Predicting Factors of Long-term Outcome of Gastrointestinal Behçet's Disease: A Chinese Retrospective Study.

PURPOSE: Behçet's disease (BD) is a complex disorder affecting multiple systems and organs, and gastrointestinal BD is poorly understood. We aimed to identify factors influencing the long-term outcomes of patients with gastrointestinal BD. METHODS: Consecutive patients with gastrointestinal BD were analyzed retrospectively. Data on the following clinical characteristics were collected: sex, age at diagnosis, symptoms, endoscopic findings, medical treatments, and surgery. Mucosal healing and surgical rates at 1, 2, and 5 years were evaluated. Log-rank test and Cox proportional hazards regression models were used to evaluate the factors affecting long-term outcomes. FINDINGS: Baseline data of 175 patients with gastrointestinal BD were included. The mean (SD) age at diagnosis was 38.3 (12.9) years. The typical clinical symptoms were oral ulcer (72.6%), abdominal pain (71.4%), and weight loss (41.1%). The most commonly involved location was the ileocecum; isolated oval ulcer was the most common ulcer type. Seventeen patients (9.7%) underwent 18 surgeries after inclusion. The cumulative surgical rates were 8.6% (n/N = 15/175), 8.6% (n/N = 15/175), and 9.1% (n/N = 16/175) in 1, 2, and 5 years, respectively. Data from 101 patients who underwent at least 2 endoscopies were included in the analysis for mucosal healing. Kaplan-Meier curve showed that the cumulative mucosal healing rates at 1, 2, and 5 years were 34.7% (n/N = 35/101), 41.6% (n/N = 42/101), and 61.4% (n/N = 62/101), respectively. We compared cumulative mucosal healing rates between 4 treatment groups, including 5-aminosalicylic acid (3% [n/N = 3/101]), mono-immunosuppressant (31.7% [n/N = 32/101]), combined therapy (36.6% [n/N = 37/101]), and escalation therapy (28.7% [n/N = 29/101]), and found that mono-immunosuppressant achieved earlier mucosal healing than combined therapy (P = 0.0008) and escalation therapy (P = 0.0008). The univariate analysis showed that moderate to severe disease activity (P = 0.013, P = 0.004), diameter of the maximal ulcer >4 cm (P = 0.002), and nonsimple esophageal involvement (P < 0.001) were risk factors, and number of ulcers between 2 and 5 was the protective factor of mucosal healing (P = 0.001). Multivariate regression analysis indicated that nonsimple esophageal involvement (P < 0.001) and the maximal ulcer >4 cm (P = 0.041) were independent risk factors of mucosal healing. IMPLICATIONS: Most patients with gastrointestinal BD need long-term treatment to achieve mucosal healing. The location and size of ulcers have a significant impact on the mucosal healing of gastrointestinal BD.

Endovascular repair with a physician-modified fenestrated endograft to treat abdominal aortic pseudoaneurysm with Behcet's disease: a case report.

BACKGROUND: Aortic involvement in patients with Behcet's disease (BD) is rare, but it is one of the most severe manifestations. Open surgical repair of aortic aneurysm is challenging considering the high risk of postoperative recurrent anastomotic pseudoaneurysms and is associated with a much higher mortality rate. Recently, endovascular treatment has proven to be a feasible, less invasive alternative to surgery for these patients. CASE PRESENTATION: We report a total endovascular repair of a paravisceral abdominal aortic pseudoaneurysm in a 25-year-old male patient with BD. The pseudoaneurysm was successfully excluded, and the blood supply of visceral arteries was preserved with a physician-modified three-fenestration endograft under 3D image fusion guidance. Immunosuppressive therapy was continued for 1 year postoperatively. At 18 months, the patient was asymptomatic without abdominal pain. Computed tomography angiography demonstrated the absence of pseudoaneurysm recurrence, good patency of visceral vessels. DISCUSSION AND CONCLUSIONS: Endovascular repair using physician-modified fenestrated endografts is a relatively safe and effective approach for treating paravisceral aortic pseudoaneurysm in BD patients. This technique enables the preservation of the visceral arteries and prevents aneurysm recurrence at the proximal and distal landing zones, which are common complications of open surgical repair in these patients. Furthermore, we emphasize the importance of adequate immunosuppressive therapy before and after surgical repair in BD patients, which is a major risk factor for recurrence and poor prognosis.

