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Objectives: Post-thrombotic syndrome (PTS) is a frequent and important consequence of deep vein thrombosis (DVT) for Behcet`s disease (BD) patients. Although various clinical scales are used to diagnose PTS, Villalta scale was accepted as the standard tool to diagnose and grade the severity of PTS. Poor quality of life (Qol) in the general population was defined for patients with PTS, however, studies in BD patients with PTS is limited. Our aim was to compare the performance of different scales to assess venous disease in BD patients with a history of DVT and to assess the relationship with quality of life. Methods: Patients with BD ( n = 194, M/F:157/37, age:39.1 ± 9.5 years) with a DVT history were investigated. Villalta, VCSS,CEAP scale and SF 36,Veines scales were used to assess venous disease and QoL respectively. Results: Among BD patients, 120 (61.9 %) patients were classified as having PTS by Villalta and of patients 18% had severe PTS. Half of patients with CEAP score <4 were classified as having PTS. Also, 42% of patients with CEAP>4 and almost two third of VCSS classified severe CVD patients was grouped in severe PTS by Villalta scale. VCSS and Villalta classified PTS patients had decreased disease specific and general Qol scores compared to the patients without PTS. Also, severe PTS group (by VCSS) had decreased veines QoL scores and PCS compared to mild/moderate group. Conclusion: BD patients with DVT have a high risk of PTS. Our results show that both Villalta scale and VCSS should be used to assess venous disease BD patients with DVT. However, VCSS classified severity of PTS can show better correlation with venous disease -specific QoL., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To evaluate tocilizumab (TCZ) efficacy in patients with refractory Behçet disease (BD)., Methods: This is a multicenter study of 30 patients fulfilling the international criteria for BD and treated with TCZ at different European referral centers. The clinical response was evaluated at 6 months from TCZ initiation., Results: Ninety percent of patients with BD were refractory or intolerant to anti-tumor necrosis factor (anti-TNF) agents. Overall, TCZ was effective in 25 (83%) patients with BD of whom 18 (60%) and 7 (23%) were complete and partial responders, respectively. The complete response was 67%, 60%, and 42% in patients with uveitis (18/30), neurological manifestations (5/30), and mucocutaneous and/or articular (7/30) manifestations, respectively. TCZ had a significant steroid-sparing effect allowing patients to decrease their median daily prednisone dose from 20 (IQR 10-40) mg/day to 9 (IQR 5-13) mg at 6 months ( P < 0.001). The number of patients with BD needing concomitant disease-modifying antirheumatic drug therapy fell from 7 (23%) to 4 (13%) at 6 months. Mild to moderate side effects were observed in 6 (20%) patients, and 3 (10%) presented with serious adverse events (pneumonia, intestinal perforation, and septicemia) requiring therapy discontinuation in 2 cases., Conclusion: TCZ seems to be an effective alternative to anti-TNF agents in treating BD-related uveitis and neurological manifestations., (Copyright © 2023 by the Journal of Rheumatology.)

