Behcet's Disease Research Articles

Accuracy and Performance Characteristics of Administrative Codes for the Diagnosis of Autoinflammatory Syndromes

Objective The aim of this study was to evaluate and validate the accuracy and performance characteristics of administrative codes in diagnosing autoinflammatory syndromes (AISs). Methods We identified potential AIS patients from the electronic medical records at the University of Iowa Hospital and Clinics and the Stead Family Children's Hospital using a screening filter based on the 10th edition of the International Classification of Diseases (ICD-10) codes and interleukin-1 antagonists. Diagnostic criteria for adult-onset Still disease, systemic juvenile idiopathic arthritis, Behçet disease (BD), familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and chronic nonbacterial osteomyelitis (SAPHO-CNO) were reviewed for each patient. Patients who did not meet the diagnostic criteria were categorized as non-AIS. In this cross-sectional study, we calculated the sensitivity, specificity, positive predictive value, negative predictive value, and area under the receiver operating characteristic curve for the ICD codes in diagnosing AIS. Results Out of the 502 patients with potential AIS, 338 patients (67%) had a true AIS diagnosis. Sensitivity ranged from 80% (SAPHO-CNO) to 100% (BD and FMF), and positive predictive value ranged from 15% (FMF) to 80% (SAPHO-CNO). Specificity ranged from 81% (FMF) to 99% (CAPS and SAPHO-CNO), whereas negative predictive value ranged from 98% (adult-onset Still disease) to 100% (systemic juvenile idiopathic arthritis, BD, FMF, and CAPS). All ICD codes or code combinations for the diagnosis of specific AIS subtypes showed high accuracy with areas under the receiver operating characteristic curve ≥0.89. Conclusions This study validated the accuracy of administrative codes for diagnosing AIS, supporting their use in constructing AIS cohorts for clinical outcomes research.

Clinical course of pediatric-onset Behçet's Disease in young adulthood.

Although Behçet's disease (BD) typically manifests in the second or third decade of life, initial symptoms may appear at a younger age. It may also take a longer time for the full disease phenotype to develop after the first symptom onset in pediatric patients. In this study we aimed to assess the clinical course of pediatric-onset BD in adulthood period. The files of 112 patients diagnosed with BD before the age of 18, selected from five tertiary clinics, were retrospectively examined. Patients with a follow-up of less than six months were excluded. The study comprised 93 patients with pediatric-onset BD, of whom 64.5% (n = 60) were male. The median age of diagnosis was 15 years (13-17). Major organ involvement was present in 49 (52.5%) patients. The most commonly affected organ was the eye (29%).Sixty-eight (73.1%) patients had follow-up data in adulthood. Fourty patients had only mucocutaneous manifestations in the pediatric period. During follow-up in adulthood, 15 (53.3% were male) had new major organ involvement with a mean of 10.1 (SD: 7.9) years after diagnosis. Twenty-eight patients (41.1%) experienced major organ involvement during the pediatric period. In adulthood follow-up, 12 (42.8%) developed new major organ involvement and/or relapse of the same organ. Eighteen (26.5%) of 68 pediatric-onset BD patients had new major organ involvement, and 9 (13.2%) had a relapse during adulthood follow-up. Our results showed that nearly one-third of pediatric BD patients have a new major organ involvement or a relapse in adulthood. Regular follow-up of pediatric BD patients in adulthood is essential to prevent long-term damage in this disease subset.

Uveitis as a Predictor for Myocardial Infarction in Patients with Behçet's Disease, Multi Centric Cohort Study from Aseer Region, Saudi Arabia

Introduction: Behçet's disease (BD) is a chronic, multisystem inflammatory disorder characterized by a diverse array of clinical manifestations, including recurrent oral and genital ulcers, ocular inflammation, skin lesions, and joint problems. Its presentation varies, requiring tailored diagnosis and management. Methods: Retrospective cohort study, though review of medical charts and data for patients who meet the diagnostic criteria for BD actively receiving care in rheumatology clinics. The primary objective was to investigate the correlation between uveitis and Myocardial infarction in this population. Results: This study included a total of 111 patients. Approximately 33% of the patients exhibited positive CRP levels. Approximately 10% (11 patients) of the subjects were diagnosed with uveitis. Notably, the majority of individuals with uveitis were males. Three patients with uveitis had arthritis, none of whom presented with gastrointestinal tract (GIT) Complaints. Remarkably, none of the patients diagnosed with uveitis had a documented history of myocardial infarction (MI). Conclusion: In our cohort, those with a history of MI did not have a documented history of uveitis. Larger, prospective studies are needed to evaluate the associations between MI and uveitis in a BD population.

