Behcet's Disease Research Articles

Anticoagulant Treatment May Decrease the Relapse Rate of Pulmonary Arterial Involvement in Behçet's Disease.

Pulmonary arterial involvement (PAI) is one of the most common causes of mortality in Behçet's disease (BD). In this study, we aimed to evaluate the clinical features, course, and recurrence risk factors of BD-associated PAI. BD patients who were followed up in Marmara University BD outpatient clinic between 1990 and 2023 were included. All data were acquired from the medical records of the patients. PAIs were classified according to the type of the vascular involvement as thrombosis or aneurysm. Factors affecting the risk of relapses were determined using multivariate Cox regression analysis. Among 1350 BD patients, 110 (8.1%) had PAI. The mean age (SD) of patients with PAI was 42.4 (11.6) years, and the male/female ratio was 2.2 (76/34). Thirty-two (29.1%) of 110 patients were asymptomatic. Asymptomatic patients with PAI were significantly younger (p = 0.031) than the symptomatic group. Also, a greater prevalence of females (p = 0.001) and higher recurrence rates (p = 0.019) were observed in the symptomatic group. Pulmonary arterial thrombosis was seen in 104 (94.5%) and aneurysms in 9 patients (6.6%). At least one PAI relapse was observed in 31 patients (28.2%). In multivariate analysis, the Cox regression model was significant (p = 0.013), and not starting anticoagulants independently increased the PAI relapse risk (hazards ratio, 4.36; 95% confidence interval, 1.14-24.1; p = 0.042). Pulmonary arterial thrombosis is the main presentation type of PAI in BD, whereas aneurysmatic formation is rare. Despite immunosuppressive treatment, relapses occur during follow-up in one third of patients with PAI. When anticoagulant therapy is added to immunosuppressive therapy, the relapse rate in BD patients with PAI is significantly reduced.

Real-World Safety and Effectiveness of Infliximab in 255 Patients with Intestinal, Neurological, and Vascular Behçet's Disease: A Post-Marketing Surveillance.

Behçet's disease (BD) with intestinal, neurological (NBD), and vascular (VBD) manifestations often leads to poor outcomes. Infliximab is approved for the treatment of intestinal BD, NBD, and VBD in Japan; however, evidence regarding its safety and effectiveness in these patients is limited. We conducted a 2-year post-marketing surveillance to evaluate the safety and effectiveness of infliximab in patients with intestinal BD, NBD, and VBD in Japan. This 2-year, multicenter, prospective, observational study included all patients with intestinal BD, NBD, or VBD, who had experiencedan insufficient response to conventional therapies (e.g., glucocorticoids and immunosuppressants/immunomodulators), and initiated infliximab for the first time at participating medical institutions. The safety endpoints included adverse events and adverse drug reactions (ADRs), and the effectiveness endpoints included global improvement, and for patients with acute NBD, acute attacks. Between October 2015 and August 2018, 255 patients (171 intestinal BD, 49 NBD, and 51 VBD; including 16 with two disease types) were enrolled from 133 medical centers and treated with infliximab. Adverse events, ADRs, and serious ADRs occurred in 100 (39.2%), 72 (28.2%), and 38 (14.9%) patients, respectively; incidences were generally similar across intestinal BD, NBD, and VBD groups. No new safety concerns were identified. At the final evaluation, 68.8% of patients with intestinal BD showed improvement, most patients with chronic progressive NBD and VBD had not worsened (100% and 91.7%, respectively), and 93.3% of patients with acute NBD had no new acute attacks during the observation period. These results confirmed the safety and effectiveness of infliximab in clinical practice in 255 patients with intestinal BD, NBD, and VBD. There were no new safety concerns.

ABO blood groups and increased risk for the development of vascular involvement in Behçet's disease.

