGI findings in 101 patients with BD who underwent endoscopy between April 2005 and December 2022 at the University of Tokyo Hospital. The patients were divided into two groups based on the presence or absence of UGI findings. Patient backgrounds, clinical symptoms, colonoscopy (CS) findings, and blood test findings were compared between the groups. RESULTS: In total, 18.8% (19/101) of the patients had UGI lesions. The prevalence rates in the esophagus, stomach, and duodenum were 6.9%, 6.9%, and 8.9%, respectively. Of these 19 patients, BD treatment were intensified in 10 (52.6%) patients after esophagogastroduodenoscopy (EGD), and all showed improvement in symptoms or endoscopic findings. In the multivariate analysis, symptoms (OR: 37.1, P < 0.001), CRP > 1 mg/dL (OR: 11.0, P = 0.01), and CS findings (OR: 5.16, P = 0.04) were independent predictors of UGI involvement in BD patients. The prediction model for UGI involvement using these three factors was highly accurate, with an AUC of 0.899 on the ROC curve. In the subgroup analysis of intestinal BD, symptoms (OR: 12.8, P = 0.01) and ESR > 20 mm/h (OR: 11.5, P = 0.007) were independent predictors. CONCLUSIONS: EGD should be conducted in BD patients with high CRP, GI symptoms, and lower GI involvement, which leads to better management of BD in terms of improving symptoms and endoscopic findings.

Trends in health care of patients with vasculitides, including giant cell arteritis, Takayasu arteritis, ANCA-associated vasculitis and Behçet's disease: cross-sectional data of the German National Database 2007-2021.

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO's have not improved. Work participation increased but remains lower than that in the German population.

Behçet's syndrome: recent advances to aid diagnosis.

Behçet's syndrome is a recurring inflammatory multiorgan disorder affecting the skin, mucosa, eyes, joints, stomach, and central nervous system. Behçet's syndrome epidemiology varies greatly among populations (0.64-420/100,000), and Behçet's syndrome has gained increasing international acclaim in the recent 50 years due to raising awareness of the syndrome, although it is rare in most population. In addition to the unclear etiology of the syndrome, the diagnosis of Behçet's syndrome is complicated by a vague clinical presentation, phenotypic heterogeneity and/or incomplete representation, and the lack of any specific laboratory, radiographic, or histological findings. There exists a dire need to elucidate factors that contribute to disease pathogenesis and/or are associated with clinical features of Behçet's syndrome and the classification of different forms of the syndrome. The identification of such molecular, cellular, and/or clinical factors are crucial for timely diagnosis and efficacious management of Behçet's syndrome. We discuss recent advances in the clinical diagnosis of Behçet's syndrome and related contributions of genetics, epigenetics, microbiome, inflammasomes, and autoantibodies to the improved diagnosis, management, and understanding of Behçet's syndrome.

Behçet's syndrome: one year in review 2023.

This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical features and management. Studies on pathogenesis included potential biomarkers mostly related to macrophages, neutrophil and cytokine balance, new GWAS and polymorphism studies, and studies on miRNAs and long non-coding RNAs. Clinical studies showed that application of pneumococcal vaccine to the prick site increased the sensitivity and specificity of the pathergy test and the prevalence of AA amyloidosis had decreased over the years. Studies on management indicated that more data are needed to understand the effect of apremilast on BS manifestations other than oral ulcers, and new BS manifestations may develop during treatment with infliximab. Other biologics and Jak inhibitors might be an option for patients who are refractory to TNF-α inhibitors. Moreover, endovascular repair of arterial aneurysms might be an alternative to open surgery.

A Comparison of Adult and Juvenile Behcet Patients and a Look at Clinical Trends: Retrospective Data from a Turkish Follow-Up Cohort Study.