Objectives: The lower extremity venous wall thickness (VWT) of Behçet's disease (BD) patients was reported to be significantly increased in adults, suggesting its use for the support of BD diagnosis. This prospective study aimed to investigate the lower extremity VWT in childhood-onset definite and incomplete BD patients and compare it to healthy age-matched controls., Methods: Paediatric patients classified with BD according to the 2015 international paediatric BD criteria in our centre were included in the study. Intima-media thickness of the lower extremity veins to evaluate VWT was measured by ultrasonography, including common femoral vein (CFV), femoral vein (FV), vena saphena magna, vena saphena parva and popliteal vein (PV)., Results: In this cross-sectional study, VWT was measured in 35 patients (63% male) and 27 healthy controls (55% male). Thirteen (37%) of 35 patients met the criteria for the diagnosis of BD. The remaining 22 (63%) had incomplete BD and met two criteria. The median VWT values of both definite and incomplete BD patients were significantly higher than the control group in all veins on both sides. Regarding the best cut-off values of VWT for all lower extremity veins, the sensitivity rates were between 63% and 86%, while specificity rates were between 71% and 100%., Conclusion: Increased VWT was present not only in BD patients with vascular involvement but also in those without. We suggest that VWT may be a new criterion in supporting the diagnosis of childhood BD both in definite and incomplete BD patients., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Purpose: We sought to investigate alterations in the corneal subbasal nerve plexus and endothelium in patients with Behçet's disease (BD)., Methods: This cross-sectional study included 64 patients with BD and 30 age- and gender-matched healthy control subjects. Those with BD were classified as having ocular or non-ocular disease. All subjects underwent a corneal endothelial and subbasal nerve density evaluation using in vivo confocal microscopy (IVCM). The differences among groups were analyzed using the Kruskal-Wallis test followed by Dunn's multiple comparison procedure., Results: The mean age of study participants was 35.7 ± 10.2 years (16-58) in the ocular BD group, 39.6 ± 14.9 years (11-66) in the non-ocular BD group, and 34.1 ± 11.2 years (21-55) in the control group. No statistical significance was found in terms of age (p = 0.259) or sex (p = 0.560) between groups. The mean endothelial cell density determined with IVCM was 2124.9 [Formula: see text] 417.4 cells/mm 2 (1811-3275) in the ocular group and 2546 [Formula: see text] 335 cells/mm 2 (1798-3280) in the control group (p = 0.000). In the ocular group, the mean density of the subbasal nerve plexus was significantly lower (p = 0.004), and nerve tortuosity was significantly higher (p = 0.002)., Conclusions: Ocular BD could be responsible for changes in the corneal layers, especially endothelial and corneal nerve structures. Nerve density and tortuosity differences could be inflammatory indicators for BD., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is characterized by overlapping features of relapsing polychondritis (RP) and Behcet's disease (BD). To date, no studies have defined the clinical spectrum of disease in a cohort of patients with MAGIC syndrome., Methods: Adult patients within an ongoing prospective, observational cohort study in RP were clinically assessed for MAGIC syndrome. A systematic review was conducted to identify additional cases of MAGIC syndrome by searching four databases: PubMed (US National Library of Medicine), Embase (Elsevier), Scopus (Elsevier) and Web of Science: Core Collection (Clarivate Analytics). The inclusion criteria used were: [1] patients of any age or gender who were diagnosed with MAGIC syndrome, or both RP and BD; [2] case report or case series study; [3] published from 1985 - July 2020; and [4] in English language. Risk of bias was assessed using a checklist developed by the authors and based on the Consensus-based Clinical Case Reporting (CARE) Guidelines. Search results screening, article inclusion, data extraction and risk of bais assessment was performed independently by two investigators. Clinical characteristics, particularly BD-related features, were compared between patients with MAGIC syndrome and cases of non-MAGIC RP. The performance characteristics of different criteria to classify MAGIC syndrome were also evaluated., Results: Out of 96 patients with RP, 13 (14%) patients were diagnosed with MAGIC syndrome. For the systematic review, 380 articles were retrieved of which 90 were screened at title and abstract levels. Of these screened, 60 were excluded and 30 proceeded to full text review where an additional 8 were excluded. Twenty-two articles were included in our review and from which 27 additional cases of MAGIC syndrome were identified. Pooling all 40 cases together and comparing them with non-MAGIC RP, there was a significantly higher prevalence of ocular involvement (28% vs 4%, p<0.01), cutaneous involvement (35% vs 1%, p<0.01), GI involvement (23% vs 4%, p<0.01), and CNS involvement (8% vs 0, p = 0.04) in MAGIC syndrome. A higher prevalence of aortitis (23% vs 1%, p<0.01), Raynaud's phenomenon (54% vs 11%, p<0.01), and elevated anti-collagen II antibodies (50% vs 9%, p = 0.04) were observed in MAGIC syndrome. Fulfillment of either McAdam's or Damiani's Criteria for RP plus the International Criteria for Behçet's Disease had excellent sensitivity (98%) to classify cases of MAGIC syndrome., Conclusion: A substantial proportion of patients with RP can be clinically diagnosed with MAGIC syndrome. These patients have features of RP, BD, and other unique features including aortitis, Raynaud's phenomenon and elevated anti-collagen II antibodies., Competing Interests: Declaration of Competing Interest None., (Copyright © 2021. Published by Elsevier Inc.)

