De novo manifestations during adalimumab treatment in Behçet's syndrome.

OBJECTIVES: Treatment response may be variable across organ manifestations of Behçet syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment. METHODS: We conducted a chart review of all BS patients who received adalimumab in our center between 2008 and 2023. Demographic data, reasons for initiating adalimumab, concurrent medications, previous treatments, and outcomes were recorded. We defined de novo manifestations as new BS manifestations that occurred for the first time during treatment with adalimumab. For patients with vascular involvement, a new vascular event at another vessel was also considered as a de novo manifestation. RESULTS: Among the 335 patients, a de novo manifestation developed in 14 (4%) patients. De novo manifestations were vascular involvement in 5 patients, arthritis in 3, anterior uveitis in 2, nervous system involvement in 2, gastrointestinal involvement in 1, and epididymitis in 1 patient. The primary reasons for adalimumab treatment were vascular involvement in 5 patients, uveitis in 4, arthritis in 3, mucocutaneous involvement in 1, and epididymitis in 1 patient. Upon the development of de novo manifestation, adalimumab was switched to another biologic in 4 patients, dose was intensified in 3, colchicine, conventional immunosuppressives, and/or glucocorticoids were added in 5, and topical eye drops were added in 2 patients, leading to remission of de novo manifestations in all patients. CONCLUSION: De novo manifestations were infrequent (4%) among BS patients treated with adalimumab. Of these, 57% were major organ involvement, mainly vascular involvement. None of the patients developed posterior uveitis.

Validation of the Behçet's Syndrome Overall Damage Index (BODI) for Retrospective Studies and a Proposal for Modification.

OBJECTIVE: Assessment of damage accrual over time is important for evaluating and comparing long-term results of treatment modalities and strategies. Retrospective studies may be useful for assessing long-term damage, especially in rare diseases. We aimed to validate Behçet's Syndrome Overall Damage Index (BODI) for use in retrospective studies by evaluating its construct validity, reliability, and feasibility in retrospectively collected data. Additionally, we aimed to determine missing items by evaluating Behçet's syndrome patients with different types of organ involvement and long-term follow-up. METHODS: We included 300 patients who had at least 2 clinic visits at 1-year intervals. The construct validity for use in retrospective trials was assessed by comparing BODI scores calculated from patient charts and during face-to-face visits. BODI was additionally scored using retrospective chart data by 2 different observers and by the same observer six months apart, in a blinded manner. The time for filling BODI was evaluated to assess feasibility. Additionally, damaged items that were missing from BODI were identified. RESULTS: There was a good correlation between the retrospective and face-to-face evaluation of BODI (ICC 0.99; %95 CI 0.99-0.99). Inter-observer and intra-observer agreement were good (ICC 0.96 and 1, respectively). The main damage items that BODI did not capture were hypertension, liver failure, lung parenchymal involvement, glaucoma, and lymphedema. CONCLUSION: BODI seems to be a reliable and feasible instrument for assessing damage in retrospective studies. Modifying BODI using the additional damage items identified in this study may make it an even better scale.

HLA-B5 prevalence in patients with spondyloarthritis and impact on disease phenotype: a multicentric case-control study.

OBJECTIVE: The study aimed to estimate the prevalence of HLA-B51 and HLA-B52 in Lebanese patients with spondyloarthritis (SpA) compared to healthy controls (HC). We further aimed to evaluate the impact of HLA-B51 on phenotype and identify the distribution of the alleles in the HLA-B locus. METHODS: A case-control study enrolled consecutive SpA patients from three rheumatology clinics in Lebanon, including axial (axSpA), peripheral SpA (pSpA), and psoriatic arthritis (PsA) and HC from blood donors. Demographic and disease data were collected through interviews and file reviews, with testing of the entire HLA-B locus using molecular techniques. The prevalence of HLA-B51 and B52 was estimated in SpA patients versus controls. Prevalence comparisons were made, and logistic regression identified factors associated with HLA-B51 in patients. RESULTS: Data from 120 HC and 86 SpA patients (65 axSpA, 15 pSpA, 6 PsA), mean age 25.6 and 46.4 years, respectively, showed a higher HLA-B51 prevalence in SpA (25.6%), especially axSpA (29.2%) versus HC (12.5%), p = 0.016, and a numerically higher HLA-B52 prevalence (8.1% versus 4.2%, p = 0.230). HLA-B51 correlated with recurrent oral ulcerations (OR 7.99(95%CI 2.14-29.84) and radiographic juxta-articular erosions (OR 7.65(95%CI 1.14-38.03)). HLA-B35 was the most dominant allele in both groups (18.7%), followed by HLA-B27 (15.7%) and HLA-B51 (13.4%) in SpA. CONCLUSION: HLA-B51 was identified more frequently in patients with SpA compared to HC and was associated with recurrent oral ulcerations and juxta-articular radiographic erosions. Longitudinal studies are needed to determine whether this association indicates a disease overlap or might correlate with a specific SpA phenotype.