BACKGROUND: Previous reports indicate that juvenile Behçet's disease (BD) may have a different course than adult BD. However, as a direct comparison with adult Behçet patients has only been made in a limited number of studies, the issue is still controversial. OBJECTIVES: The primary aim of our study was to compare clinical manifestations in a large cohort of juvenile and adult Behçet patients registered in a single centre. The secondary aim of our study was to compare the data of newly diagnosed patients registered between 1998 and 2020 with the data of those registered between 1976 and 1997. METHODS: Data were collected retrospectively from medical records of patients registered between 1998 and 2020. Juvenile BD was defined as fulfilment of International Criteria for Behçet's Disease at or before 16 years of age. RESULTS: A similar course of disease was noted in juvenile and adult Behçet patients with no significant difference in the frequency of mucocutaneous findings, major organ involvement, and positivity of the pathergy test. A comparison of the periods, 1976-1997 and 1998-2020, revealed no significant difference in the prevalence of mucocutaneous lesions and major organ involvement. CONCLUSIONS: Our results indicate that juvenile and adult Behçet patients have a similar course with a similar frequency of clinical manifestations. Contrary to reports suggesting an overall tendency to milder disease over time, no decrease in the risk of major organ involvements was observed. A significant trend towards a decline in pathergy test positivity was noted.

Unfolding dermatologic spectrum of Behçet's disease in Italy: real-life data from the International AIDA Network Behçet's disease Registry.

Behçet's disease (BD) is a heterogeneous multifactorial autoinflammatory disease characterized by a plethora of clinical manifestations. Cutaneous lesions are considered hallmarks of the disease. However, their evolution over time and a thorough description are scarcely reported in non-endemic regions. The aim of this study was to detail BD skin manifestations and their evolution over time in Italy, as well as the dermatological prognostic impact of specific cutaneous features in long-standing disease. Data were collected in a double fashion, both retrospectively and prospectively, from the AutoInflammatory Disease Alliance (AIDA) international registry dedicated to BD, between January 2022 and December 2022. A total of 458 Italian patients were included. When assessing skin manifestations course, the constant or sporadic presence or absence of cutaneous involvement between onset and follow-up was considered. Oral ulcers (OU) (88.4%) and genital ulcers (GU) (52.6%), followed by skin involvement (53.7%) represented the most common presenting mucocutaneous manifestations at disease onset. Up to the time of enrolment into the AIDA registry, 411 (93.8%) patients had suffered from OU and 252 (57.9%) from GU; pseudofolliculitis (PF) accounted for the most common skin manifestation (170 patients, 37.1%), followed by erythema nodosum (EN) (102 patients, 22.3%), skin ulcers (9 patients, 2%) and pyoderma gangrenosum (4 patients, 0.9%). A prospective follow-up visit was reported in 261/458 patients; 24/148 (16.2%) subjects with skin involvement as early as BD onset maintained cutaneous lesions for the entire period of observation, while 120 (44.1%) patients suffered from sporadic skin involvement. Conversely, 94/113 (83.2%) with no skin involvement at disease onset did not develop skin lesions thereafter. At follow-up visits, cutaneous involvement was observed in 52 (20%) patients, with a statistically significant association between PF and constant skin involvement (p = 0.031). BD in Italy is characterized by a wide spectrum of clinical presentations and skin manifestations in line with what is described in endemic countries. Patients with skin disease at the onset are likely to present persistent cutaneous involvement thereafter; mucocutaneous lesions observed at the onset, especially PF, could represent a warning sign for future persistent skin involvement requiring closer dermatological care.

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency.

OBJECTIVE: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. METHODS: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. RESULTS: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. CONCLUSIONS: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Adamantiades-Behçet disease: Between dermatology and ophthalmology.

Adamantiades-Behçet disease is an inflammatory, vascular disease of unknown etiology. The disease is named after two physicians, Benediktos Adamantiades and Hulȗsi Behçet, who both made significant contributions to the study of the disease. It was probably first described by Hippocrates in 500 BCE. Adamantiades-Behçet disease is most common in the region encompassing the ancient trade route known as the Silk Road. In Turkey, the disease is estimated to affect 80 to 370 people per 100,000 inhabitants, and it is also the country with the highest incidence rate. The frequency of the disease associated with the clinical picture differs from the origin of the onset. The disease is characterized by recurrent aphthous ulcers of the mouth, genitals, skin lesions, and eye lesions. The disease process can also involve other organs, including the joints, nervous system, large vessels, heart, and gastrointestinal tract. Aphthous oral ulcers appear as the first harbinger of the disease and affect almost all patients (97%-99%). The scientific interest in Adamantiades-Behçet disease has increased exponentially in the past decade.