Lung transplantation for alpha-1-antitrypsin deficiency and Behçet's disease: A case report

INTRODUCTIONAlpha-1-Antitrypsin deficiency (A1ATD) is a genetically determined anti-proteinase deficiency which predisposes to early onset emphysema and liver disease. Lung transplantation (LTx) is the final therapeutic option. Behçet's disease (BD) is a rare autoimmune disease characterized by oral and genital ulcers, deep venous thrombosis associated with large and small-vessel vasculitis and aneurysms. The association of A1ATD and BD is unknown. We aim to describe a rare case of concomitant presentation of these pathologies in a patient submitted to LTx. CASE REPORTA 31-years-old female presented with oral and genital ulcers associated with cavernous sinus thrombosis in 2012, being diagnosed with BD. In 2018 debuted with progressive respiratory symptoms. CT-scan revealed extensive pulmonary emphysema associated with decreased pulmonary function. A1ATD was identified with a heterozygous “MZ” allelic combination. She was referred to LTx center in 2021. After additional investigation, the patient was considered to LTx. She underwent standard bilateral LTx in July of 2022. During post-operative time, special attention was given to the risk of vascular complications, and pos-operative angio-CT was performed actively searching for this possible outcome. Nevertheless, no major events occurred. After 1 year, she is fully functional with no signs of BD activity. CONCLUSIONTo our knowledge, this is the first description of LTx for A1ATD in a patient with BD, proving its feasibility in a highly selected patient at an experienced and specialized center. Nonetheless, solid organ transplantation in patients with BD remains a high risk procedure and should be indicated with caution.

Influence of gender on Behçet's Disease phenotype and irreversible organ damage: Data from the International AIDA Network Behçet's Disease Registry.

Objectives Gender impact on phenotypical expression of Behçet's disease (BD) has been specifically investigated only in a few large-scale studies. The main goal of the study was to examine gender differences in a large cohort of patients affected by BD. Methods Data were retrieved from the International AIDA Network Registry for BD. We assessed differences between males and females in terms of Behçet's syndrome Overall Damage Index (BODI), differences in the disease manifestations at onset and in the cumulative manifestations throughout disease course, as well as differences in the cardiovascular risk. Finally, predictive factors leading to major organ involvement were investigated. Results In total, 1024 BD patients (567 males, 457 females) were enrolled in the study, with a male-to-female ratio of 1.24/1. Males displayed a significantly higher mean ± SD BODI (1.92 ± 2.09) at the last follow-up, compared to female patients (1.25 ± 1.87) (p<0.0001). Uveitis (p<0.0001) and vascular involvement (p=0.0076) were significantly more frequent among males whereas female patients were significantly overrepresented in arthralgia (p<0.0001), arthritis (p=0.00025), isolated headache (p<0.0001), central nervous system (CNS) involvement (p=0.040), and gastrointestinal involvement (p=0.00046). Regarding cardiovascular risk, no differences between the two groups emerged (p=0.617). Four variables were associated with the development of major organ involvement: male gender (OR=2.104, p=0.001), current treatment with biologic agents (OR=2.257, p=0.0003), origin from endemic countries (OR=2.661, p=0.0009), and disease duration (OR=1.002, p=0.024). Conclusion BD displays a more severe course among males. This subgroup develops more irreversible damage and presents more frequently ocular and vascular involvement during disease course. On the other hand, female patients are prone to experience articular involvement, headache, CNS and gastrointestinal involvement. These data suggest the existence of a gender-driven disease expression.

Clinical characteristics of Behçet's disease in an Egyptian cohort

Background: Behçet's disease is a multi-systemic vasculitis characterized by recurring lesions in the skin mucosa and sight-threatening uveitis. The aim of the research: This research aimed to investigate the clinical features of Behçet's disease (BD) in individuals residing in Egypt. Patients and methods: 150 individuals with BD were recruited, examined, and put through a skin allergy test, musculoskeletal assessment, laboratory tests, and comprehensive medical history. The disease's activity was evaluated using the Behçet Disease Current Activity Form (BDCAF). Complete ophthalmological evaluation and imaging studies were performed according to the organ involved. Results: Cases were 116 men (77.3%) and 34 women (22.7%) (M: F 3.4:1). Their mean age was 34.05 ± 9.28 years, and the disease duration range was 1–20 years. Oral and genital aphthosis were reported in 86.7% and 82.0%, respectively. Eye affection was observed in 68.7%, mainly in anterior uveitis (35.3%). Vascular manifestations occurred in 58.7%, affecting primarily the venous side (31.3%) in the form of deep venous thrombosis (DVT). 32.7% had joint affection, and the pathergy test was positive for 28%. Central nervous system (CNS), skin lesions, and gastrointestinal tract involvement were the slightest common manifestations observed in 26%, 25.3%, and 7.3%, respectively. Articular manifestations were markedly linked to skin lesions (P = 0.002), and there was a significant predominance of articular manifestations among females (P = 0.042). BDCAF score was significantly related to male gender (P = 0.037), smokers (P &lt; 0.000), those with older age (P = 0.028), delayed age of disease onset (P = 0.025), and additionally those with the eye (P = 0.000) and CNS involvement (P = 0.003). Conclusion: The clinical features of Egyptian Behçet's patients were significantly dominated by mucocutaneous involvement, followed by ocular affection. Articular manifestations were markedly more significant in women. The disease activity score was more remarkable in males, smokers, those of older age, and those with a delayed age of disease onset.

