Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricul...Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricular arrythmias are believed to be the cause of this increased risk of cardiovascular mortality and it is also thought to be related with cardiac autonomic dysfunction. Heart rate turbulence (HRT) is a new predictor of cardiac autonomic activity. HRT is an independent and powerful predictor of mortality. In this study, we investigated the cardiac autonomic activity which can be determined by HRT in patients with BD. Forty patients with BD (20 men, mean age: 40 ± 9 years, range: 27-55 years) were diagnosed according to the International Study Group Criteria (ISGC) and gender and age matched healthy volunteers (20 men, mean age: 39 ± 8 years, range: 26-56 years) were included in this study. All of the participants (patients and controls) underwent 24 hours Holter electrocardiogram. HRT parameters, turbulence onset (TO) and turbulence slope (TS) were calculated with HRT (View Version 0.60-0.1 of Software Program). There were no significant differences in TO and TS values between patients with BD and control subject (TO-BD: 0.014 ± 0.03, TO-Control: 0.011 ± 0.04;TS-BD: 7.88 ± 4.9, TS-Control: 9.42 ± 6.7 respectively). Although increased cardiovascular mortality rates in BD have been shown in many studies, HRT values—detecting the risk of sudden death—do not seem to be altered in this disease.展开更多
BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarel...BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarely been reported.CASE SUMMARY Herein,we describe a patient with intestinal BD who presented with decreased oxygen saturation and shortness of breath during a diagnostic colonoscopy.Bilateral pneumothorax,pneumomediastinum,pneumoperitoneum,pneumoretroperitoneum and subcutaneous emphysema of the neck,chest,abdomen,back and scrotum were confirmed by computed tomography scan.The sudden change in condition was considered to be associated with iatrogenic bowel perforation.After receiving closed thoracic drainage and conservative therapy,the patient was discharged in stable condition.CONCLUSION Endoscopists should be aware of the risks of colonoscopy in patients with intestinal BD and the possibility of pneumothorax associated with intestinal perforation and make adequate preparations before colonoscopy.展开更多
Background: Behcet’s disease is a vasculitis affecting several organs. A renal involvement is rarely described. It is most of the time about a renal amyloidosis. Patients and methods: It is a descriptive retrospectiv...Background: Behcet’s disease is a vasculitis affecting several organs. A renal involvement is rarely described. It is most of the time about a renal amyloidosis. Patients and methods: It is a descriptive retrospective study concerning the patients followed in our department for a Behcet’s disease having presented a renal amyloidosis. Results: It is about 4 men with average age of 38.25 years old. The renal involvement was revealed after an average delay of 5.7 years by a nephrotic syndrome in all cases. A renal insufficiency was noted in 3 cases with an average serum creatinine of 587 μmol/l (127 - 1490). The type of the amyloidosis was AA in 2 cases. The treatment contained colchicines in every case. The evolution was marked by the worsening of the renal function leading to end stage renal disease in 3 cases. Death occurred in 1 case and one patient lost to follow up. Discussion: Renal amyloidosis can complicate the evolution of a Behcet’s disease. It occurs generally 1 to 10 years after the beginning of the disease. Once installed, it evolves generally towards the chronic renal insufficiency and can condition the forecast of this affection. Conclusion: Amyloidosis is a rare complication of the Behcet’s disease. Its screening is so desirable to improve the renal prognosis of these patients.展开更多
Although cardiac involvement during Behcet syndrome is uncommon and intracardiac thrombosis is exceptional, it concerns the right ventricle much more than the left one. We report an illustrative case of left intracard...Although cardiac involvement during Behcet syndrome is uncommon and intracardiac thrombosis is exceptional, it concerns the right ventricle much more than the left one. We report an illustrative case of left intracardiac thrombosis revealing Behcet’s disease in a 33-year-old Tunisian man.展开更多