Tratamento endovascular na doença de Behçet: uma revisão integrativa / Endovascular treatment in Behçet's disease: an integrative review

Resumo A doença de Behçet (DB) é caracterizada por ser uma doença rara, crônica, recidivante, inflamatória e multissistêmica. Quanto ao diagnóstico, não existe nenhum teste universalmente descrito; logo, ele é feito de maneira clínica, por meio da presença da sua tríade clássica de sinais: úlceras orais, úlceras genitais e uveíte (inflamação do trato uveal do olho). O objetivo deste estudo foi avaliar a importância do tratamento endovascular na doença de Behçet, sendo que a revisão de literatura para obter essa resposta foi realizada a partir de 30 artigos publicados entre 2002 e 2021. A DB acomete tanto o sistema venoso quanto o arterial. A ruptura de aneurismas é a principal causa de morte e necessita de tratamento, podendo ser clínico, cirúrgico aberto ou por via endovascular. A cirurgia endovascular vem ganhando espaço no tratamento cirúrgico nos casos de aneurismas arteriais, inclusive nos relacionados com DB, apesar de a terapia de escolha ainda ser controversa.

Abstract Behçet's Disease (BD) is a rare, chronic, relapsing, inflammatory, and multisystemic disease. There is no universally described test for confirming diagnosis, so it is made clinically, on the basis of its classic triad of signs: oral ulcers, genital ulcers, and uveitis (inflammation of the uveal tract of the eye). The aim of this study is to evaluate the importance of endovascular treatment in Behçet's disease. The literature review conducted to achieve this objective covered 30 articles published between 2002 and 2021. Behçet's Disease affects both the venous and arterial systems. Rupture of aneurysms is the main cause of death and thus requires treatment, which can be clinical, open surgical, or endovascular. Endovascular surgery has been gaining ground for surgical treatment of arterial aneurysms, including those related to BD, although the therapy of choice is still controversial.

Manejo anestésico de un paciente con enfermedad de Behçet / Anesthetic management of a patient with Behçet´s Disease Sobre los autores:

RESUMEN Introducción: La enfermedad de Behçet es una enfermedad inflamatoria crónica, recurrente, multisistémica, de etiología desconocida, caracterizada por úlceras orales y genitales recurrentes, inflamación ocular, lesiones cutáneas, artritis, afecciones neurológicas, pulmonares, gastrointestinales y vasculitis sistémica. Objetivo: Describir el manejo anestésico en un paciente portador de enfermedad de Behçet. Presentación del caso: Se reporta el caso de un paciente de 52 años de edad con antecedentes patológicos personales de enfermedad de Behçet que recibe anestesia general para exéresis de adenopatía cervical izquierda metastásica de un carcinoma primario oculto. Conclusiones: El mantenimiento del tratamiento con esteroides, el uso de nadroparina cálcica junto a otras medidas preventivas de la trombosis venosa profunda, el manejo cuidadoso de la vía aérea, la protección ocular y la articular, así como de los puntos de presión y la prevención de la patergia son elementos fundamentales en el manejo de estos pacientes.

ABSTRACT Introduction: Behçet's disease is a chronic, recurrent, multisystemic inflammatory disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular inflammation, skin lesions, arthritis, neurological, pulmonary and gastrointestinal conditions, as well as systemic vasculitis. Objective: To describe the anesthetic management of a patient with Behçet's disease. Case presentation: The case is reported of a 52-year-old male patient with an individual history of Behçet's disease, who receives general anesthesia for removal of left cervical metastatic adenopathy from a hidden primary carcinoma. Conclusions: Keeping the steroid therapy, using calcium nadroparin, together with other measures for preventing deep vein thrombosis; careful airway management, eye and joint protection, as well as attention to pressure points and pathergy prevention are fundamental elements for the management of these patients.