Impact of age at diagnosis on long-term prognosis in patients with intestinal Behçet's disease.

BACKGROUND AND AIM: Although age at disease onset is considered to be a significant factor in the prognosis of Crohn's disease, little is known about its influence on the long-term prognosis of those with intestinal Behçet's disease (BD). This study aimed to evaluate the long-term clinical outcomes of patients with intestinal BD according to age of disease onset. METHODS: Patients diagnosed with intestinal BD at < 18, 18-60, and > 60 years of age were classified into early-onset, adult-onset, and late-onset groups, respectively. The influence of disease onset time on clinical prognosis, including specific medical requirements, BD-related intestinal surgery, hospitalization, and emergency room visits, was compared using the log-rank test in a large cohort of patients with intestinal BD. RESULTS: Among 780 patients, 21 (2.7%), 672 (86.2%), and 87 (11.1%) comprised the early-onset, adult-onset, and late-onset groups, respectively. Patients in the early-onset group were more likely to require immunosuppressants than those in the adult-onset group (P = 0.048). Nine (42.9%), 158 (23.5%), and 18 (20.7%) patients in the early-onset, adult-onset, and late-onset groups, respectively, underwent intestinal resection. The early-onset group exhibited a higher risk for intestinal resection than the late-onset (P = 0.043) and adult-onset (P = 0.030) groups. The late-onset group exhibited a higher risk for BD-related hospitalization than the adult-onset group (P = 0.023). CONCLUSIONS: Age at diagnosis affected the clinical course of intestinal BD, including intestinal surgery, hospitalization, and specific medical requirements. Different treatment strategies should be established according to age at diagnosis.

Enthesitis as an initial presentation of vascular Behçet's syndrome: a case-based review.

Enthesitis is a characteristic manifestation of spondyloarthropathy (SpA). Historically, Behçet's syndrome (BS) was classified within SpA. Although they are now classified separately, the association between BS and SpA remains controversial. The concept of MHC-I (major histocompatibility complex class I)-opathy has been proposed based on the overlap in immunopathological mechanisms among diseases associated with human leukocyte antigen (HLA) class I. Enthesitis is a frequent complication in patients with BS who also have acne and arthritis. However, information regarding enthesitis in patients with BS without arthritis (BS-WA) is limited. Herein, we report a case of vascular BS complicated by enthesitis. In this case, heel pain was the dominant symptom at presentation. Laboratory tests revealed chlamydia antibody positivity, leading to a tentative diagnosis of reactive arthritis. Despite treatment, C-reactive protein (CRP) levels remained elevated. Imaging revealed numerous aneurysmal lesions in the large vessels. Based on these findings and other symptoms, patient was diagnosed with vascular BS. He tested positive for HLA-B15 and HLA-B46, which are associated with peripheral SpA. Subsequent remission induction therapy for BS was effective and the patient was discharged without complications. Our case and a literature review suggest that there exists a subgroup of BS-WA with a complication of enthesitis, possibly belonging to the spectrum of MHC-I-opathies. It is important to consider BS as a differential diagnosis in patients presenting with enthesitis and to conduct a precise medical history review regarding the symptoms of BS.

Sexual dysfunction among female patients with rheumatic diseases.