Introduction: The Behcet’s disease is deemed to be scarce in Black Africa where data are still scattered. The purpose of our study is to describe the epidemiological, clinical, paraclinic and evolutive particularitie...Introduction: The Behcet’s disease is deemed to be scarce in Black Africa where data are still scattered. The purpose of our study is to describe the epidemiological, clinical, paraclinic and evolutive particularities of the patients whose presenting symptoms of the Behcet’s diseases were a venous thrombosis. Patients and Methods: It was a descriptive, multicenter, and cross-sectional study lasting 15 months. We brought together all the cases of the Behcet’s disease revealed by venous thrombosis. The diagnosis was based on clinical criteria of the international group of study of the Behcet’s disease in 2007. Results: We have grouped 10 revealing thrombosis cases of the Behcet’s diseases during our study period. The average age was 34. The average wait period between the appearances of the early symptoms and the diagnosis of the very disease was 30 months. The admission motives were the abdominal pain (2 cases), a thrombophlebitis of the lower limb (2 cases), headaches (1 case), coma (1 case), a thrombophlebitis of the upper limb (3 cases). The thrombotic symptoms were exclusively venous-located. The seats of the thrombosis were the vena cava superior in 30% of the cases, the vena cava inferior in 20% of the cases, the veins of the lower limb in 20% of the cases, the cerebral vein in 20% of the cases, and the auxiliary vein in 10% of the cases. The treatment of the deep venous thrombosis consisted in all the cases of an effective anticoagulation associated with the colchicine. Primarily, the corticotherapy with a high dose was used in all the patients. One of them in the comatose stage, manifesting both cerebral thrombophlebitis and aseptic meningitis, had died. Conclusion: Behcet’s disease is a disease of the young adult, but it must be evoked even in old age, with a view to appropriate management, in order to avoid the complications of the disease. Although it is rare in our regions, it should be sought in the etiological assessment of venous thrombosis whatever the location.展开更多
Background: Behçet’s Syndrome (BS) is characterized by a heterogeneous vessel involvement, a fluctuating natural history and by the absence of biological markers correlated to disease activity that’s why ob...Background: Behçet’s Syndrome (BS) is characterized by a heterogeneous vessel involvement, a fluctuating natural history and by the absence of biological markers correlated to disease activity that’s why objective clinical scores are needed for the assessment of its activity. The Behçet’s Disease Clinical Activity Form (BDCAF) is the most recent and widely used clinical activity score. Objectives: To perform a cross-cultural adaptation of the Behçet’s Disease Current Activity Form (BDCAF) to the Tunisian Dialect (Arabic Language) and to evaluate the metrological characteristics of the Tunisian version (Tu-BDCAF) especially its reliability in BD activity evaluation. Methods: Cross-cultural adaptation was done according to the established guidelines. Reliability of Tu-BDCAF was tested among 40 BD patients (mean age: 38 years, sex ratio: 1.37). Patients were questioned by two BD specialists at 20 minutes interval to evaluate inter-observer reproducibility and twice by the same physician at 48 hours interval to assess the intra-observer reproducibility. k Coefficient was used to test the concordance between qualitative variables and correlation between quantitative variables was evaluated used Pearson coefficient and Bland and Altman graphical method. Results: There was a good correlation between global scores calculated by the two physicians on the same day (r = 0.94, p < 0.0001) and also between the scores calculated by the same clinician at different times (r = 0.98, p k Coefficient analyses demonstrated a good intra and inter observer reliability for all the Tu-BDCAF items excepted for diarrhea and Clinician’s impression. As the original version, Tu-BDCAF is an objective, easy-calculated and reliable index for assessing disease activity in BD. The main limit of the BDCAF score remains the absence of a cut-off point defining BD activity. Conclusion: Tu-BDACF is a Tunisian version of the BDCAF score which can be used in routine to assess BD activity but also in international studies and clinical trials.展开更多