Clinical features of Behçet's disease and prediction of the use of biologics in 488 cases: a single tertiary center study.

The objectives are to describe the demographic and clinical properties of Behçet's disease (BD) and investigate their relationship with the use of biological agents. Four hundred-eighty-eight patients, (299 (61.3%) males, 189 (38.7%) females), who fulfilled the ISG classification criteria for BD were included, retrospectively. The patient's demographics, disease onset age (DOA), clinical findings of the disease, and the drugs were determined and analyzed statistically. The means of patient age and DOA were 40.7 ± 9.9 and 30.8 ± 8.8 years, respectively. The most common initial findings were oral ulcer (OU)s (30.1%), genital ulcer (GU) (27.5%), ocular involvement (OI) (12.5%), and papulopustular lesion (PPL)s (10.1%). The most common clinical manifestations were OUs (96.9%), PPLs (70.2%), HLA-B51 (64.4%), positive pathergy reaction (26.4%), GU (58.8%), OI (44.7%), erythema nodosum (29.8%), and vascular involvement (VSI) (27.3%). Although, the frequency of GU was higher in females (p = 0.01), PPLs (p = 0.001) and VSI (p = 0.001) were higher in males. Sixty-three (8.9%) patients used a biological agent. Its frequency was higher in younger patients (< 40 years) (p = 0.006), males (p = 0.012) and patients with OI (p = 0.001). Besides, the DOA (p = 0.012) and the current age (p = 0.001) were lower in biological agent users. The possibility of using biological agent was increased in males (OR = 2.2), patients with OI (OR = 2.7) and young patients (OR = 0.9). Mucocutaneous lesions are distinctive features of BD, especially OUs precede other findings. GU was more common in females and PPLs and VSI were in males. The probability of using biologics is higher in males, patients with OI, and young patients.

The large Hellenic Study of Uveitis: epidemiology, etiologic factors and classification.

PURPOSE: To analyse the demography, etiology, and classification of uveitis at a tertiary academic referral center. METHODS: An observational study was conducted on the archives of uveitic patients at the Ocular Inflammation Service of the Department of Ophthalmology at the University Hospital of Ioannina (Greece) from 1991 to 2020. This study aimed to investigate the epidemiological profile of patients, including their demographics and the main etiologic factors of uveitis. RESULTS: Out of 6191 cases with uveitis, 1925 were infectious, 4125 were non-infectious, and an overall of 141 masquerade syndromes were recorded. Among these cases, 5950 patients were adults, with a slight female predominance, while 241 were children (< 18 years old). Interestingly, 24.2% of cases (1500 patients) were associated with 4 specific microorganisms. Herpetic uveitis (HSV-1 and VZV/HZV) was the most common cause of infectious uveitis (14.87%), followed by toxoplasmosis (6.6%) and tuberculosis (2.74%). In 49.2% of non-infectious uveitis cases, no systematic correlation was found. The most frequent causes of non-infectious uveitis included sarcoidosis, white dot syndromes, ankylosing spondylitis, lens-induced uveitis, Adamantiades-Behçet disease, and idiopathic juvenile arthritis. Infectious uveitis was more common in the rural population, whereas non-infectious uveitis was more frequently recorded in the urban population CONCLUSIONS: Although our study was conducted on a predominantly white Caucasian population, it also reflects the effect of increasing immigration, improvements of diagnostic techniques, changes in referral patterns, and various actual changes in disease incidence.

Pulmonary hypertension in Behçet's disease: echocardiographic screening and multidisciplinary approach.