Inflammatory endothelial activation is a prominent feature of vascular involvement in Behçet's disease (BD), which is usually accompanied by a thrombotic tendency. Recent studies suggest an association between ABO blood groups and thrombotic vascular disease in those carrying non-O (A, B, and AB) groups. This study aimed to analyse the contribution of ABO blood groups to the vascular involvement in BD. In this retrospective study, BD patients with available ABO blood group data and meeting the ISG criteria were included in the study. The presence of vascular involvement, its clinical characteristics, and the data about other manifestations were recorded from the patient charts. This study was conducted in 411 patients; 143 (34.8%) were carrying O and 268 (65.2%) were carrying non-O groups. The vascular involvement was significantly more frequent and the risk for overall vascular and venous events was up to 2-fold higher in the patients with non-O groups. After adjustments for age, sex, and comorbidities, the risk for arterial disease was also found to be increased in association with non-O groups. ABO blood groups were shown to be independent risk factors for vascular BD by multiple regression models based on known predisposing factors. Compared with other non-O blood groups, the patients carrying blood group B had a higher risk for vascular events. The results of this preliminary study show the potential contribution of ABO blood groups to the vascular-BD phenotype and suggest an increased risk for vascular BD in association with non-O blood groups.

Evaluation of epicardial adipose tissue thickness and carotid intima-media thickness in children with Behçet's disease.

Behçet's disease (BD) is a systemic vasculitis affecting all sizes of arteries and veins. Approximately 5-10% of patients with BD are present during childhood. The chronic nature of the disease may lead to serious cardiovascular complications over time including early atherosclerosis. Increased levels of epicardial adipose tissue (EAT) and carotid intima-media thickness (CIMT) are considered early signs of subclinical atherosclerosis. Ongoing chronic inflammation may cause to increase in both EAT and CIMT. In this study, we aimed to evaluate CIMT and EAT in children with BD and determine their relationship with the clinical manifestations and course of the disease. This cross-sectional study evaluated 30 patients with juvenile-onset BD and age-sex-matched 20 healthy controls. The CIMT and EAT thickness were measured by the same paediatric cardiologist. The association between clinical features, baseline disease activity, disease duration, EAT thickness and CIMT was also evaluated. Thirty children with BD and 20 age-sex-matched healthy volunteers enrolled in the study. The most common BD-related feature was oral aphthous (n=30), followed by mucocutaneous findings (n=22). Uveitis was observed in 5 patients, vascular involvement in 4, neurological involvement in 4, and gastrointestinal involvement in 2. All patients were inactive at the time of evaluation. The EAT thickness was significantly higher in patients while CIMT levels revealed no significant differences. However, there was no correlation between disease duration, baseline disease activity, and EAT thickness. Increased EAT thickness may be a risk factor for early atherosclerosis in patients with BD. The EAT thickness was found to be significantly higher in paediatric BD patients. Confirmation of results in larger series may provide better insight into early screening for risk factors in these patients.

Cytokine Signature Differences in Major Phenotypic Groups of Behçet Disease.

Behçet disease (BD) has heterogeneous presentations, mainly mucocutaneous, vascular, and ocular manifestations. The mechanisms associated with different phenotypes have not been clarified. We aimed to investigate the expression of innate and adaptive immunity-related cytokines in these 3 main BD phenotypes in active and untreated states and remission after treatment to be able to develop a cytokine-based treatment algorithm. Serum samples were isolated from 41 patients with newly diagnosed active BD (aBD), which consisted of 19 mucocutaneous aBD, 11 ocular aBD (o-aBD), and 11 vascular aBD patients, 35 patients in remission (rBD), and 9 healthy controls (HC). Serum levels of each cytokine were measured with sandwich enzyme-linked immunosorbent assay and analyzed as both raw measurements and corrected levels for each 1 million white blood cells. The study included 41 aBD patients (female/male [F/M]: 9/32; median age, 29 years), 35 rBD patients (F/M: 9/26; median age, 29 years), and 9 HC (F/M: 3/6; median age, 28 years). The serum interferon γ level was significantly higher in the aBD group than in the rBD (116 vs. 92 pg/mL, p = 0.022). The serum interleukin 35 (IL-35) level was significantly higher in the HC group compared with aBD and rBD (p = 0.05). IL-17-related cytokines were lower in o-aBD. With treatment, they increased in o-aBD but decreased in mucocutaneous aBD and vascular aBD patients. This study supports the involvement of both innate and TH1-predominated adaptive immune responses across all BD phenotypes. The IL-17 and TH17-related immune responses appear less prominent in ocular BD, which may explain the ineffectiveness of IL-17 blockade in treating ocular BD. These findings support the need for further studies using comprehensive gene expression analyses to develop targeted treatment strategies for BD phenotypes.