Behcet’s disease (BD) is a large vessel vasculitis with a wide range of clinical manifestations. Some of these manifestation may be life threatening and rapid suppression of the inflammation with effective immunosupp...Behcet’s disease (BD) is a large vessel vasculitis with a wide range of clinical manifestations. Some of these manifestation may be life threatening and rapid suppression of the inflammation with effective immunosuppressive agent is crucial. There are traditional drugs with different response rates and all have efficacy on different manifestations of the disease. The most frightening manifestations of the disease are ocular, neurologic, intestinal and vascular types of involvement. Besides benign and easily treated manifestations there are also refractory cases with complicated involvement. The novel biologic agents have been used for these resistant patients and favorable response rates have been reported. In this review, we have shared our experience with biologic agents in BD and also reviewed the literature for the efficacy and safety for these novel agents for refractory patients.展开更多
BACKGROUND Behcet's disease(BD)is an inflammatory disorder known for various symptoms,including oral and genital ulcers and ocular inflammation.Panuveitis,a severe eye condition,is rare as the first sign of BD.CAS...BACKGROUND Behcet's disease(BD)is an inflammatory disorder known for various symptoms,including oral and genital ulcers and ocular inflammation.Panuveitis,a severe eye condition,is rare as the first sign of BD.CASE SUMMARY We present an unusual case of a 30-year-old man who developed panuveitis after receiving the mRNA-based coronavirus disease 2019(COVID-19)vaccine(Moderna).Laboratory tests ruled out infections,but he had a positive HLA-B51 result and a history of genital ulcer and oral ulcers,leading to a BD diagnosis.Treatment with corticosteroids improved his condition.Interestingly,he had another episode of panuveitis after the second mRNA vaccine dose,which also responded to corticosteroids.CONCLUSION This case highlights the rare onset of BD following mRNA COVID-19 vaccination,suggesting a potential link between these vaccines and BD's eye symptoms,emphasizing the importance of quick treatment in similar cases.展开更多
Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in ...Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.展开更多
AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the ca...AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the candidate gene region(rs11792633,rs7025417,rs10975519 and rs1048274 in IL33;rs2310220,rs12712142,rs13424006 and rs3821204 in IL1RL1)were genotyped in783 BD patients and 701 healthy controls by the Sequenom Mass Array i PLEX platform.RESULTS:A statistically significant association was observed between IL1RL1 rs12712142 and BD patients.The frequency of IL1RL1 rs12712142 variant allele A was significantly lower in BD patients than that in controls(OR=0.8,95%CI:0.69-0.94,Pc=0.039);the genotype distribution(Pc=0.043)and additive and dominant genetic model analyses(OR=0.8,95%CI:0.69-0.94,Pc=0.040 and OR=0.72,95%CI:0.58-0.88,Pc=0.011)also indicated a strong association between rs12712142 and BD patients.CONCLUSION:This is the first study to reveal the association between IL1RL1 rs12712142 variant allele A and the decreased risk of BD in the Chinese Han population,indicating a protective role of IL1RL1 in the pathogenesis of BD.展开更多
Behcet’s disease is defined as a multisystemic inflammatory disease.Although the precise pathogenesis and etiology is still a mystery,accumulating evidence shows that genetic variants of immune-related genes have a p...Behcet’s disease is defined as a multisystemic inflammatory disease.Although the precise pathogenesis and etiology is still a mystery,accumulating evidence shows that genetic variants of immune-related genes have a profound influence on the development of Behcet’s disease.To explore the genetic factors for Behcet’s disease,our group investigated the association of Behcet’s disease with multiple immune response genes and has identified multiple Behcet’s disease-related immunoregulatory pathways in the Chinese Han population.A large number of gene polymorphisms were studied including STAT4,IL23R,CD40,CCR1/CCR3,STAT3,OPN,IL17,JAK2,MCP-1,CTLA4,PD-1,PD-L1,PD-L2,TGRBR3,CCR6,PTPN22,FCRL3,IRF5,SUMO4 and UBAC2.Significant associations were found between Behcet’s disease and STAT4,IL23R,CD40,CCR1/CCR3,STAT3,MCP-1,TGFBR3,FCRL3,SUMO4,UBAC2.These genetic predisposition studies support an important role for both lymphocyte differentiation as well as ubiquitination pathways.These findings are helpful in elucidating the pathogenesis of Behcet’s disease and hopefully will allow the development of novel treatment regimes.展开更多