To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet's syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.e., female sexual function index (FSFI), Beck depression inventory (BDI), body cathexis scale, and marital adjustment test (MAT) were performed. Scale scores were compared, and binary logistic regression was used to identify predictors for SD in the whole group. The total FSFI and body cathexis scores among the patient groups were significantly lower than those of the HCs (p < 0.001). Depression was significantly more frequent in the patient groups. MAT scores did not differ significantly between the study groups. Patients with SSc had the worst scores in each psychometric index, including MAT. Decreased body cathexis score [OR 0.974, 95% CI (0.957-0.991), p = 0.003] and low MAT score [OR 0.937, 95% CI (0.896-0.980), p = 0.005], and being diagnosed with SSc [OR 6.6, 95% CI (1.975-22.498), p = 0.002], SLE [OR 2.7, 95% CI (0.998-7.753), p = 0.050], and BS [OR 2.8, 95% CI (1.100-7.359), p = 0.031], were identified as independent predictors for SD. Body cathexis seems to be the most important independent predictor for SD, and the burden of SD appears heavier in patients with SSc, probably due to poor body image satisfaction.

The Association of Behçet's Disease on the Quality of Life for Children and Their Parents.

Background and Aim Chronic diseases profoundly impact people's quality of life (QoL). Behçet's Disease (BD) is a multisystemic chronic disease characterized by vasculitis of various vessels. We aimed to assess QoL in pediatric BD patients and their parents. Methods We included pediatric BD patients meeting the pediatric BD classification criteria in a cross-sectional study conducted from June to December 2022. We recorded patients' clinical characteristics and assessed their QoL using PedsQL. Parents' QoL was evaluated using WHOQOL-Bref. Results The study involved 38 patients (60.5% girls, 39.5% boys) with a median age of 15.5 years (10-17) and a disease duration of 2 years (1-4) and their 38 parents. All patients exhibited oral aphthae, and many had other mucocutaneous findings: genital ulcers (78.9%), pseudofolliculitis (76.3%), and erythema nodosum (23.6%). Some patients had uveitis (13.1%), vascular (13.1%), neurological (10.5%), and gastrointestinal (5.2%) involvement. All patients were in remission under treatment during the study. Median PedsQL scores for total, physical health, and psychosocial health were 74.5(40-94.8), 76.5(43-100), and 75(25-92), respectively, with 14 patients scoring below the cut-off value. Girls had lower physical health scores than boys (p=0.004), and a negative correlation emerged between disease duration and PedsQL score (r=0.648, p=0.001). The median WHOQOL score among parents was 50(25-100), with 20 scoring below the cut-off value. Conclusion The QoL was low for one-third of children with pediatric BD and more than half of their parents.Similar to numerous chronic illnesses, factors such as the duration of the disease and gender were associated with the QoL in pediatric BD.

Screening for familial disease presence in first-degree relatives of Behçet's disease patients: Is measurement of common femoral vein wall thickness valuable for the diagnosis?

OBJECTIVES: We aimed to assess first degree relatives (FDRs) of BD patients for the presence of clinical symptoms and signs of BD and evaluate common femoral vein (CFV) wall thickness measurement for the diagnosis. METHODS: Patients with BD(n=129) and FDRs(n=230) of these patients were included. FDRs were questioned in terms of BD symptoms by phone. Pathergy test and CFV wall thickness measurement were performed among 111 FDRs who accepted the clinical assessment. Clinical assessment group were classified according to the criteria sets for BD. FDRs who did not meet the criteria sets and had at least one clinical finding in addition to oral aphthae(OA) were categorized as the "suspected BD". RESULTS: :We observed increased frequency of isolated BD manifestations in FDRs. Ten FDRs were diagnosed with BD during clinical evaluation. Significantly increased CFV wall thickness was observed in FDRs of BD patients fulfilling diagnostic BD criteria (p<0.001 for both sides) and also in those with suspected BD group (p<0.05 for both sides). Presence of OA, genital ulcer, folliculitis or erythema nodosum were associated with increased CFV wall thickness (p<0.05). CONCLUSION: . Our results suggest that CFV wall thickness measurement can be used in diagnosis of familial BD.

Impact of celiac disease in Behçet's syndrome patients: a study based on the database of Türkiye.