Update on Behçet syndrome

Behçet syndrome (BS) is avasculitis of variable vessels with multiple organ manifestations. This article gives an overview of innovations in the last 2 years. A literature search was carried out using the keyword "Behcet" in 2022-2024 in PubMed. The selection of suitable articles was based on the relevance. With respect to the pathophysiology it is now clear that BS occupies an intermediate position between autoinflammatory and autoimmune clinical pictures. It is now classified as MHC-I-opathy, i.e., adisease that has astrong association with HLA classI antigens, which also play aprominent role in the pathogenesis. The diagnostic international criteria for Behcet's disease (ICBD) from 2014 with ascore of 4 points or more that makes the diagnosis of BS probable have become established; however, in countries with alow prevalence of BS, the differential diagnosis of BS from other diseases is difficult and ahigher point limit in the diagnostic score seems to make sense in order to avoid incorrect diagnoses. Clusters or phenotypes of the disease have now been described in various countries in which different symptom complexes frequently occur together; however, the clusters differ between the different countries of origin and depending on the age of the patients. Sonography of the common femoral vein with specific wall thickening in BS patients has been established as an additional tool for the differential diagnosis. Typical characteristics of oral aphthae in BS were also described and the frequency of positivity in the pathergy test could be significantly increased using pneumococcal antigens as the reagent. The treatment recommendations of the EULAR from 2018 still apply; in treatment-refractory cases, tocilizumab, secukinumab, Janus kinase inhibitors (JAKi) and ustekinumab have now also been successfully used. The new EULAR treatment recommendations are expected in 2025.

Advanced Echocardiography in Assessment of Subclinical Right Ventricular Dysfunction in Behcet's Disease.

Advanced Echocardiography in Assessment of Subclinical Right Ventricular Dysfunction in Behcet's Disease.

Role of Optical Coherence Tomography in Vasculitis-Associated Pulmonary Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

Identifying and understanding the microstructural changes within the wall of the pulmonary artery (PA) is crucial for elucidating disease mechanisms and guiding treatment strategies. We assessed the utility of optical coherence tomography (OCT) in identifying such changes within segmental/subsegmental PAs and compared the morphological variations in WHO group 4 pulmonary hypertension associated with Behcet Disease (BD), Takayasu arteritis (TA) and chronic thromboembolic pulmonary hypertension (CTEPH). Idiopathic pulmonary arterial hypertension (IPAH) patients served as controls.Methods and Results: A total of 197 cross-sectional images were analyzed from 20 consecutive patients. BD patients exhibited lower %wall area and mean wall thickness (MWT) compared with CTEPH, TA and, IPAH patients. TA patients showed a notably higher %wall area, which was significant in IPAH and BD patients. Variations in %wall area measurements were observed across distinct cross-sectional segments of the PA within individual patients (22% in CTEPH, 19% in BD, 16% in TA, 23% in IPAH patients). Intravascular webs, bands, and thrombi were observed in BD and CTEPH patients. OCT provided clear delineation of vascular wall calcifications and adventitial vasa vasorum. No procedure-related complications were observed. PA involvement differs among the various etiologies of PH, with the PA being heterogeneously affected. OCT offers promise in elucidating microstructural vascular wall changes and providing insights into disease mechanisms and treatment effects.

Clinical characteristics, treatment strategy, and clinical outcomes in type 2 intestinal failure

