UNASSIGNED: Behcet\'s disease (BD) is a systematic vasculitis that affects vessels with various sizes, presenting as venous thrombosis and arterial pseudoaneurysms. The most severe manifestation in BD is ascending aortic pseudoaneurysm, which is associated with high risks of rupture and mortality.
UNASSIGNED: We present a case of ascending aortic pseudoaneurysm in a 50-year-old patient with BD. After preoperative evaluation, coil embolization was successfully performed to treat the pseudoaneurysm, resulting in a satisfactory outcome at the 1-year follow-up.
UNASSIGNED: Coil embolization serves as an effective treatment option for ascending aortic pseudoaneurysm in BD when open surgical repair and stent graft placement are unsuitable.

BACKGROUND: We present a rare case of NeuroBehcet\'s-related intracranial hypertension without cerebral venous thrombosis (NBrIHwCVT), occurring as the first presentation of NeuroBehcet\'s. In addition, we describe the novel use of subcutaneous tocilizumab for this indication. This is followed by a review of the literature on this topic.
METHODS: The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet\'s disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of > 40cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity.
METHODS: We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later.
CONCLUSIONS: As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.

UNASSIGNED: Oral ulcers, genital ulcers, and uveitis represent the typical trial of Behcet\'s disease (BD). It is well common on the Old Silk Road. The mucocutaneous lesions are the hallmark of BD, but neurological involvement is one of the severe symptoms. Headaches may be an early sign of BD neurological involvement. This study aims to investigate the headache prevalence and its types, and characteristics in a Syrian sample of BD patients.
UNASSIGNED: BD patients were clinically interviewed and examined to collect their information, symptoms, and signs. the International Study Group for Behcet\'s Disease diagnosis criteria was used to confirm the BD diagnosis. The International Classification of headaches was used when classifying the headaches.
UNASSIGNED: One hundred twenty participants were included in the study. half of them were BD patients and the control group was also 60 participants. Among Syrian BD patients, 36.7% suffer from primary headaches and 36.7% suffer from secondary headaches. These findings were not significantly different between the BD patients and the healthy population. Our results showed that there was no statistically significant difference between the two groups.
UNASSIGNED: Headache should not be considered a predictor for neurological involvement among BD patients. Additional attention to BD patients or the specific treatment for headaches is not required and does not differ from the general population.

Acute genital ulcers can affect females of all ages. In children, they often appear as an emergency and remain a diagnostic challenge for pediatricians, gynecologists and dermatologists. Prompt diagnosis and identification of disease- related factors help to implement appropriate treatment. Firstly, it is crucial to properly compile the past medical history of the patient. Past infectious, autoimmune, malignant or traumatic conditions, as well as vaccinations may contribute to the occurrence of acute genital ulcers. Moreover, new infectious agents, such as severe acute respiratory syndrome coronavirus 2 and vaccinations against Coronavirus disease of 2019, may play a significant role in the development of atypical clinical symptoms. Here we present a case of a 12-year-old girl with acute genital ulcers. Additional symptoms accompanying the ulcer included: abdominal pain, nausea, vomiting, dysuria, vulvar pain and fever. Blood test showed leukocytosis, especially neutrophilia and monocytosis and increased levels of c-reactive protein and procalcitonin. Serological tests for the most common infections were negative. Moreover, the patient had a history of autoimmune diseases. She had periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, and IgA vasculitis, also known as Henoch-Schönlein purpura in her past medical history. Additionally, she was vaccinated against SARS-CoV-2 shortly before the lesions appeared.

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.
METHODS: 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.
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.

UNASSIGNED: Behcet\'s disease is a multi-systemic inflammatory disorder. Paravalvular leakage and aortic pseudoaneurysm are rare in patients with Behcet\'s disease after aortic root replacement. Complicated post-operative infective endocarditis can make the treatment more difficult. We applied a flanged Bentall procedure to treat one such case.
UNASSIGNED: A 27-year-old man with aortic regurgitation and Behcet\'s disease underwent aortic root replacement. Post-operative electrocardiogram showed a complete atrioventricular block. One year after the operation, he underwent percutaneous temporary pacemaker implantation and endovascular stent graft exclusion because of pseudoaneurysm of the ascending aorta. Post-operative fever and blood culture confirmed infective endocarditis. Examination showed paravalvular leakage and pseudoaneurysm recurrence. Then, the patient underwent a third operation in our hospital. Aortic root replacement with a flanged composite valved conduit was performed. Immunosuppressants and antibiotic treatment were given after surgery. After 3 months, the cardiovascular examination was normal, and the patient was in good condition.
UNASSIGNED: Surgical treatment of aortic regurgitation caused by Behcet\'s disease was characterized by a high rate of paravalvular leakage, which led to reoperation and high mortality. Combined infective endocarditis would further increase the difficulty and risk of treatment. It is important to maintain effective immunosuppressive therapy while monitoring serum biomarkers and inflammation indicators. The potential hazards of immunosuppressants are increased risk of infection and poor tissue healing. In our case, targeted antibiotic treatment and appropriate immunosuppressive therapy were well balanced. The flanged Bentall procedure was also the key to success, which could increase aortic effective orifice area and reduce the risk of dehiscence.