Behcet's disease is a vasculitis disease involving multiple systems,and its pathogenesis is still unclear.The paper discusses the research progress on the treatment of Behcet's disease in traditional Chinese m...Behcet's disease is a vasculitis disease involving multiple systems,and its pathogenesis is still unclear.The paper discusses the research progress on the treatment of Behcet's disease in traditional Chinese medicine and integrated traditional Chinese and Western medicine.The clinical efficacy of integrated traditional Chinese and western medicine in the treatment of Behcet's disease is significantly better than that of Western medicine alone,which is worthy of further research.展开更多
Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The ...Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.展开更多
Behet's disease(BD) is a chronic inflammatory condition with multisystem involvement. Approximately 10%-15% of patients present with gastrointestinal involvement. Involved sites and the endoscopic view usually r...Behet's disease(BD) is a chronic inflammatory condition with multisystem involvement. Approximately 10%-15% of patients present with gastrointestinal involvement. Involved sites and the endoscopic view usually resemble Crohn's disease(CD). In addition to intestinal involvement, oral mucosa, the eyes, skin, and joints are commonly affected. No pathognomonic laboratory test is available for the diagnosis of either disease. Management approaches are also similar in various aspects. Differentiating BD from CD is highly challenging. In this article, the similarities and differences between BD and CD in terms of epidemiology, etiopathogenesis, clinical and imaging findings, and histopathological and therapeutic approaches are reviewed.展开更多
AIM:To investigate effects of anti-TNF biologic drugs on uveitis severity(comparing visual acuity log MAR levels) in Behcet patients.METHODS:Three databases Pub Med, Scopus, and the Web of Science were searched for qu...AIM:To investigate effects of anti-TNF biologic drugs on uveitis severity(comparing visual acuity log MAR levels) in Behcet patients.METHODS:Three databases Pub Med, Scopus, and the Web of Science were searched for qualified papers focusing on the anti-TNF-α factors treatment in Behcet’s disease(BD)-associated uveitis. Studies that were designed pre and post anti-TNF drug treatment, were selected. After determining the search strategy for this study, the relevant data were extracted. RESULTS:The initial search was performed in the target databases and a total of about 1458 articles were found. Fifteen articles were selected for systematic review and only 12 of them had inclusion criteria for Meta-analysis(with visual acuity data). The mean dose of prednisolone before and after biological treatments was reported in 5 studies(28.56 and 7.56 mg/kg, respectively). Also, the preliminary results indicate a significant reduction in visual acuity log MAR levels(MD=-1.5 IU/L, 95%CI:-2.1,-0.01).CONCLUSION:Biological drugs significantly reduce the dose of prednisolone and affect visual acuity values.展开更多
Objective: To elucidate the outcomes of open surgery for popliteal artery aneurysm (PAA) in a single Japanese institution. Materials and Methods: Between 1999 and 2016, 35 patients (44 PAAs) underwent open surgery in ...Objective: To elucidate the outcomes of open surgery for popliteal artery aneurysm (PAA) in a single Japanese institution. Materials and Methods: Between 1999 and 2016, 35 patients (44 PAAs) underwent open surgery in our department. The first-line surgical strategy involved the medial approach, with excision of the aneurysm sac and autologous vein bypass grafting. Associations between the outcomes and 1) the surgical management of the aneurysmal sac (aneurysmectomy vs. ligation with bypass) and 2) the preoperative clinical symptoms were evaluated. Results: There were no aneurysm-related deaths during the follow-up period (mean, 4 years;range, 1 - 17.6 years). The 5-year primary, primary-assisted, and secondary patency rates were 67.9%, 72.7%, and 94.5%, respectively. Re-intervention was required for 19 PAAs (14 patients), including two PAAs in patients with suspected Behçet’s disease. Patency rates did not differ between the aneurysmectomy and ligation groups. Remnant sac enlargement occurred in 3 cases. Among asymptomatic patients, clinical symptoms did not deteriorate;however, the ankle brachial pressure index decreased ≥0.15 in patients with artery-graft mismatching. Conclusion: Patency rates were good, and our modified ligation with bypass procedure, which involves excising the sac as much as possible, exhibited less risk in terms of remnant sac enlargement compared to that in previous studies.展开更多