Objective: To evaluate the characteristics, clinical management and clinical outcomes of type 2 intestinal failure (IF). Methods: A descriptive case-control study was carried out. The inclusion criteria were as follows: (1) the diagnosis of IF was performed according to the European Society for Parenteral and Enteral Nutrition (ESPEN) consensus statement. (2) using a requirement for parenteral nutrition (PN) of 28 days or more as surrogate marker. (3) a multidisciplinary team (MDT) included surgeons, nutritionist, pharmacist, stoma therapists, and critical care physicians. (4) complete laboratory data. Patients with type 1 and type 3 IF and those who do not cooperate with follow-up. All the data of 67 type II IF were collected from the database in Sir Run Run Shaw Hospital from Jan 2016 to Dec 2023. The pathophysiology, clinical management, and outcomes of type II IF were analyzed. Results: A total of 67 type II IF were included. The median age was 54 (15-83) with 43 males and 24 females. The body mass index was (17.5±3.8) kg/m2, the incidence of malnutrition was 67.2% (45/67), the incidence of sarcopenia was 74.6% (50/67), the median number of previous surgeries was 2.0 (1-13), and the median duration time of PN was 2.1 (1-12) months. The underlying disease of type 2 IF included 36 Crohn`s disease, 2 ulcerative colitis, 3 radiation enteritis, 2 intestinal Behcet's disease, 4 mesenteric infarction, 1 aggressive fibromatosis, 5 abdominal cocoon syndrome, 5 gastrointestinal perforation, 1 hernia, 4 intestinal dysmotility, and 4 other reasons (gastrointestinal tumor, trauma, and non-Hodgkin's lymphoma). According to the pathophysiology of IF, there were 33 intestinal fistula, 12 intestinal dysmotility, 6 mechanical obstruction, 13 short bowel syndrome, and 3 extensive small bowel mucosal disease. After treatment with MDT, 67 patients with type 2 IF received nutritional support therapy for intestinal rehabilitation treatment, of which 36 patients recovered with oral diet or enteral nutrition, 31 patients underwent reconstructive surgery after intestinal rehabilitation treatment failure. The median duration time of reconstructive surgery was 2.7 (1-9) months. 24 patients recovered intestinal autonomy after surgery, with 7 deaths, including 6 deaths due to abdominal infections and 1 case of intestinal dysmotility with abiotrophy and liver failure. Conclusion: Standardized multidisciplinary treatment plays an important role in type II intestinal failure, and it promotes patients with intestinal failure regain enteral autonomy.

Interpreting the Function of the IL-23/IL-17 Axis through Bioinformatics.

Bioinformatic analysis is a valuable tool that allows us to collect, archive, analyze, and disseminate biological data for further interpretation. Analysis of the IL-23/IL-17A axis and its receptors will provide us with essential information about their functions, interactions, and relationships with various diseases. This review aims to identify the central genes co-expressed in the IL-23/IL-17A axis and their receptors and to understand their ontology and modifying factors. We used several databases, including COXPRESdb to obtain the co-expressed genes, ShinyGO and ToppGene platforms to explore gene functional enrichment, and the NetworkAnalyst 3.0 platform for gene expression profiling. We found that genes encoding IL-23/IL-17A axis proteins and their receptors mainly respond to microbial components, participate in the inflammatory response, and are primarily associated with inflammatory and autoimmune diseases. In addition, we observed an association of the IL-23/IL-17 axis with Behcet's disease, Graft-versus-host disease, and Hodgkin's disease, although there is no direct evidence of their interaction. The IL-23/IL-17A axis is associated with several inflammatory and autoimmune pathologies. Therefore, we suggest further research to confirm its role in these pathologies and, if possible, use it as a therapeutic target.

MASSIVE HEMORRHAGE DUE TO RUPTURE OF A RIGHT ILIAC ANEURYSM REVEALING BEHCET'S DISEASE: A CASE REPORT FROM THE FESTOC CENTER IN BAMAKO

INTRODUCTION: Behçet's disease is a chronic inflammatory disorder. It is clinically characterized by oral and genital aphthoses; skin lesions and systemic manifestations. Arterial aneurysms are defined as a loss of parallelism of the arterial walls, localized to an arterial segment, and corresponding to an increase of at least 50% of the normal arterial diameter. We report a case of Behcet's disease revealed by a ruptured aneurysm of the right primitive iliac artery. OBSERVATION: This was a 44-year-old patient with no known history, referred for management of a right inguinal mass that had progressively increased in volume and had been evolving for about 1 month. On admission, the physical examination revealed conjunctival pallor, a pulsatile right inguinal mass approximately 10 cm in diameter, and a suppurating sore on the scrotum. Doppler ultrasound of the lower limbs revealed an aneurysm of the right common iliac artery. The aneurysm ruptured during the short preparation period for surgery, resulting in cataclysmic hemorrhage. This prompted manual compression and the transfusion of several packed red blood cells. During transport to the OR, the patient experienced hemorrhagic shock and altered consciousness, prompting the administration of drugs (noradrenaline) and emergency orotracheal intubation. The aneurysm was controlled upstream and downstream by approaching the right primitive iliac artery, followed by dissection and clamping after general heparinization. The common femoral artery was then approached and checked. Surgical exploration revealed numerous clots which were evacuated, revealing a large neck extending over 7 cm. The procedure involved reconstruction of the common femoral artery with a patch saphenous graft and excision of the necrotic tissue. The post-operative course was marked by suppuration of the surgical wound, which progressed well with appropriate antibiotics and local care. CONCLUSION: Arterial involvement in Behçet's disease manifests as arterial thrombosis or, more frequently, aneurysm. This is due to inflammation of the media, intima and vasa vasorum. Their management can be envisaged in our context.