Behcet\'s disease (BD) is a chronic inflammatory disorder characterized by a relapsing and remitting course and multisystem involvement. The authors present a case report of a 20-year-old male who presented with bilateral knee joint pain, oral and genital ulcers, and papulopustular skin lesions. The patient\'s clinical history, physical examination, laboratory findings, and biopsy results were consistent with the diagnosis of BD. The patient tested positive for the HLA-B51 allele, confirming a genetic predisposition. The diagnosis was supported by a positive pathergy test and a skin biopsy showing vasculitis. The diagnostic criteria established by the international study group and the International Criteria for Behcet\'s Disease were fulfilled. Treatment consisted of colchicine, azathioprine, and topical corticosteroids. This case highlights the importance of recognizing the varied clinical presentations of BD and the need for a multidisciplinary approach to diagnosis and management. Early and accurate diagnosis is crucial to prevent severe complications and improve patient outcomes.

Behçet\'s disease is a systematic, inflammatory disorder affecting vessels of all sizes. It affects both venous and arterial systems. Vascular involvement carries a high risk of morbidity and mortality. Knowing that Behçet\'s disease is the most common vasculitides that causes pulmonary artery aneurysms, with a mortality rate of around 25%, makes early detection crucial. Thrombosis in Behçet\'s disease is mainly caused by an inflammatory process rather than a thrombophilic state, thus vascular thrombosis control is achieved with immunosuppressant medications rather than anticoagulants. An exception to the use of anticoagulants in Behçet\'s disease appears to be due to cerebral venous thrombosis. The occurrence of multiple site thrombosis and aneurysm simultaneously makes the management very challenging, as we will highlight in our case. We present a case of a 31-year-old female patient with many prior hospitalizations due to cerebral venous thrombosis, bilateral pulmonary thrombi, right ventricular thrombus, and right pulmonary artery aneurysm. The patient was diagnosed with Behçet\'s disease according to the Behçet\'s Syndrome International Study Group criteria and then managed with the prophylactic low molecular weight heparin, cyclophosphamide, and prednisolone, resulting in significant improvement in the patient\'s symptoms. Presentation with cerebral venous thrombosis, pulmonary thrombosis, and aneurysm simultaneously is very rare in Behçet\'s disease. This made this case distinct and challenging in achieving good control of thrombosis and aneurysm simultaneously, which needs close monitoring and a multidisciplinary team to deal with the case.

UNASSIGNED: Behcet\'s disease is a vasculitis of unknown origin that can involve multiple organs or tissues. Aneurysm or pseudoaneurysm, also one of the complications of Behcet\'s disease, is usually accompanied by a poor prognosis. Surgery is usually accompanied by a high risk of complications, such as the recurrence of anastomotic pseudoaneurysms and blockage of the target vessel. Using hybrid surgery, we successfully treated a complex and recurrent abdominal aortic pseudoaneurysm in a patient with BD.
UNASSIGNED: We report a 32-year-old female diagnosed with Behcet\'s disease with recurrent thoracoabdominal aortic aneurysm. Adequate immunotherapy was given during the perioperative period. The splanchnic artery branches were reconstructed, and the aneurysm was sequestered with endovascular repair. The patient recovered uneventfully and was discharged from the hospital 8 days after hybrid surgery. At the 60-month follow-up, no aneurysm was observed, the stent had no displacement or internal leakage, and the reconstructed blood vessels were unobstructed.
UNASSIGNED: Hybrid surgery could be a feasible and effective strategy for BD aneurysms. Adequate preoperative and postoperative immunotherapy with arterial anastomosis away from the diseased artery may be the key to success.

Few case reports have mentioned the aortic sinus aneurysm invading ventricular septum and dissection caused by Behcet\'s disease. Here, we reported a 36-year-old male patient with an aortic sinus aneurysm invading the ventricular septum and dissection caused by Behcet\'s disease, who manifested as recurrent chest tightness and shortness of breath. Cardiac ultrasound showed the rupture of the right aortic sinus and the formation of ventricular septal dissection. Ascending aortic valve prosthesis replacement, mitral valvuloplasty with ring implantation and tricuspid valvuloplasty were performed. Postoperatively, he was treated with hormones, hydroxychloroquine sulfate, mycophenolate mofetil tablets, thalidomide and warfarin, and his symptoms were relieved. This is a rare case easily being misdiagnosed and missed, early diagnosis and in-time treatment are crucial to avoid surgical complications. The diagnostic and therapeutic approaches of this patient were reported and related literature was reviewed in this case report.