UNASSIGNED: We report a unique case of non-necrotizing occlusive retinal vasculitis presenting two years following chronic hypertensive uveitis.
UNASSIGNED: Case Report.
UNASSIGNED: A 32-year-old Iraqi woman with a history of Posner-Schlossman Syndrome diagnosed 10 years prior presented with blurred vision and redness in her left eye. Examination demonstrated ocular hypertension, keratic precipitates, and inflammatory cells in the anterior chamber. Quantitative real-time PCR confirmed the presence of cytomegalovirus in the aqueous humor, and dilated posterior segment examination was negative for any signs of intraocular inflammation, retinitis, or vasculitis. Her uveitis workup was otherwise negative, and she was treated with valganciclovir for 6 months. Two years after her initial presentation, she was noted to have a new vitreous hemorrhage in the left eye. Fluorescein angiography demonstrated an occlusive retinal vasculitis with extensive neovascularization without retinitis. Quantitative real-time PCR again demonstrated the presence of cytomegalovirus in the anterior chamber. Her uveitis workup was repeated, which has now returned positive for HLA-B51. She otherwise did not demonstrate any systemic signs of Behcet\'s Disease. She was restarted on valganciclovir and oral prednisone and referred to rheumatology for consideration of adalimumab initiation. Thus far she has responded very well to treatment.
UNASSIGNED: This case highlights the importance of serial posterior segment examinations and HLA-B51 testing in individuals with cytomegalovirus anterior uveitis.

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.

BACKGROUND: 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.
CONCLUSIONS: 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.

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.

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.

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.

UNASSIGNED: To describe the distribution, clinical findings, treatment, complications, and visual outcomes of pediatric uveitis at a tertiary referral ophthalmic center.
UNASSIGNED: The medical records of all patients ≤18 years diagnosed with and managed as uveitis from August 2016 to August 2021 were reviewed retrospectively.
UNASSIGNED: Of the 97 patients, 52.6% were female, and the mean age at the onset was 10.5 ± 4.6 years (6 months to 18 years). Uveitis cases were predominantly anterior (33 [34%]), chronic (59 [60.8%]), bilateral (63 [64.9%]), and non-infectious (80 [82.5%]). A total of 36.1% (35 patients) of cases were idiopathic, and the most frequent systemic associations were juvenile idiopathic arthritis (JIA), 16 [16.5%]) and Behcet\'s disease (15 [15.5%]). Most patients (74 [76.3%]) experienced ocular complications, including vasculitis (29 [29.9%]), posterior synechiae (23 [23.7%]), and cataracts (22 [22.7%]). Patients with uveitis of all anatomic locations experienced an improvement in best-corrected visual acuity during the follow-up period (p < 0.01).
UNASSIGNED: The most prevalent systemic associations were JIA and Behcet\'s disease. Ocular Behcet is a common etiology of pediatric uveitis in northeastern Iran. A timely and appropriate treatment could result in satisfactory visual outcomes.