Introduction Behcet＇s disease（BD） is an multi-system inflammatory vasculitis based on small and large vessels of the venous and arterial system.About 80%of BD patients have ocular lesions characterized by recurrent...Introduction Behcet＇s disease（BD） is an multi-system inflammatory vasculitis based on small and large vessels of the venous and arterial system.About 80%of BD patients have ocular lesions characterized by recurrent iridocyclitis,retinal vasculitis,and retinal ischemia.Frequent recurrence of these lesions,leading to macular edema and optic atrophy,can eventually result in severe loss of vision.Western medicine treatment focuses on steroid and immunosuppressive agents;展开更多
文摘Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricular arrythmias are believed to be the cause of this increased risk of cardiovascular mortality and it is also thought to be related with cardiac autonomic dysfunction. Heart rate turbulence (HRT) is a new predictor of cardiac autonomic activity. HRT is an independent and powerful predictor of mortality. In this study, we investigated the cardiac autonomic activity which can be determined by HRT in patients with BD. Forty patients with BD (20 men, mean age: 40 ± 9 years, range: 27-55 years) were diagnosed according to the International Study Group Criteria (ISGC) and gender and age matched healthy volunteers (20 men, mean age: 39 ± 8 years, range: 26-56 years) were included in this study. All of the participants (patients and controls) underwent 24 hours Holter electrocardiogram. HRT parameters, turbulence onset (TO) and turbulence slope (TS) were calculated with HRT (View Version 0.60-0.1 of Software Program). There were no significant differences in TO and TS values between patients with BD and control subject (TO-BD: 0.014 ± 0.03, TO-Control: 0.011 ± 0.04;TS-BD: 7.88 ± 4.9, TS-Control: 9.42 ± 6.7 respectively). Although increased cardiovascular mortality rates in BD have been shown in many studies, HRT values—detecting the risk of sudden death—do not seem to be altered in this disease.
文摘BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarely been reported.CASE SUMMARY Herein,we describe a patient with intestinal BD who presented with decreased oxygen saturation and shortness of breath during a diagnostic colonoscopy.Bilateral pneumothorax,pneumomediastinum,pneumoperitoneum,pneumoretroperitoneum and subcutaneous emphysema of the neck,chest,abdomen,back and scrotum were confirmed by computed tomography scan.The sudden change in condition was considered to be associated with iatrogenic bowel perforation.After receiving closed thoracic drainage and conservative therapy,the patient was discharged in stable condition.CONCLUSION Endoscopists should be aware of the risks of colonoscopy in patients with intestinal BD and the possibility of pneumothorax associated with intestinal perforation and make adequate preparations before colonoscopy.
文摘Background: Behcet’s disease is a vasculitis affecting several organs. A renal involvement is rarely described. It is most of the time about a renal amyloidosis. Patients and methods: It is a descriptive retrospective study concerning the patients followed in our department for a Behcet’s disease having presented a renal amyloidosis. Results: It is about 4 men with average age of 38.25 years old. The renal involvement was revealed after an average delay of 5.7 years by a nephrotic syndrome in all cases. A renal insufficiency was noted in 3 cases with an average serum creatinine of 587 μmol/l (127 - 1490). The type of the amyloidosis was AA in 2 cases. The treatment contained colchicines in every case. The evolution was marked by the worsening of the renal function leading to end stage renal disease in 3 cases. Death occurred in 1 case and one patient lost to follow up. Discussion: Renal amyloidosis can complicate the evolution of a Behcet’s disease. It occurs generally 1 to 10 years after the beginning of the disease. Once installed, it evolves generally towards the chronic renal insufficiency and can condition the forecast of this affection. Conclusion: Amyloidosis is a rare complication of the Behcet’s disease. Its screening is so desirable to improve the renal prognosis of these patients.