Characterization of potential salivary biomarkers in Behçet's disease.

Diagnosis of Behçet's disease (BD) has always been a challenging task due to the heterogeneity and complexity of the disease, while available literature on BD biomarkers is largely inconclusive. The aim of this study was to evaluate the expression of a panel of salivary biomarkers in a group of BD patients and test their association with BD. The study population comprised 23 BD patients (12 females and 11 males) and a control group of age and gender-matched healthy individuals. Enzyme-linked immunosorbent assays were used to test the expression of 5 salivary proteins including: interleukin 1β (IL-1β), epidermal growth factor (EGF), endothelin, vascular endothelial growth factor (VEGF) and transferrin (TrF). BD activity was evaluated using the International Society for Behçet's disease (ISBD) current activity form. Salivary endothelin, EGF and VEGF were significantly higher in the BD group compared to controls (16.3 Vs. 7.7pg/ml, 194.5 Vs. 163.4pg/ml and 262 Vs. 150.2pg/ml, p<0.05, respectively) whereas TrF was significantly lower in BD group compared to controls (497 Vs. 2206pg/ml, p<0.001). The correlation analysis showed a trend of a positive linear association between endothelin expression and both BD activity (r=0.41) and BD activity index score (r=0.4), without reaching statistical significance (p>0.05). In conclusion, salivary TrF, endothelin, VEGF and EGF may represent potential biomarkers for BD. These findings require further validation in larger samples and different activity states of BD.

Postoperative complications in patients with Behçet's disease.

To assess the proportion of postoperative complications of various surgeries in patients with Behçet's disease (BD) and compare the risk of surgical complications between BD patients and controls. We analyzed 389 BD patients who underwent surgeries at Seoul National University Hospital between January 2003 and December 2019. Controls were 1:1 matched with BD patients based on age at surgery, sex, and type of surgery. Generalized estimating equation analyses were performed to compare the postoperative complications between BD patients and controls. Among 632 surgeries in BD patients, 36 (5.7%) surgical site complications, including wound dehiscence (38.9%), bleeding (13.9%), infections (8.3%), anastomotic dehiscence (22.2%), and stricture (5.6%), occurred after median 12days (IQR 7.8-22.0). Surgical site complications developed frequently after cardiac (33.3%) and vascular (22.2%) surgeries and rarely occurred after ophthalmic (1.5%) surgeries. Seventeen (2.7%) cases required reoperation within median 15days (IQR 7.0-43.0). Four patients died within median 21days (IQR 8.3-41.3days). After adjustment for confounders, BD patients exhibited a significantly higher risk of surgical site complications (OR 3.4, 95% CI 1.4-8.0) and reoperation (OR 5.2, 95% CI 1.2-22.8) after cardiovascular surgery than controls. However, the risks of surgical site complications and reoperation after other types of surgery were similar in both groups. The risk of postoperative complications varies according to the type of surgeries among BD patients. While cardiovascular surgeries result in higher perioperative complications in BD patients, ophthalmic surgeries show a safety profile similar to those in non-BD patients. Key Points • Patients with Behçet's disease exhibited an increased risk of surgical site complications and subsequent reoperations after cardiovascular surgery than controls. • The safety profile of ophthalmic surgery was comparable between patients with Behçet's disease and controls. • In patients with Behçet's disease, the risk of postoperative complications varies according to the type of surgery.

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.

Infliximab in the Treatment of a Giant Coronary Aneurysm in Behçet's Disease.

Behçet's disease (BD) is a rare, multisystemic inflammatory disorder with a diverse range of manifestations, primarily affecting the mucocutaneous and ocular systems. While vascular involvement is less frequent it can be severe, with coronary aneurysms being particularly rare. We report a 28-year-old male with BD who developed a giant anterior interventricular artery aneurysm. The patient, initially managed with azathioprine and colchicine, presented with acute chest pain and elevated cardiac markers. Imaging revealed a coronary aneurysm measuring 5.03 cm. Due to the inflammatory nature of the aneurysm surgical intervention was initially deferred, and the patient was treated with corticosteroids and cyclophosphamide. Despite these treatments, recurrent symptoms prompted the initiation of infliximab therapy. Administered at 5 mg/kg at weeks 0, 2 and 6, with subsequent doses every two months, infliximab led to a significant reduction in aneurysm size to 3.73 cm. This case highlights the effectiveness of infliximab in managing giant coronary aneurysms associated with BD, demonstrating its potential as a treatment option for severe vascular complications in BD. The substantial reduction in aneurysm size following infliximab therapy underscores the need for further investigation into its role in treating such rare but serious manifestations of BD. A giant coronary aneurysm is a rare complication of Behçet's disease.Infliximab effectively reduced the aneurysm size.Imaging shows a substantial decrease in aneurysm size after infliximab treatment.

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.