UNASSIGNED: Behçet\'s disease is a chronic, multisystemic, and relapsing inflammatory disorder. It lacks a permanent cure, the focus of treatment is on mitigating symptoms, decreasing the frequency and severity of relapses, and preventing life-threatening complications. This study aims to report the experience of a single center in managing patients with Behçet\'s disease and discuss the treatment outcomes.
UNASSIGNED: This study was a retrospective case series conducted over 2 years. All cases were clinically diagnosed according to the International Criteria for Behçet\'s Disease. The extracted data were demographics, family history, clinical findings, criteria scores, treatment, and outcomes.
UNASSIGNED: A total of 31 patients were included, consisting of 13 males (42%) and 18 females (58%). Most cases were over the age of 30, and both genders were nearly equally distributed among age groups. The most commonly affected site was the oral cavity, observed in 96.77% of cases. Genital, cutaneous, and vascular involvements were more common in males, while females were more likely to have oral, ocular, and musculoskeletal involvements. For various treatment regimens, oral, cutaneous, vascular, and musculoskeletal involvements showed complete response in all cases. Among cases with genital involvement, complete response was achieved in seven cases (41.2%), while four cases (23.5%) showed only partial response, and six cases (35.3%) experienced recurrence. In cases with ocular involvement, only partial responses were observed.
UNASSIGNED: Oral, cutaneous, vascular, and musculoskeletal involvements may have a higher likelihood of a complete response to treatment regimens. However, genital involvement may be the most recurrent manifestation, followed by ocular involvement.

Behcet\'s disease (BD) is an uncommon, long-term inflammatory condition characterized by recurring ulcers in the mouth and genital area, uveitis, and various systemic issues. One of the particularly rare but severe complications of this disease is the formation of pulmonary artery aneurysms (PAAs). Although these aneurysms are uncommon, they can lead to dangerous pulmonary hemorrhages (PHs), which are often fatal, requiring prompt diagnosis and intervention. We present a case of lower segment PAA in an 18-year-old patient with recently diagnosed BD, presenting with life-threatening PH and managed successfully with microcoil embolization of the aneurysm and immunosuppressive (IS) medications, achieving stable remission without complications.

Oral ulcers are one of the most common complaints seen by general practitioners in their offices. Recurrent aphthous stomatitis affects roughly 20% of the general population. When ulcers persist despite conventional treatment, it is crucial to consider systemic diseases such as Behçet\'s disease to prevent delays in care. Early recognition and appropriate management of underlying conditions are essential for improving patient outcomes and quality of life. We present a case of a 41-year-old Scottish male who came in with complaints of recurrent oral ulcers and oral thrush. Initial treatment by an infectious disease specialist resolved the oral thrush but not the ulcers. Despite further treatment attempts for three years, including biopsy and antiviral therapy, ulcers persisted. Finally, referral to rheumatology led to comprehensive autoimmune testing, revealing positive HLA B51 and a diagnosis of Behçet\'s disease. Treatment with topical steroids and colchicine yielded significant improvement.

Acute myocardial infarction (AMI) in young patients is an important issue because of its impact on health and social life. The mechanisms and disease courses of ST-elevation myocardial infarction (STEMI) in young individuals may differ from those in the elderly. Behcet disease (BD) is a multisystem autoimmune disorder of unknown etiology. Cardiac involvement is rare, yet it was reported to affect 6% of patients, with 17% of the cases presenting as the first manifestation. We present the case of a 33-year-old male heavy smoker with negative medical history, who presented with acute inferior myocardial infarction. His coronary angiography showed huge thrombosis in proximal right coronary artery. He was treated with primary coronary intervention and implantation of drug-eluting stent, with subsequent intervention and implantation of two more drug-eluting stents due to acute stent thrombosis within 48 h. Rheumatologic assessment revealed the history of four different attacks of oral ulcers and one attack of genital ulcer. His workup showed positive human leukocyte antigen (HLA) allele (B51) which is strongly associated with BD. AMI in young adults due to arterial thrombosis can be attributed to hypercoagulable state related to early manifestation of BD. Increased knowledge of AMI in young adults and its presentation in BD is necessary to reduce morbidity and mortality. Corticosteroids and colchicine may improve cardiac manifestations in BD.

BACKGROUND: Behçet disease (BD) is a multisystemic recurrent inflammatory disorder that was originally described as a triad of oral and genital ulcerations with uveitis (Behcet, 1937 [1]). Arterial involvement is the most common cause of mortality in patients with BD. Aneurysms are common among the arterial lesions and affect various arteries, but mostly the abdominal aorta. Vascular lesions are encountered in 7 %-29 % of patients, gravely affecting the course of the disease. Extracranial carotid aneurysms due to Behçet\'s disease are extremely rare (Bouarhroum et al. (2006) [2]).
METHODS: Herein, we present a 19 year old man presented with hoarsness due to pressure effect to our outpatient clinic.
UNASSIGNED: Due to findings in the computed angiography, he underwent surgery twice.A 100*8 COVERA-covered stent was deployed at the bifurcation of the brachiocephalic artery. Then a 40*13.5 FLUENCY stent with a 2 cm overlap from the previous stent was deployed.
CONCLUSIONS: Further investigations regarding endovascular approach for this rare disease is recommended.