BACKGROUND: Little is known about the prevalence and causes of pulmonary hypertension (PH) in Behçet's disease (BD). This study was conducted to determine the prevalence and causes of PH in BD. METHODS: In this descriptive study, we screened 154 patients with BD for PH using transthoracic echocardiography between February 2017 and October 2017. An estimated systolic pulmonary arterial pressure (sPAP ≥ 40 mmHg) was used as the cutoff value to define PH. Patients with BD were categorized into 5 groups according to organ involvement including mucocutaneous/ articular, ocular, vascular, gastrointestinal, and neurologic involvement. Additional laboratory and imaging results were obtained from hospital file records to determine the causes of PH. RESULTS: PH was detected in 17 (11%) patients. Nine (52.9%) of these patients had group II PH (due to left heart disease), 4 (23.5%) had IV PH (due to pulmonary arterial involvement), and 1 had III PH (due to chronic obstructive lung disease). The frequency of PH was higher in BD patients with vascular involvement than those without (52.9% vs 28.5%; p = 0.04). Among 10 patients with pulmonary artery involvement (PAI) 4 (40%) had PH. Although the vascular BD group had the highest rate of PH, we observed no statistically significant difference in the frequency of PH between the predefined BD subgroups. DISCUSSION: : PH is not rare in patients with BD. The majority of BD patients with PH are in group II or IV PH. Patients with vascularinvolvement carry a higher risk for the development of PH. Monitoring BD patients with PAI should be considered for the development of group IV PH.

Cardiovascular risk in vasculitis.

The present review summarizes the burden, risk factors, biomarkers of and therapeutic consideration for cardiovascular disease in systemic vasculitis. Ischemic heart disease (IHD) and stroke are intrinsic features of Kawasaki disease, Takayasu arteritis, Giant Cell Arteritis (GCA), and Behcet's disease. The risk of IHD and stroke is increased in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and cryoglobulinemic vasculitis. Behcet's disease could present with venous thromboembolism. The risk of venous thromboembolism is increased in AAV, polyarteritis nodosa, and GCA. The risk of cardiovascular events is greatest at or immediately after the diagnosis of AAV or GCA, therefore, controlling vasculitis disease activity is of utmost importance. Traditional as well as disease-related risk factors drive the heightened cardiovascular risk in vasculitis. Aspirin or statins reduce the risk of IHD or stroke in GCA or the risk of IHD in Kawasaki Disease. Venous thromboembolism in Behcet's disease should be treated with immunosuppressive therapy rather than with anticoagulation.

Characteristics of Patients With Behçet Disease From the Van Province, Eastern Turkey: Definition of Disease Clusters in a Tertiary Referral Center.

BACKGROUND: Behçet disease (BD) is a chronic inflammatory systemic disease that affects skin mucosa, joints, eyes, and blood vessels. Behçet disease shows some clinical differences in terms of disease manifestations and prognosis among the Silk Road countries, as well as various ethnicities even in the same country. In this study, we aimed to evaluate the clinical features and disease course of BD using cluster analysis in Van province, Eastern Turkey. METHODS: This study was carried out in a tertiary referral center in Van province, by reviewing medical records. Seven disease manifestations were included to the cluster analysis as follows: mucocutaneous findings (oral ulcer, genital ulcer, erythema nodosum-like lesions, pseudofolliculitis), uveitis, superficial thrombophlebitis, musculoskeletal involvement, gastrointestinal system involvement, vascular involvement, and parenchymal central nervous system involvement. RESULTS: We identified 467 patients. After the exclusion of 6 patients who had missing data and 17 patients who did not live in Van, 444 patients (59.2% male) included into the study. Meeting the International Study Group and the International Criteria for Behçet Disease criteria were 91.6% and 96%, respectively, and 91.3% (n = 379/415) of these patients met both criteria. Four clusters were identified in the analyses: 132 patients (31.2%) in vascular (C1), 66 patients (15.6%) in ocular (C2), 60 patients (14.2%) in musculoskeletal (C3), and 165 patients (39%) in mucocutaneous (C4) clusters. Male gender ( p = 0.002; odds ratio [OR], 6.5; 95% confidence interval [CI], 2-21.4), superficial thrombophlebitis ( p = 0.001; OR, 4.7; 95% CI, 1.9-11.4), and uveitis ( p = 0.01; OR, 3.6; 95% CI, 1.3-9.9) were associated with vascular involvement in multivariate analysis. CONCLUSIONS: In our study, 4 clusters were detected in patients with BD from Van province. The prevalence of severe manifestations of BD may be related to genetic or environmental factors, such as differences in ethnicity and/or geographical differences. Despite the higher proportion of patients with a more severe disease, a favorable outcome was observed in our cohort.