Background: Previous studies demonstrated an increased cancer risk in autoimmune diseases. Behçet's disease (BD) was also reported to be associated with an increased risk of cancer, although the data is limited., Aims: In this study, we aimed to assess cancer incidence in a large cohort of BD patients and to compare with the data of the same age and gender groups., Methods: The study cohort consisted of BD patients of > 18 years of age who were prospectively recorded in the Hacettepe University Vasculitis Center. Data on any cancer was collected from the patient files. Cancer incidence was compared with age- and gender-specific cancer incidence rates of the normal population retrieved from the 2014 Turkish National Cancer Registry (TNCR) data using standardized incidence rates (SIR)., Results: Totally, 451 adult cases with BD were included. The median age of the cohort was 43 (20-75), and 52.5% of the patients were males. Eleven cancer cases were observed during a median of 124 months follow-up. Behçet's disease was associated with an increase in cancer risk compared with expected counts in the corresponding age and sex group (SIR 2.84, 95% CI 1.50-4.94, p < 0.001). Patients with papulopustular lesions had a trend toward a decreased risk of cancer (p = 0.060), and patients using azathioprine had a significantly decreased cancer risk (p = 0.031)., Conclusion: This study revealed BD patients had approximately three times increased cancer risk compared with corresponding age and sex groups. Besides the routine care, increased attention for cancer surveillance is required in the follow-up of BD patients.

Behcet's syndrome (BS) is a variable vessel vasculitis with heterogeneous clinical features. Skin, mucosa and joint involvement can cause impairment of quality of life but do not cause permanent damage whereas untreated eye, vascular, nervous system and gastrointestinal system involvement can cause serious damage and even death. Management of BS as a multidisciplinary team enables a faster and more accurate diagnosis and well-integrated treatment strategies. Corticosteroids are the mainstay of therapy. Colchicine, AZA, ciclosporin-A, cyclophosphamide, IFN alpha, and tumour necrosis factor alpha inhibitors are other agents used as induction and/or maintenance therapy. Although biologic agents have been increasingly used, there are still unmet needs. Head-to-head comparison studies of some therapeutic options (e.g. TNF inhibitors vs IFN alpha in uveitis) are required. Novel therapeutic agents in the pipeline could change the standard of care for BS in the future., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background/aim: Neurological involvement (Neuro-Behcet's Disease: NBD) is a rare manifestation of Behcet's Disease (BD) and it is related with significant mortality and morbidity. We aimed to evaluate disease course and outcome of NBD patients registered in Hacettepe University Vasculitis Center (HUVAC) prospective database starting from October 2014., Methods: Totally, 419 patients (329 of the patients had fulfilled the International Study Group (ISG) criteria and 90 patients were considered as incomplete BD) were recorded as BD to March 2018. We retrospectively reviewed the charts of 123 patients with neurological complaints/ symptoms according International Consensus Recommendations (ICR) Criteria for Neuro-Behçet's disease. In final analysis, 77 NBD patients (Definite NBD = 61, possible NBD = 16) were included. Demographics, clinical features, treatment characteristics, disability status and survival status of the patients were evaluated., Results: Forty-seven (61%) of the patients were male. Median time to neurological involvement from first diagnosis of BD is 6 (IQR = 8.8) years in patients who had diagnosis of BD before neurological involvement. Distribution of NBD: parenchymal (pNBD), non-parenchymal (npNBD), mixed (mNBD) and peripheral nervous system (pnsNBD) were 47 (61%), 22 (28.6%), 5 (6.5%), 3 (3.9%), respectively. Eye involvement was more frequent in pNBD compared to npNBD. Brainstem (72.9%) was the most frequently affected parenchymal area and followed by cerebellum (43.8%) and diencephalon (37.5%). Twelve patients had spinal cord involvement (n = 12, 24.5%). Among the patients with pNBD and mNBD (total n = 52), 48 patients were considered as acute onset parenchymal disease and 4 patients were evaluated as chronic progressive disease. Fifty-eight percent of the patients with acute onset parenchymal disease had only one attack. Totally 14 BD patients deceased during a median 9.4 (IQR = 13) years disease duration and 9 of these patients had NBD (pNBD = 6, mNBD = 2, pnsNBD = 1). Corticosteroids (IV pulse = 75.5% and oral medium-high dose = 90%), alpha-interferon (76.9%), cyclophosphamide (57.1%), and TNF inhibitors (23.5%) were the most frequently preferred treatment options for pNBD., Conclusions: Neurological involvement is seen about 5 years after the diagnosis of BD, and ocular involvement more commonly seen in these patients than non-NBD patients. More than half of the patients with acute onset parenchymal NBD had only one attack. No death was observed in the patients with non-parenchymal NBD. Biologic agents (Interferon-alpha and anti-TNF agents) were used in most patients., Competing Interests: Declaration of Competing Interest The authors have received no financial support or other benefits from commercial sources for the work reported here, and the authors have no other financial interests that could create a potential conflict of interest or the appearance of a conflict of interest regarding the present study., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objectives: Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of vessels.The usage of TNF-α antagonists is increasing in different involvements of BD that is inadequately controlled by standard immunosuppressive regimens. Methods: Here we reported a rare BD case that is controlled by only infliximab (IFX) treatment. Results: A 24-years-old male patient was diagnosed as BD with recurrent oral and genital ulcerations,uveitis,femoral vein thrombosis and HLA-B5 positivity.He had had terminal ileitis,epididymitis,bilateral internal iliac artery aneurysm and superior sagittal sinus thrombosis after the diagnosis.He admitted to our outpatient clinic with dyspnea,orthopnea,chest pain and facial swelling.We detected facial edema,plethora,venous distention on neck and chest.We suspected vena cava superior syndrome(VCSS) and performed CT angiography that demonstrated high-grade vena cava superior stenosis and thrombus where SVC opens to the right atrium.Patient was anticoagulated with warfarin and a shunt was implanted between the left brachiocephalic vein and right atrium.Colchicine and interferon-alfa treatment were continued and 1mg/kg steroid was added.But the patients' symptoms relapsed one month later and his shunt was revised with balloon angioplasty.5mg/kg IFX was started.We  performed a CT-angiography annually for two years.VCSS and other symptoms of BD are resolved and have not relapsed yet. Conclusions: Vascular involvement of BD are generally treated with immunosuppressive agents such as steroids and immunomodulators.IFX was found effective and well tolerated in the treatment of intestinal,neurological and vascular involvement of BD especially in patients with poor response or intolerance to conventional therapy.This case report supports IFX as a new therapeutic option for patients with vascular BD.

Background: Behçet's disease (BD) is one of the reasons of acquired visual impairment among young adults. Ocular involvement is a significant cause of disability in BD. The objective of this study is to assess the prevalence of BD among a group of adults who has visual impairment., Methods: Ankara Metropolitan Municipality Education and Technology Center is one of the official institutions which records and follows the demographic data of visually impaired people in Turkey. In November 2014, there were 675 visually impaired people recorded at this center. Medical history was taken from 294 adults by phone in November and December of 2014. Participants were asked if the visual impairment had been either acquired or congenital. If the patients had BD or suspicious BD, they were recalled for detailed investigation which would be carried out by an internist, a rheumatologist and an ophthalmologist., Results: Two hundred thirteen of 294 (72.4%) visually impaired adults were male. One hundred nine of 294 (37.1%) had acquired visual impairment. Six (5.5%) of those 109 patients had BD. Overall prevalence of BD among study group was 2.04%.The median age of people with BD was 35 years. The median age at BD diagnosis was 16.5 years and the median duration from diagnosis to visual loss was 2.5 years., Conclusion: BD is still one of the causes of acquired visual impairment in Turkey. In this study, BD prevalence among a visually impaired adult group was 2.04%. BD accounted for 5.5% among adults who had acquired visual impairment. In a study of 1965, BD prevalence among people with acquired blindness was 12%. However, this study was conducted at pre-immunosuppressive period. Our prevalence is obviously lower than those studies. Extended population-based studies are needed for population estimations.