文摘Although cardiac involvement during Behcet syndrome is uncommon and intracardiac thrombosis is exceptional, it concerns the right ventricle much more than the left one. We report an illustrative case of left intracardiac thrombosis revealing Behcet’s disease in a 33-year-old Tunisian man.
文摘Introduction: The Behcet’s disease is deemed to be scarce in Black Africa where data are still scattered. The purpose of our study is to describe the epidemiological, clinical, paraclinic and evolutive particularities of the patients whose presenting symptoms of the Behcet’s diseases were a venous thrombosis. Patients and Methods: It was a descriptive, multicenter, and cross-sectional study lasting 15 months. We brought together all the cases of the Behcet’s disease revealed by venous thrombosis. The diagnosis was based on clinical criteria of the international group of study of the Behcet’s disease in 2007. Results: We have grouped 10 revealing thrombosis cases of the Behcet’s diseases during our study period. The average age was 34. The average wait period between the appearances of the early symptoms and the diagnosis of the very disease was 30 months. The admission motives were the abdominal pain (2 cases), a thrombophlebitis of the lower limb (2 cases), headaches (1 case), coma (1 case), a thrombophlebitis of the upper limb (3 cases). The thrombotic symptoms were exclusively venous-located. The seats of the thrombosis were the vena cava superior in 30% of the cases, the vena cava inferior in 20% of the cases, the veins of the lower limb in 20% of the cases, the cerebral vein in 20% of the cases, and the auxiliary vein in 10% of the cases. The treatment of the deep venous thrombosis consisted in all the cases of an effective anticoagulation associated with the colchicine. Primarily, the corticotherapy with a high dose was used in all the patients. One of them in the comatose stage, manifesting both cerebral thrombophlebitis and aseptic meningitis, had died. Conclusion: Behcet’s disease is a disease of the young adult, but it must be evoked even in old age, with a view to appropriate management, in order to avoid the complications of the disease. Although it is rare in our regions, it should be sought in the etiological assessment of venous thrombosis whatever the location.
文摘Background: Behçet’s Syndrome (BS) is characterized by a heterogeneous vessel involvement, a fluctuating natural history and by the absence of biological markers correlated to disease activity that’s why objective clinical scores are needed for the assessment of its activity. The Behçet’s Disease Clinical Activity Form (BDCAF) is the most recent and widely used clinical activity score. Objectives: To perform a cross-cultural adaptation of the Behçet’s Disease Current Activity Form (BDCAF) to the Tunisian Dialect (Arabic Language) and to evaluate the metrological characteristics of the Tunisian version (Tu-BDCAF) especially its reliability in BD activity evaluation. Methods: Cross-cultural adaptation was done according to the established guidelines. Reliability of Tu-BDCAF was tested among 40 BD patients (mean age: 38 years, sex ratio: 1.37). Patients were questioned by two BD specialists at 20 minutes interval to evaluate inter-observer reproducibility and twice by the same physician at 48 hours interval to assess the intra-observer reproducibility. k Coefficient was used to test the concordance between qualitative variables and correlation between quantitative variables was evaluated used Pearson coefficient and Bland and Altman graphical method. Results: There was a good correlation between global scores calculated by the two physicians on the same day (r = 0.94, p < 0.0001) and also between the scores calculated by the same clinician at different times (r = 0.98, p k Coefficient analyses demonstrated a good intra and inter observer reliability for all the Tu-BDCAF items excepted for diarrhea and Clinician’s impression. As the original version, Tu-BDCAF is an objective, easy-calculated and reliable index for assessing disease activity in BD. The main limit of the BDCAF score remains the absence of a cut-off point defining BD activity. Conclusion: Tu-BDACF is a Tunisian version of the BDCAF score which can be used in routine to assess BD activity but also in international studies and clinical trials.