Background/aim: Our primary aim was to investigate the effects of concomitant celiac disease (CD) on the clinical characteristics of Behçet's syndrome (BS) patients. Materials and method: The study was a retrospective, nationwide, multicenter study. Turkish Ministry of Health National Electronic Database (e-Nabiz) is used under Health Ministry's supervision to extract the subject's data. Statistical analysis: Statistical analyses were made by the Statistical Package for Social Sciences (SPSS) software version 20 (IBM Corp., Armonk, New York). Continuous variables were presented by mean ± standard derivation (SD) or median (min-max) according to normality and compared by student-t test. A binary logistic regression analysis was performed to further investigating the relation between having a concomitant CD with each BD manifestation and comorbidity, frequencies of which were detected to be significantly different in the student-test. Results: A total of 84,241 patients diagnosed with BS were analyzed, and CD was identified in 175 (0.21 %) patients. The group with CD had a mean age of 41.30 ± 13.69 which was significantly younger. the prevalence of females was significantly higher (71.4%). The mean age of first admission for BS was also significantly younger in the group with CD (36.64 ± 13.28). BS patients with CD had a significantly higher prevalence of inflammatory bowel disease (27.2% vs. 7.3%, p < 0.001). When comorbid conditions were investigated depression (35.4% vs. 23.3%, p < 0.001), migraine (7.4 % vs. 2.6%, p < 0.001), fibromyalgia (10.9% vs. 4.5%, p < 0.001) and osteoporosis (12.6% vs. 6.6%, p = 0.001) were significantly more frequent in BS patients with CD. Conclusion: Our results suggest coexistence of CD in BS patients is related to female dominance and probably to an earlier disease onset. Several CD-related comorbidities as well as inflammatory bowel disease were more frequent in the CD group which implied an increased overall disease burden.

Effectiveness and safety of adalimumab in patients with intestinal Behçet's disease: a real-world prospective observational study in South Korea.

BACKGROUND: Intestinal Behçet's disease (BD) is characterized by typical gastrointestinal ulcers in patients with BD followed by complications such as bleeding, perforation and fistula. Biologic agents are currently under active investigation to delay the disease course. Various data regarding infliximab are available, but there is relatively lack of data regarding adalimumab. METHODS: This was a multicenter, real-world prospective observational study to evaluate the effectiveness and safety of adalimumab in intestinal BD. The primary endpoint was disease activity at each follow up, including disease activity index for intestinal Behçet's disease (DAIBD), serum C-reactive protein (CRP) level, and endoscopic findings. The secondary endpoint was the incidence of adverse drug reactions (ADRs). RESULTS: A total of 58 patients were enrolled and 8 of them were excluded. Adverse events were reported in 72.0% of patients with 122 events. ADRs were reported in 24.0% with 28 events. For adverse events, arthralgia was most commonly reported (13.1%: 16/122) and only one experienced critical adverse event (0.82%, 1/122: death due to stroke). On multivariable regression analysis, a longer disease duration was significantly associated with decreased ADRs [Odds ratio 0.976 (0.953-0.999, 95% CI); p = 0.042]. Clinical response rates as assessed by DAIBD were 90.9% at Week 12 and 89.7% at Week 56, respectively. The mean serum CRP level at baseline was significantly decreased after 12 weeks (3.91 ± 4.93 to 1.26 ± 2.03 mg/dL; p = 0.0002). CONCLUSION: Adalimumab was found to be safe and effective in Korean patients with intestinal BD. A longer disease duration was significantly associated with decreased ADRs.

Thrombotic manifestations in pediatric Behcet syndrome: A multicenter comparative study from the EUROFEVER registry.

INTRODUCTION: Vascular events account for a considerable burden of morbidity and mortality in Behçet syndrome (BS). Thrombosis occurs in 1.8-21 % pediatric BS patients, even if the real prevalence is still largely unknown. OBJECTIVES: To report clinical features and outcomes of pediatric BS patients with thrombosis and to compare the demographic and clinical characteristics of BS patients with and without thrombosis. METHODS: Retrospective data collection of BS patients with thrombosis (T+) included in the EUROFEVER registry. BS patients without thrombosis (T-), belonging to the same rheumatology units, were matched in a 2:1 ratio. RESULTS: 37 T+ were compared to 74 T- patients. At onset, ICBD criteria fulfillment was higher in the T- group (p = 0.015). Caucasian patients were more often T-, Turkish patients were more frequent in T+ group (p = 0.002). At onset, pustulosis was most frequently observed in the T- (p < 0.001) as well as gastrointestinal symptoms (p < 0.001) and ocular involvement (p = 0.022). Neurological symptoms were more often described in T+ (p = 0.034). As for T+, thrombosis was reported at BS presentation in 8/37 (21.6 %). For the T + e patients who developed thrombosis later, oral aphthosis (p = 0.003), genital aphthosis (p = 0.014) were more frequently observed at BS onset, while pustulosis (p = 0.005) and fever (p = 0.043) coexisted with thrombosis. Thrombosis was mainly venous (26/37,70.3 %), involving the cerebral sinuses (21/37, 56.8 %). After thrombosis, 35/37 (94.6 %) T+ patients received an immunomodulatory treatment compared with 16/29 (55.2 %) pre-thrombosis. A recurrence was reported in 6/31(19.4 %). CONCLUSION: Thrombosis was reported at BS presentation in one fifth of cases. Pustolosis and fever were more frequently concomitant to thrombosis. Sinus veins were the most frequent site.