Clinical Characteristics and Outcomes of Mexican Patients With Behçet's Syndrome.

OBJECTIVE: The aim of this study was to describe the clinical characteristics and outcomes of Mexican patients with Behçet syndrome (BS) from a single center. METHODS: This medical records review study included patients with established BS diagnosis, followed up in a tertiary care center in Mexico City from 2000 to 2020. Demographics, clinical characteristics, laboratory and imaging studies, disease activity, damage, treatment, and outcomes were assessed and compared according to sex and with other international cohorts. Descriptive statistics were used, and differences between groups were evaluated using the χ2 or Mann-Whitney U tests. RESULTS: Thirty-eight patients were included, 23 (60%) women and 15 (40%) men; the median age at BS diagnosis was 33 years (range, 24-39 years). The most frequent manifestations at diagnosis were recurrent oral and genital ulcers in 34 (89%) and 29 (76%), respectively, musculoskeletal in 21 (55%), and cutaneous in 15 (39%). The most frequent phenotype was the joint involvement. Treatment comprised prednisone in all, colchicine (24 [63%]), pentoxifylline (12 [32%]), and thalidomide (10 [26%]). During a median follow-up time of 12.5 years (range, 7-23 years), relapses were frequent (97%), severe infections were present in 5 patients (13%), and 2 patients (5%) died due to sepsis and pneumonia. Men showed a higher frequency of arterial hypertension (47% vs. 13%, p = 0.03) and thrombosis (20% vs. 0, p = 0.05), and a lower frequency of genital ulcers (40% vs. 78%, p = 0.03) than women. CONCLUSIONS: This cohort of Mexican patients with BS showed a predominance of female sex, joint involvement phenotype, frequent relapses, and favorable outcomes. Differences in comorbidities and clinical manifestations were identified according to sex.

The impact of Behcet syndrome on total knee arthroplasty outcomes: a retrospective matched cohort study.

PURPOSE: Articu lar involvement is a common manifestation of Behcet syndrome (BS), which can eventually result in significant arthralgia and necessitate total knee arthroplasty (TKA). However, outcomes of BS patients after TKA are still poorly documented in the literature. The purpose of this study was to evaluate BS as a potential risk factor for complications after TKA. METHODS: BS patients undergoing primary TKA were identified from the PearlDiver Mariner database from 2010 to 2021 and compared to 10:1 matched controls. Multivariable logistic regression analyses were done for medical complications up to 90 days and surgical complications up to two years. Ninety-day emergency department (ED) visit and inpatient readmission were also documented. RESULTS: A total of 4286 patients undergoing primary TKA were queried, of which 390 had BS. Patients with BS demonstrated significantly higher rates of medical complications, including deep venous thrombosis. The rates of surgical complications were similar between the two groups with the exception of periprosthetic instability, aseptic loosening, and wound complications in BS patients. Additionally, a significantly higher rate of ED visits but markedly lower rates of 90-day readmissions were noted in patients with BS. CONCLUSION: Patients with BS undergoing TKA are at higher risks of medical and surgical complications. Special considerations for a unique postoperative course with the higher complications should be made. It is crucial for orthopedic surgeons and patients alike to consider these risks when determining the expected course after TKA for patients with BS.

Epidemiology and clinical domains of Behçet's disease in the Cantabria region, Northern Spain.