Behçet's disease modifies the gingival inflammatory response.

Behçet's disease (BD) pathogenesis involves severe outcomes such as blindness, central nervous system manifestations, and deep venous thrombosis that impacts systemic and local inflammatory changes. We tested the hypothesis that BD negatively affects gingival health and increases the severity of gingivitis. The study included 37 BD patients with gingivitis without any sign of periodontitis. Systemically healthy 19 patients with gingivitis (G) and 20 periodontally and systemically healthy individuals (C) were recruited as controls. BD patients were further grouped as stable and unstable based on their responses to BD treatment. Clinical periodontal parameters were measured to determine the impact of BD on gingival health. Serum and saliva levels of ELA-2 (neutrophil elastase-2), SLPI (secretory leukocyte protease inhibitor), α1-AT (alpha1-anti-trypsin), VEGF (vascular endothelial growth factor), IL-6 (interleukin-6), IL-8 (interleukin-8), and TNF-α (tumor necrosis factor alpha) were analyzed using multiplex immunoassay to measure the systemic and local inflammatory impact of BD. Plaque index (PI), probing pocket depth (PPD), and bleeding on probing (BOP) were significantly higher in the BD group than in the controls (p<0.05). IL-6 was higher in both serum and saliva in the BD group than in the G group (p<0.05). ELA-2 levels in saliva were higher in the stable BD group than in the controls, while TNF-α and SLPI were statistically significantly higher in BD than in the control (p<0.05). Salivary α1-AT level was statistically lower in the BD group compared to the control group. Our study suggested that the gingival inflammatory profile was impaired in patients with BD.

Transcription factor EGR2 alleviates autoimmune uveitis via activation of GDF15 to modulate the retinal microglial phenotype

Uveitis is a vision-threatening disease primarily driven by a dysregulated immune response, with retinal microglia playing a pivotal role in its progression. Although the transcription factor EGR2 is known to be closely associated with uveitis, including Vogt-Koyanagi-Harada disease and Behcet's disease, and is essential for maintaining the dynamic homeostasis of autoimmunity, its exact role in uveitis remains unclear. In this study, diminished EGR2 expression was observed in both retinal microglia from experimental autoimmune uveitis (EAU) mice and inflammation-induced human microglia cell line (HMC3). We constructed a mice model with conditional knockout of EGR2 in microglia and found that EGR2 deficiency resulted in increased intraocular inflammation. Meanwhile, EGR2 overexpression downregulated the expression of inflammatory cytokines as well as cell migration and proliferation in HMC3 cells. Next, RNA sequencing and ChIP-PCR results indicated that EGR2 directly bound to its downstream target growth differentiation factor 15 (GDF15) and further regulated GDF15 transcription. Furthermore, intravitreal injection of GDF15 recombinant protein was shown to ameliorate EAU progression in vivo. Meanwhile, knockdown of GDF15 reversed the phenotype of EGR2 overexpression-induced microglial inflammation in vitro. In summary, this study highlighted the protective role of the transcription factor EGR2 in AU by modulating the microglial phenotype. GFD15 was identified as a downstream target of EGR2, providing a unique target for uveitis treatment.

The Current and Novel Imaging Modalities for Ocular Vasculitis in Behcet's Disease: A Review.