Pseudotumoral neuro-behcet's disease: case series and review of literature.

BACKGROUND: Behcet's disease (BD) is a multisystem autoimmune relapsing vasculitis with an almost unknown etiology involving both large and small vessels. The neurological involvement called neuro-Behcet's disease (NBD) is rare. NBD can be responsible for tumor-like masses mimicking low-grade gliomas in only a few cases. METHODS: We report here the main characteristics, treatment, and outcome of 43 patients (4 personal cases and 39 patients from the literature) with a pseudotumoral presentation of NBD (PT NBD). We compared our findings with those of the classical form of NBD. RESULTS: The median age was 35.86 (12-59 years) years, with a male predominance (67.4%). PT NBD was the inaugural of the disease in 51.2% of cases. The neurological manifestations included headache (n = 31), pyramidal syndrome (n = 28), cerebellar syndrome (n = 5), behavioral changes (n = 5), and pseudobulbar signs (n = 2). Ophthalmologic examination revealed papilledema in 3 cases. On cerebral imaging, the most affected regions of the brain were the capsulothalamic region (n = 15, 37.5%) and the brainstem (n = 14, 35). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoral or infectious lesions. Treatment consisted of corticosteroids (n = 40, 93%) and immunosuppressive agents (n = 28, 65.11%), leading to complete clinical and imaging remission in 41.5% of patients. CONCLUSION: PT NBD is a rare but life-threatening condition.

Behçet's syndrome masquerading as infective endocarditis: A diagnostic conundrum and therapeutic challenge.

This case report presents a 20-year-old male patient initially diagnosed with infective endocarditis, later correctly identified as Behçet's syndrome. The patient's complex clinical presentation, including chest pain, aortic dilation, severe aortic regurgitation, and aortic root abscess, posed significant diagnostic and therapeutic challenges. Despite initial misdiagnosis and treatment difficulties, the patient's condition significantly improved with appropriate immunosuppressive therapy, underscoring the potential for successful management of this complex condition. This case serves as a valuable reminder of the diagnostic challenges posed by Behçet's syndrome and the importance of considering this condition in patients presenting with symptoms suggestive of infective endocarditis.

Gender disparities in Behçet's syndrome: identifying distinct phenotypes through cluster analysis.

Behçet's syndrome (BS) is a complex, multi-systemic disorder with a global occurrence, notably concentrated along the Silk Road. This study aimed to investigate gender-specific expressions and clinical phenotypes in BS patients within the Eastern Black Sea Region of Turkey. A total of 290 BS patients were retrospectively analyzed between January 2013 and December 2023. Demographic characteristics, clinical manifestations, medical treatment, and pathergy test results were obtained from a review of medical records. The mean age was 45.79 ± 13.05, with a male-to-female ratio of 48.6:51.4. Male patients had higher papulopustular lesions (p < 0.001) and ocular involvement (p = 0.036), while females showed more frequent genital ulcers (p = 0.032). Medication usage showed gender-based variations, notably higher corticosteroid, azathioprine, and tumor necrosis factor-alpha inhibitor (TNFi) use in males (p < 0.001). Cluster analysis revealed five distinct clusters, each with unique features and gender predominance. Cardiovascular type, ocular type, and skin type predominantly featured male patients, while joint involvement type and neurologic and mucosal involvement type were more prevalent among female patients with BS. This research contributes valuable insights into the gender-related clinical variations of BS within a specific geographic region, fostering a more comprehensive understanding of this challenging syndrome. The identification of distinct clinical phenotypes facilitates the development of tailored treatment strategies, potentially leading to improved outcomes for patients with BS.