OBJECTIVES: The prevalence of Behçet's disease (BD) has a considerable geographical and temporal variability. Data regarding epidemiology in Spain are limited. Our study aimed to assess the epidemiology and clinical domains of BD in a population-based cohort from Northern Spain and to compare the results with other geographical areas of other countries. METHODS: We conducted a cross-sectional study of a well-defined population in Northern Spain. Cases of suspected BD between January 1980 and December 2018 were identified. The diagnosis of BD was established according to the International Study Group (ISG) for Behçet's Disease. The incidence of BD between 1999 and 2018 was estimated by sex, age, and year of diagnosis. RESULTS: Of 120 patients with probable BD, 59 patients met ISG criteria and were finally included in the study, with a male/female ratio of 0.97; mean age 49.7±14.7 years. Incidence during the period of study was 0.492 per 100,000 people, observing an increase from January 1999 to December 2018. Prevalence was 10.14 per 100,000 inhabitants in 2018. Clinical manifestations were relapsing aphthous stomatitis (100%), genital ulcers (78%), skin involvement (84.7%), joint involvement (64.4%), uveitis (55.9%), central nervous system (16.9%), vascular (10.2%), and gastrointestinal manifestations (6.8%). CONCLUSIONS: The prevalence of BD in Cantabria is higher than in other Southern European countries. This difference may reflect a combination of geographic, genetic, or methodological variations, as well as the free accessibility to the Spanish Public Health System for the entire population. Clinical phenotypes observed are similar to those described in other world regions.

The impact of the COVID-19 pandemic on patients with Behçet's syndrome within the framework of Maslow's hierarchy of needs: implications for patient empowerment in national or global emergencies.

OBJECTIVES: The aim of this online survey was to assess the impact of the COVID-19 pandemic on patients with Behçet's syndrome (BS) using Maslow's hierarchy of needs. METHODS: Sixty-eight BS patients from the UK completed an online questionnaire that covered three components of Maslow's hierarchy of needs regarding Physiological needs (sleep, food, mobility), Safety needs (employment, health), Social needs (relationships), personal precautions and main concerns regarding COVID-19. RESULTS: The patients received a letter from the NHS (66.2%) advising shielding. Health concerns were addressed using Tele-medicine by telephone consultations and e-mails to access BS specialists during lockdown (44.1%; 27.9%). In addition, patients also obtained information through official government and Behçet corporate health web pages related to Behçet's disease for self-empowerment. During lockdown, their weekly working hours were reduced (26.65±13.86 vs. 20.79±17.94) (p=0.017) and the employment rate was decreased (32.6% vs. 26.7%) in comparison to pre-lockdown. Moreover, the reduction in frequency of exercise per week (4.39±1.94 vs. 3.71±2.26) and healthy food consumption (73.5 % vs. 47.1%) per week were also reported (p=0.007; p=0.001, respectively). Furthermore, patients experienced sleeping less (54.4%) and feeling lonely (66.1%). CONCLUSIONS: Using Maslow's hierarchy of needs provided a framework for reviewing the quality of basic needs in patients' life during lockdown. These needs are required for healthy social lives and the deprivation should be considered while considering healthcare planning. Since lockdown appeared to have a negative influence on patients' lives and disease management in the framework of patient-centred, the empowerment of patients gained importance at this point.

Association of Behçet's disease with the risk of metabolic syndrome and its components: a systematic review and meta-analysis.

The present meta-analysis aimed to elucidate the association of Behçet's disease (BD) with the risk of metabolic syndrome (MetS) and its components. Observational cohort studies were searched from the Embase, Web of Science, Medline, and Cochrane Library databases. The primary outcome was the association of BD with the risk of MetS and its relevant components. Effect estimates with odds ratios (ORs) were pooled using either the random-effects or fixed-effects models, according to heterogeneity. Leave-one-out sensitivity analyses were used to determine the stability of the results. Twenty-three studies, comprising 42,834 patients with BD, were included. Overall, a significant association between BD and the risk of MetS was found (pooled OR 2.26; 95% confidence interval [CI] 1.61-3.17; P < 0.0001). Among the components of MetS, significant associations were found between BD and diabetes mellitus (OR 1.21; 95% CI 1.10-1.33; P < 0.0001), BD and hypertension (OR 1.39; 95% CI 1.13-1.70; P = 0.002), and BD and dyslipidemia (OR 1.21; 95% CI 1.01-1.45; P = 0.04). Our study indicated an association between BD and the risk of MetS and some of its components (diabetes mellitus, hypertension, and dyslipidemia). Physician should consider these associations so that specific treatments are available for patients with comorbidities. Moreover, patients with BD should regularly monitor their blood pressure, fasting plasma glucose, and blood lipid levels.