Behcet's syndrome is a multisystem inflammatory disorder characterized by a chronic relapsing course and diverse clinical manifestations, prominently affecting young adults along the ancient Silk Road and beyond. The disease pathogenesis involves complex interactions between genetic predisposition, environmental triggers, and dysregulated immune responses, leading to systemic vasculitis and tissue damage. Ocular involvement, a hallmark of Behcet's Disease (BD), significantly impacts morbidity, with uveitis as a common initial presentation that can progress to severe vision-threatening complications like retinal vasculitis and occlusive disease. This review consolidates current knowledge on ocular manifestations in BD, emphasizing the pivotal role of multimodal imaging in diagnostic evaluation and management. Fundus photography serves as a baseline tool for documenting intraocular lesions and monitoring treatment responses. Fluorescein angiography remains the gold standard for detecting acute inflammatory changes and vascular leakage patterns essential for disease staging and prognostication. Recent advancements in imaging, such as ultra-wide field imaging (UWF), indocyanine green angiography (ICGA), Doppler ultrasonography, Optical Coherence Tomography (OCT), OCT angiography (OCTA), adaptive optics (AO), and retinal function imaging (RFI), provide unprecedented insights into microvascular dynamics, structural changes, and functional impairments associated with ocular BD. Integration of these advanced imaging modalities enhances early detection of subclinical disease, facilitates precise localization of inflammatory lesions, guides therapeutic interventions, and monitors treatment efficacy. OCT and OCTA, in particular, offer non-invasive, high-resolution assessments of macular edema, vascular perfusion abnormalities, and choroidal thickness alterations critical for optimizing patient care. In conclusion, multimodal imaging represents the cornerstone in the comprehensive management of ocular manifestations in Behcet's Disease, offering clinicians invaluable tools for accurate diagnosis, treatment planning, and long-term monitoring of disease progression and treatment outcomes.

Swinging Between Hope and Despair: Experiences of Patients with Behçet's Disease: A Phenomenological Research Study

Background: Behçet's disease (BD) can severely impact the psychosocial and emotional aspects of patients, significantly disrupting their quality of life. Objectives: The purpose of this study was to describe the psychosocial and emotional experiences of patients with BD and their coping strategies. Methods: This study was conducted using a qualitative method with a hermeneutic phenomenological approach. Data were collected through unstructured and in-depth interviews (9 conducted face-to-face and 6 via WhatsApp video calls) until data saturation was reached. Face-to-face interviews took place at the rheumatology clinic and the rheumatologist’s office from April to July 2023. Data analysis was performed using Diekelmann's seven-step method. The trustworthiness of the study was ensured based on the criteria of Lincoln and Guba, as well as Sim and Sharp. Results: By analyzing the data, 580 codes were extracted, leading to the identification of 12 sub-themes and 2 main themes. The first theme, "emotional, mental, and communicative instability," included the sub-themes of indecisiveness and uncertainty, moving away from desires and wishes, feelings of despair and demise, fears caused by the disease outcomes, sadness and mental anguish, isolationism, and reluctance to seek support. The second theme, "psychological and communicative strengthening strategies," comprised the sub-themes of having an occupation, seeking support, hoping for miracles and healing, doing mental exercises, and engaging in hobbies. Conclusions: This study revealed numerous emotional, psychological, and communicative challenges faced by patients with BD, as well as the strategies they use to cope with these problems. It is recommended that the findings of this study be used to enhance the care team's understanding of the mental and communicative issues experienced by these patients and to improve their skills in teaching psychological strengthening strategies.

Detecting the presence of anti-hla antıbodies in autoimmune diseases

Aim: By creating antibodies, the immune system protects the body from foreign antigens. The immune system may occasionally sustain damage that results in a loss of tolerance to its antigens, which triggers the creation of antibodies directed against those antigens. Another challenge in solid organ transplantation is the existence of these anti-HLA antibodies. Our goal was to find out if common autoimmune disorders such type 1 diabetes (T1D), Behçet's disease (BD), and ankylosing spondylitis (AS) result in the development of anti-HLA antibodies. Materials and Methods: 100 patients with AS, 100 patients with BD, 60 patients with T1D, and 100 healthy people were included in this study. PRA screening tests were performed on serum from blood samples taken from both patients and healthy individuals to look for the presence of anti-HLA antibodies. Results: Of the AS patients, 5 were positive for PRA class II alone, 7 were positive for both PRA class I and class II, and 1 patient was positive for PRA class I. In BD, 3 patients was positive for both PRA class I and II, 7 patients was positive for PRA class II alone, and 1 patient was positive for PRA class I. In T1D, 1 patient were positive for PRA class I, 3 patients were positive for PRA class II alone, and 2 patients were positive for both. In summary: When three patient groups were compared with the control group, there was no statistically significant difference in the detection of anti-HLA antibodies between the patient groups and the control group.