Brain white matter microstructural alterations in Behcet's syndrome correlate with cognitive impairment and disease severity: A diffusion tensor imaging study.

OBJECTIVES: To evaluate the microstructural integrity of brain white matter tracts in patients with Neuro-Behcet's syndrome (NBS) and Behcet's syndrome (BS) without neurological manifestations using diffusion tensor imaging (DTI) and to investigate potential utility of DTI as a surrogate biomarker of neurocognitive functioning and disease severity. METHODS: This cross-sectional study comprised 34 NBS patients and 32 BS patients without neurological involvement, identified based on the International Study Group of the Behcet's disease (ISGBD) and the International Consensus Recommendation (ICR) criteria, as well as 33 healthy controls. Cognitive functions, including attention, memory, language, abstraction, executive control, visuospatial skills, and sensorimotor performance were assessed using standardized questionnaires. DTI data were analyzed using tract-based spatial statistics (TBSS) and automated probabilistic tractography to investigate inter-group differences. Subsequently, correlations between tensor-derived parameters of white matter tracts, neurocognitive test scores, and disease severity measures were examined. RESULTS: DTI revealed decreased fractional anisotropy and increased radial diffusivity, mean diffusivity, and axial diffusivity in both supratentorial and infratentorial white matter in NBS patients, indicating widespread loss of microstructural integrity. Moreover, this loss of integrity was also observed in BS patients without neurological manifestations, albeit to a lesser extent. In NBS patients, certain white matter tracts, including cingulum bundle, were associated with poor cognitive performance across multiple domains and disease severity. DISCUSSION: DTI findings might potentially serve as a neuroimaging marker to predict the extent of neurocognitive impairment and disease severity associated with central nervous system involvement in BS.

Deficiency of peripheral CLA+ Tregs and clinical relevance in Behcet's syndrome.

BACKGROUND: Autoimmune responses have been suggested to involvement in patients with Behcet's syndrome (BS). There has been growing attention towards the roles of cutaneous lymphocyte antigen (CLA)+ regular T cells (Tregs) in autoimmune diseases. The role of CLA+ Tregs in BS is still uncertain. This study aims to clarify the impact of CLA+ Tregs on BS. METHODS: We collected peripheral blood from a total of 107 patients with BS and 114 healthy controls (HCs). The number of CLA+ Tregs, natural killer (NK) cells, B cells, and several subtypes of CD4+ T cells were detected using flow cytometry and compared between patients and HCs. RESULTS: The absolute number and proportion of CLA+ Tregs among CD4+ T lymphocytes and CD4+ Tregs were lower in patients with BS than in HCs. CLA+ Tregs were positively related with NK cells (r = 0.500, P < 0.001) and B cells (r = 0.470, P < 0.001) and negatively related with effector T cells (r=-0.402, P < 0.001) in patients with BS. Patients with BS and arterial aneurysms had CLA+ Treg cell deficiency. A decreased proportion of CLA+ Tregs was associated with arterial aneurysms in patients with BS. The proportion of CLA+ Tregs in patients with BS increased with corticosteroids and immunosuppressants. CONCLUSION: CLA+ Tregs decrease in association with arterial aneurysm in patients with BS. CLA+ Tregs may be a predictor of response to BS treatment.

Successful splenic artery embolization in a patient with Behçet's syndrome-associated splenic rupture: A case report.

BACKGROUND: Splenic rupture associated with Behçet's syndrome (BS) is extremely rare, and there is no consensus on its management. In this case report, a patient with BS-associated splenic rupture was successfully treated with splenic artery embolization (SAE) and had a good prognosis after the intervention. CASE SUMMARY: The patient was admitted for pain in the left upper abdominal quadrant. He was diagnosed with splenic rupture. Multiple oral and genital aphthous ulcers were observed, and acne scars were found on his back. He had a 2-year history of BS diagnosis, with symptoms of oral and genital ulcers. At that time, he was treated with oral corticosteroids for 1 month, but the symptoms did not alleviate. He underwent SAE to treat the rupture. On the first day after SAE, the patient reported a complete resolution of abdominal pain and was discharged 5 d later. Three months after the intervention, a computed tomography examination showed that the splenic hematoma had formed a stable cystic effusion, suggesting a good prognosis. CONCLUSION: SAE might be a good choice for BS-associated splenic rupture based on good surgical practice and material selection.