文摘Behcet’s disease (BD) is a large vessel vasculitis with a wide range of clinical manifestations. Some of these manifestation may be life threatening and rapid suppression of the inflammation with effective immunosuppressive agent is crucial. There are traditional drugs with different response rates and all have efficacy on different manifestations of the disease. The most frightening manifestations of the disease are ocular, neurologic, intestinal and vascular types of involvement. Besides benign and easily treated manifestations there are also refractory cases with complicated involvement. The novel biologic agents have been used for these resistant patients and favorable response rates have been reported. In this review, we have shared our experience with biologic agents in BD and also reviewed the literature for the efficacy and safety for these novel agents for refractory patients.
文摘BACKGROUND Behcet's disease(BD)is an inflammatory disorder known for various symptoms,including oral and genital ulcers and ocular inflammation.Panuveitis,a severe eye condition,is rare as the first sign of BD.CASE SUMMARY We present an unusual case of a 30-year-old man who developed panuveitis after receiving the mRNA-based coronavirus disease 2019(COVID-19)vaccine(Moderna).Laboratory tests ruled out infections,but he had a positive HLA-B51 result and a history of genital ulcer and oral ulcers,leading to a BD diagnosis.Treatment with corticosteroids improved his condition.Interestingly,he had another episode of panuveitis after the second mRNA vaccine dose,which also responded to corticosteroids.CONCLUSION This case highlights the rare onset of BD following mRNA COVID-19 vaccination,suggesting a potential link between these vaccines and BD's eye symptoms,emphasizing the importance of quick treatment in similar cases.
文摘Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.
基金the National Natural Science Foundation of China (No.81770917)。
文摘AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the candidate gene region(rs11792633,rs7025417,rs10975519 and rs1048274 in IL33;rs2310220,rs12712142,rs13424006 and rs3821204 in IL1RL1)were genotyped in783 BD patients and 701 healthy controls by the Sequenom Mass Array i PLEX platform.RESULTS:A statistically significant association was observed between IL1RL1 rs12712142 and BD patients.The frequency of IL1RL1 rs12712142 variant allele A was significantly lower in BD patients than that in controls(OR=0.8,95%CI:0.69-0.94,Pc=0.039);the genotype distribution(Pc=0.043)and additive and dominant genetic model analyses(OR=0.8,95%CI:0.69-0.94,Pc=0.040 and OR=0.72,95%CI:0.58-0.88,Pc=0.011)also indicated a strong association between rs12712142 and BD patients.CONCLUSION:This is the first study to reveal the association between IL1RL1 rs12712142 variant allele A and the decreased risk of BD in the Chinese Han population,indicating a protective role of IL1RL1 in the pathogenesis of BD.
文摘Behcet’s disease is defined as a multisystemic inflammatory disease.Although the precise pathogenesis and etiology is still a mystery,accumulating evidence shows that genetic variants of immune-related genes have a profound influence on the development of Behcet’s disease.To explore the genetic factors for Behcet’s disease,our group investigated the association of Behcet’s disease with multiple immune response genes and has identified multiple Behcet’s disease-related immunoregulatory pathways in the Chinese Han population.A large number of gene polymorphisms were studied including STAT4,IL23R,CD40,CCR1/CCR3,STAT3,OPN,IL17,JAK2,MCP-1,CTLA4,PD-1,PD-L1,PD-L2,TGRBR3,CCR6,PTPN22,FCRL3,IRF5,SUMO4 and UBAC2.Significant associations were found between Behcet’s disease and STAT4,IL23R,CD40,CCR1/CCR3,STAT3,MCP-1,TGFBR3,FCRL3,SUMO4,UBAC2.These genetic predisposition studies support an important role for both lymphocyte differentiation as well as ubiquitination pathways.These findings are helpful in elucidating the pathogenesis of Behcet’s disease and hopefully will allow the development of novel treatment regimes.