Epidemiology of Neuro-Behçet's Disease in Northern Spain 1999-2019: A Population-Based Study.

Background/Objectives: Neuro-Behçet's disease (NBD) is one of the most severe complications of Behçet's disease (BD). The incidence of NBD varies widely worldwide. This study aimed to estimate its current incidence in Northern Spain. Methods: This was a retrospective population-based cohort study of 120 patients in Northern Spain diagnosed with BD according to the 2013 International Criteria for BD (ICBD) between 1 January 1999 and 31 December 2019. NBD diagnoses were made according to International Consensus Recommendation (ICR) criteria. Overall, 96 patients were included, and their demographic and clinical data were collected. The incidence of NBD was estimated by age, gender, and year of diagnosis between 1999-2019. Results: NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44 ± 13.9 years). HLA-B51 was positive in 5 of 13 (38.4%) cases tested. A total of 10 (43.5%) patients had parenchymatous NBD, 10 (43.5%) had non-parenchymatous NBD, and 3 (13%) had mixed NBD. Incidence during the study period was 0.13 (95% CI, 0.11-0.26) per 100,000 people-years. There were no significant differences in gender in the incidence rate stratified by age (p > 0.05). Furthermore, there was a linear relationship with a mild decrease in age at diagnosis over time. Conclusions: Epidemiological characteristics of NBD in Northern Spain are similar to those of neighboring countries, except female gender predominance.

Case Report on Ulcerative Lesion? Behcet's Disease

Case Report on Ulcerative Lesion? Behcet's Disease

Aortic root replacement is superior to isolated aortic valve replacement for aortic regurgitation secondary to Behcet's disease.

Aortic root replacement is superior to isolated aortic valve replacement for aortic regurgitation secondary to Behcet's disease.

Intestinal vascular diseases misdiagnosed as Crohn ​disease: analysis of 13 cases

Behçet ​disease (BD), enterocolic lymphocytic phlebitis (ELP), idiopathic myointimal hyperplasia of mesenteric veins (IMHMV), and mesenteric arteriovenous dysplasia/vasculopathy (MAVD/V) are rare vascular diseases (VDs) that cause bowel ischaemia, often presenting with a histological pattern resembling chronic enteritis, leading to diagnostic confusion with Crohn ​disease (CD). In this retrospective study, we compared the clinical and pathological characteristics of these VDs with those of CD. The study cohort comprised 13 patients misdiagnosed with CD but later identified as having VDs, including five, three, two, and three patients with BD, ELP, IMHMV, and MAVD/V, respectively. Moreover, 15 patients diagnosed with CD served as the control group. Data on disease history, patient demographics, symptoms and signs, endoscopic findings, clinical diagnosis, and follow-up status were collected, and the histological features of VDs were compared with those of CD. Despite substantial overlap in clinical and pathological characteristics between VDs and CD, several histological features were helpful in differentiating the two conditions. Intestinal stenosis, multisegmental disease, transmural inflammation, inflammatory polyps, submucosal lymphangiectasia, intramural abscesses, and epithelioid granulomas were significantly more frequent in patients with CD (p<0.001). In contrast, toxic ischaemic changes and vascular lesions were significantly more frequent in patients with VDs (p<0.001). The location of diseased vessels varied widely in patients with VDs, whereas vascular changes were limited to areas with more severe inflammation in those with CD. Furthermore, inflammatory cells within diseased vessels exhibited a polarised distribution in cases of CD, manifesting in two distinct patterns: pattern 1, where the inflammatory cells are densely populated in the outer layer of the vessel wall and sparsely populated in the inner layer; pattern 2, where the inflammatory cells are densely populated on the side with the ulcer and sparsely populated on the opposite side. Vascular occlusion, which was observed in all patients with VDs, was rare in those with CD. In summary, VDs and CD can be distinguished through careful histological analysis, particularly focusing on characteristics of vascular changes, in combination with medical history.