Coronary involvement in behçet's disease: what are its risks and prognosis? (rare cases and literature review)

Abstract Objective: In our clinic, we aimed to investigate the effect of preoperative risk factors and postoperative complications on reoperation and mortality in cases with Behçet's disease which presents very rare coronary artery involvement. Methods: Thirteen patients with Behçet's Disease who had undergone coronary artery bypass grafting in our center between 2003 and 2015 were analyzed. We evaluated the clinical and laboratory findings, complications and mortality rates of our patients in light of the literature. Results: The mean age was 38.5 (30-55; 3 women). The mean time from onset of Behçet's disease to coronary artery disease was 4,7 (3-11) years. Fifty-four percent of the patients were asymptomatic. Coronary artery disease of these was exposed while peripheral vascular surgery was planned due to complications of Behçet's disease. Symptomatic patients presented angina pectoris (31%), acute coronary syndrome (8%) and arrhythmia (8%). In coronary pathology of patients, distal type obstruction (31%), aneurysm and pseudoaneurysm (31%), proximal segment thrombus (15%), chronic type stenosis and occlusions (31%) were present. Early mortality (15%) was due to acute myocardial infarction while the late mortality (15%) was due to cerebral and gastrointestinal bleeding. Reoperation was due to bleeding in one case on the 1st postoperative day and due to acute pulmonary embolism in another case in the 3rdpostoperative year. Conclusion: In Behçet's disease, coronary artery bypass grafting is a procedure with high mortality, especially in the acute period. The on-pump surgery technique in these cases can be safely performed for multiple bypasses and in patients above 40 years old.

Outcomes of surgery for patients with Behcet's disease causing aortic pseudoaneurysm: a shift from open surgery to endovascular repair

OBJECTIVES: Behcet’s disease is a form of systematic vasculitis that affects vessels of various sizes. Aortic pseudoaneurysm is one of the most important causes of death among patients with Behcet’s disease due to its high risk of rupture and associated mortality. Our study aimed to investigate the outcomes of Behcet’s disease patients with aortic pseudoaneurysms undergoing open surgery and endovascular aortic repair. METHODS: From January 2003 to September 2014, ten consecutive patients undergoing surgery for aortic pseudoaneurysm met the diagnostic criteria for Behcet’s disease. Endovascular repair was the preferred modality and open surgery was performed as an alternative. Systemic immunosuppressive medication was administered after Behcet’s disease was definitively diagnosed. RESULTS: Eight patients initially underwent endovascular repair and two patients initially underwent open surgery. The overall success rate was 90% and the only failed case involved the use of the chimney technique to reach a suprarenal location. The median follow-up duration was 23 months. There were 7 recurrences in 5 patients. The median interval between operation and recurrence was 13 months. No significant risk factors for recurrence were identified, but a difference in recurrence between treatment and non-treatment with preoperative immunosuppressive medication preoperatively was notable. Four aneurysm-related deaths occurred within the follow-up period. The overall 1-year, 3-year and 5-year survival rates were 80%, 64% and 48%, respectively. CONCLUSIONS: Both open surgery and endovascular repair are safe and effective for treating aortic pseudoaneurysm in Behcet’s disease patients. The results from our retrospective study indicated that immunosuppressive medication was essential to defer the occurrence and development of recurrent aneurysms.

Falso aneurisma femoral en enfermedad de Behcet / Femoral pseudoaneurism in Behcet's disease

La enfermedad de Behcet es una patología poco frecuente en Chile que afecta principalmente a adultos jóvenes de países Mediterráneos y Japón. Se caracteriza por compromiso multisistémico siendo las manifestaciones cardiovasculares inusuales. Presentamos el caso de un paciente portador de Enfermedad de Behcet, quien es hospitalizado por dolor y aumento de volumen pulsátil inguinal derecho. El estudio angiográfico revela falso aneurisma femoral común de 5 cm de diámetro. Luego de destacar compromiso arterial en otra localización es intervenido demostrándose que el falso aneurisma se origina desde un defecto posterolateral el cual es reparado con parche de vena safena. No se presentaron complicaciones postoperatorias. El estudio histopatológico no demostró fragmentación o disminución en el número de fibras elásticas ni el depósito de inmunoglobulinas o complemento