Optimal Treatment Approaches to Intestinal Behçet's Disease Complicated by Myelodysplastic Syndrome: The KASID and KSBD Multicenter Study.

PURPOSE: Studies on intestinal Behçet's disease (BD) complicated by myelodysplastic syndrome (MDS) are rare, and no established therapeutic guidelines exist. This study aimed to evaluate the clinical presentation and outcomes of patients with intestinal BD complicated by MDS (intestinal BD-MDS) and suggest a treatment strategy. MATERIALS AND METHODS: Data from patients with intestinal BD-MDS from four referral centers in Korea who were diagnosed between December 2000 and December 2022 were retrospectively analyzed. Clinical features and prognosis of intestinal BD-MDS compared with age-, sex-matched intestinal BD without MDS were investigated. RESULTS: Thirty-five patients with intestinal BD-MDS were included, and 24 (70.6%) had trisomy 8. Among the 35 patients, 23 (65.7%) were female, and the median age at diagnosis for intestinal BD was 46.0 years (range, 37.0-56.0 years). Medical treatments only benefited eight of the 32 patients, and half of the patients underwent surgery due to complications. Compared to 70 matched patients with intestinal BD alone, patients with intestinal BD-MDS underwent surgery more frequently (51.4% vs. 24.3%; p=0.010), showed a poorer response to medical and/or surgical treatment (75.0% vs. 11.4%; p<0.001), and had a higher mortality (28.6% vs. 0%; p<0.001). Seven out of 35 patients with intestinal BD-MDS underwent hematopoietic stem cell transplantation (HSCT), and four out of the seven patients had a poor response to medical treatment prior to HSCT, resulting in complete remission of both diseases. CONCLUSION: Patients with intestinal BD-MDS frequently have refractory diseases with high mortalities. HSCT can be an effective treatment modality for medically refractory patients with intestinal BD-MDS.

Short- and long-term outcomes of surgical treatment in patients with intestinal Behcet's disease.

BACKGROUND: Behcet's disease (BD), a chronic vasculitic disorder affecting multiple organs, is characterized by recurrent oral and genital ulcers, arthritis, vasculitis, and intestinal ulcers. Although intestinal involvement of BD is common in East Asia, the efficacy and long-term outcomes of surgical treatment of intestinal BD still remain to be established. AIM: To evaluate the postoperative clinical course of intestinal BD and determine factors associated with its recurrence. METHODS: Data from patients who underwent surgical treatment for intestinal BD between January 2010 and August 2021 were retrospectively reviewed. Patients' demographics, clinical features, postoperative course, complications, and follow-up data were evaluated. RESULTS: We analyzed 39 surgeries in 31 patients. The mean patient age was 45.1 years, and the mean interval between the diagnosis of intestinal BD and surgical treatment was 4.9 years (range 1.0-8.0 years). The most common indication for surgery was medical intractability (n = 16, 41.0%), followed by fistula or abscess (n = 11, 28.2%). Laparoscopic approaches were used in 19 patients (48.7%), and 5 patients (12.8%) underwent emergency surgeries. The most common surgical procedure was ileocecal resection (n = 18, 46.2%), followed by right colectomy (n = 11, 28.2%). A diverting stoma was created in only one patient (2.6%). During a mean follow-up period of 45 (range 8-72) months, eight cases (20.5%) of recurrence in five patients required reoperation. The interval between operations was 12.1 months (range 6.3-17.8 mo). Four patients (10.3%) experienced recurrence within 1 year postoperatively, and all eight recurrences occurred within 2 years of the initial surgery. The reoperation rates at 1 and 3 years were 10.3% and 20.5%, respectively. A redo ileocolic anastomosis was performed in all recurrent cases. In multivariate Cox regression analysis, emergency surgery [hazard ratio (HR) 9.357, 95% confidence interval (CI): 1.608-54.453, P = 0.013] and elevated C-reactive protein (CRP) levels (HR 1.154, 95%CI: 1.002-1.328, P = 0.047), but not medication use, were predictors of recurrence. CONCLUSION: Surgical resection is a feasible treatment option for complicated BD. Reoperation is associated with severe inflammatory conditions, reflected by increased CRP levels and the requirement for emergency surgery.