文摘Behcet's disease is a vasculitis disease involving multiple systems,and its pathogenesis is still unclear.The paper discusses the research progress on the treatment of Behcet's disease in traditional Chinese medicine and integrated traditional Chinese and Western medicine.The clinical efficacy of integrated traditional Chinese and western medicine in the treatment of Behcet's disease is significantly better than that of Western medicine alone,which is worthy of further research.
文摘Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.
文摘Behet's disease(BD) is a chronic inflammatory condition with multisystem involvement. Approximately 10%-15% of patients present with gastrointestinal involvement. Involved sites and the endoscopic view usually resemble Crohn's disease(CD). In addition to intestinal involvement, oral mucosa, the eyes, skin, and joints are commonly affected. No pathognomonic laboratory test is available for the diagnosis of either disease. Management approaches are also similar in various aspects. Differentiating BD from CD is highly challenging. In this article, the similarities and differences between BD and CD in terms of epidemiology, etiopathogenesis, clinical and imaging findings, and histopathological and therapeutic approaches are reviewed.
文摘AIM:To investigate effects of anti-TNF biologic drugs on uveitis severity(comparing visual acuity log MAR levels) in Behcet patients.METHODS:Three databases Pub Med, Scopus, and the Web of Science were searched for qualified papers focusing on the anti-TNF-α factors treatment in Behcet’s disease(BD)-associated uveitis. Studies that were designed pre and post anti-TNF drug treatment, were selected. After determining the search strategy for this study, the relevant data were extracted. RESULTS:The initial search was performed in the target databases and a total of about 1458 articles were found. Fifteen articles were selected for systematic review and only 12 of them had inclusion criteria for Meta-analysis(with visual acuity data). The mean dose of prednisolone before and after biological treatments was reported in 5 studies(28.56 and 7.56 mg/kg, respectively). Also, the preliminary results indicate a significant reduction in visual acuity log MAR levels(MD=-1.5 IU/L, 95%CI:-2.1,-0.01).CONCLUSION:Biological drugs significantly reduce the dose of prednisolone and affect visual acuity values.
文摘Objective: To elucidate the outcomes of open surgery for popliteal artery aneurysm (PAA) in a single Japanese institution. Materials and Methods: Between 1999 and 2016, 35 patients (44 PAAs) underwent open surgery in our department. The first-line surgical strategy involved the medial approach, with excision of the aneurysm sac and autologous vein bypass grafting. Associations between the outcomes and 1) the surgical management of the aneurysmal sac (aneurysmectomy vs. ligation with bypass) and 2) the preoperative clinical symptoms were evaluated. Results: There were no aneurysm-related deaths during the follow-up period (mean, 4 years;range, 1 - 17.6 years). The 5-year primary, primary-assisted, and secondary patency rates were 67.9%, 72.7%, and 94.5%, respectively. Re-intervention was required for 19 PAAs (14 patients), including two PAAs in patients with suspected Behçet’s disease. Patency rates did not differ between the aneurysmectomy and ligation groups. Remnant sac enlargement occurred in 3 cases. Among asymptomatic patients, clinical symptoms did not deteriorate;however, the ankle brachial pressure index decreased ≥0.15 in patients with artery-graft mismatching. Conclusion: Patency rates were good, and our modified ligation with bypass procedure, which involves excising the sac as much as possible, exhibited less risk in terms of remnant sac enlargement compared to that in previous studies.
文摘Introduction Behcet＇s disease（BD） is an multi-system inflammatory vasculitis based on small and large vessels of the venous and arterial system.About 80%of BD patients have ocular lesions characterized by recurrent iridocyclitis,retinal vasculitis,and retinal ischemia.Frequent recurrence of these lesions,leading to macular edema and optic atrophy,can eventually result in severe loss of vision.Western medicine treatment focuses on steroid and immunosuppressive agents;