UNASSIGNED: Ulcus Vulvae Acutum Lipschütz (UVAL) is a largely unknown disease with a broad and complex differential diagnosis.
UNASSIGNED: To provide a description of the main characteristics of UVAL, determine the most appropriate diagnostic process and describe the current therapeutic approach.
UNASSIGNED: We designed a retrospective, descriptive cohort study using the gynecological-ER database of our institution. Inclusion criteria: female patients aged between 10 and 20 years old with suspicion of a UVAL diagnosis at CHUV\'s gynecological ER. Data extraction: epidemiological characteristics, clinical presentation, laboratory tests, established diagnostics, treatment, and ulcer outcomes.
UNASSIGNED: 15 patients were included for the analysis; average age: 15 years old; 60% of patients were virgo at the time of ulcer onset; all patients had at least one flu-like symptom concomitant with the vulvar lesion; the most-performed serology was for EBV and acute disease was present in only one patient; for diagnostic purposes two biopsies were performed with both inconclusive histopathology analysis; the main prescribed treatments were: oral NSAIDs, Paracetamol, and Lidocaine gel; 93% of cases presented signs of regression; the average follow-up time was 10 days. The diagnostic algorithm of Sadoghi et al: 10 out of 15 cases were retrospectively diagnosed with UVAL by the algorithm; half were diagnosed with UVAL, and the other half received a diagnosis of \"ulcers of unknown origin\" at the time of the gynecological ER visit.
UNASSIGNED: We highly recommend the diagnostic and therapeutic algorithms developed by Sadoghi et al. as valuable tools to guide clinical reasoning and, consequently, improve acute vulvar ulcers management.

Amyloid deposition in the thyroid gland is a common presentation, yet amyloid goiter remains relatively rare. Proper differentiation of this condition from other goiter types and malignancies is essential. Although amyloid extensively invades the thyroid gland, patients are usually euthyroid, and many different presentations may occur. We report a case of a 42-year-old male patient who was diagnosed with secondary amyloidosis due to Behcet\'s disease. He presented with clinical manifestations of hyperthyroidism and systemic amyloidosis complicated by chronic kidney disease, which is the first case of such an entity to be reported in Palestine.

Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We describe a rare case of a 39-year-old Saudi male who presented with isolated neurological manifestations as the first sign of BD. The patient had recurrent strokes, both ischemic and hemorrhagic, over an 11-month period before developing typical BD features. A thorough investigation excluded other potential etiologies of his neurological disorders. Imaging showed multiple brainstem lesions compatible with parenchymal neuro-BD (NBD). The patient was positive for HLA-B51, a genetic marker linked to BD, but had a negative pathergy test. Treatment with corticosteroids and infliximab resulted in symptom improvement. The diagnosis of NBD requires a comprehensive clinical, imaging, and laboratory assessment to rule out other possible causes. This case demonstrates the need to include NBD in the differential diagnosis of young patients with unexplained neurological manifestations, especially if they are followed by an onset of BD features. Treatment with corticosteroids and biologic agents can achieve favorable outcomes. NBD can present with isolated neurological symptoms, emphasizing the need for a high level of suspicion and a multidisciplinary approach for accurate diagnosis and effective management.

BACKGROUND: Behcet disease (BD) as a variable vessel vasculitis is mainly characterized by ocular involvement, genital and oral aphthosis, and erythema nodosum. However, major organ involvements including gastrointestinal involvement, nervous system, and vascular involvement are among the severe complications. Osteonecrosis is a rare complication of patients with BD. We aim to report the largest series of BD patients suffering from osteonecrosis.
METHODS: We have retrospectively reviewed all patients in Iran Behcet\'s Disease Registry and reported those with osteonecrosis. Patients\' medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed.
RESULTS: Seven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated.
CONCLUSIONS: ON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated.

UNASSIGNED: Non-infectious uveitis is often secondary to systemic autoimmune diseases, with Behçet\'s disease (BD) and Vogt-Koyanagi-Harada disease (VKHD) as the two most common causes. Uveitis in BD and VKHD can show similar clinical manifestations, but the underlying immunopathogenesis remains unclear.
UNASSIGNED: To understand immune landscapes in inflammatory eye tissues, we performed single-cell RNA paired with T cell receptor (TCR) sequencing of immune cell infiltrates in aqueous humour from six patients with BD (N = 3) and VKHD (N = 3) uveitis patients.
UNASSIGNED: Although T cells strongly infiltrated in both types of autoimmune uveitis, myeloid cells only significantly presented in BD uveitis but not in VKHD uveitis. Conversely, VKHD uveitis but not BD uveitis showed an overwhelming dominance by CD4+ T cells (> 80%) within the T cell population due to expansion of CD4+ T cell clusters with effector memory (Tem) phenotypes. Correspondingly, VKHD uveitis demonstrated a selective expansion of CD4+ T cell clones which were enriched in pro-inflammatory Granzyme H+ CD4+ Tem cluster and showed TCR and Th1 pathway activation. In contrast, BD uveitis showed a preferential expansion of CD8+ T cell clones in pro-inflammatory Granzyme H+ CD8+ Tem cluster, and pathway activation for cytoskeleton remodelling, cellular adhesion and cytotoxicity.
UNASSIGNED: Single-cell analyses of ocular tissues reveal distinct landscapes of immune cell infiltration and T-cell clonal expansions between VKHD and BD uveitis. Preferential involvements of pro-inflammatory CD4+ Th1 cells in VKHD and cytotoxic CD8+ T cells in BD suggest a difference in disease immunopathogenesis and can guide precision disease management.