The immunosuppressive drugs are widely used in systemic involvements of Behçet's disease. This study is aimed to investigate the extent of clinical involvement and preferred treatment approaches for type of involvements in Behçet's patients from the whole country. All patients with the diagnosis of Behçet's disease were enrolled to the study. These patients analyzed whether they fulfill the International Study Group Criteria, and only those were further evaluated. Demographic and clinical characteristics, laboratory results and treatments ever used were recorded. Further analysis is done regarding clinical manifestations and preferred therapeutic approaches. A total of 863 patients with the diagnosis of Behçet's disease were detected, but 682 of them (female/male: 113/569) found to be appropriate for analysis. The remaining patients were included to the analysis. The frequencies of articular, ophthalmic and vascular involvement were 49, 43 and 21 %, respectively. Colchicine and corticosteroids were the most preferred agents. The immunosuppressive agents frequently used for organ involvements were azathioprine, cyclosporine A, interferon-α, sulphasalazine and cyclophosphamide with decreasing order of frequency. In this relatively young population composed from all over the country, the frequency of ophthalmologic, venous and neurological involvement is less frequent than previous reported cohorts. Azathioprine and cyclosporine were the drugs of choice as a chronic immunosuppressive agent in patients with organ involvement. The previously reported increased frequencies in other cohorts could be a result of the reference of severe patients to dedicated centers.

Angiogenesis is controlled by a variety of angiogenesis stimulators and inhibitors. The increased power Doppler (PD) signals determined by ultrasonography is an indirect marker of synovial vascularity in arthritis. We aimed to investigate relationship between ultrasonographic findings and synovial angiogenesis modulators. Thirteen Behcet's disease (BD), 15 spondyloarthropathy, 21 rheumatoid arthritis (RA), and 15 osteoarthritis (OA) patients with knee arthritis were included. Cumulative effusion, synovial hypertrophy, and PD signal scores were calculated in arthritic joints. In synovial fluid samples, angiogenesis inhibitors (angiostatin, thrombospondin-1, and endostatin) and stimulators [bFGF (basic fibroblast growth factor), angiopoietin-1] were studied. The comparisons between groups were made by Kruskal-Wallis test, and correlation analysis was calculated with Pearson and Spearman tests. Effusion scores were significantly higher in inflammatory arthritis than in OA. Synovial hypertrophy scores were higher in RA and spondylarthritis than in OA and BD. PD scores were not different between the groups. Synovial angiostatin and bFGF levels were significantly higher in patients with inflammatory arthritis than in OA. Cumulative effusion scores were positively correlated with angiopoietin-1, angiostatin, and bFGF and negatively correlated with thrombospondin-1 levels. Synovial hypertrophy scores were positively correlated with angiostatin and bFGF levels and negatively correlated with thrombospondin-1. No correlation was found between PD scores and modulators of angiogenesis. In large joints like knee, detecting PD signals alone was not sufficient to assess the angiogenesis. However, cumulative activity scores were positively correlated with angiogenesis stimulators. Therefore, when investigating the angiogenesis, PD technique should be added to gray-scale examinations.

Objectives: Subclinical cardiac involvement may occur in patients with Behçet's disease (BD). The purpose of our study was to assess the noninvasive parameters of biventricular function derived from Doppler tissue imaging (DTI) of the tricuspid and mitral annular motion in BD., Methods: Twenty-one patients with BD and 20 control subjects were enrolled in this study. All subjects were selected to exclude those with cardiovascular risk factors. Standard echocardiography and pulsed DTI were obtained in every patient., Results: Peak systolic (13.71 +/- 2.09 vs 20.01 +/- 1.57, P < 0.001), peak early diastolic (11.26 +/- 2.52 vs 15.35 +/- 2.06, P < 0.001) tricuspid annular velocities were significantly lower in patients than controls. Peak systolic (8.68 +/- 1.4 vs 12.25 +/- 1.7, P < 0.001), peak early diastolic (7.89 +/- 1.07 vs 9.94 +/- 1.12, P < 0.001), and peak end diastolic (8.30 +/- 1.32 vs 9.23 +/- 0.91, P = 0.013) lateral mitral annular velocities were significantly lower in patients than controls., Conclusions: We demonstrated that myocardial velocities, were affected in patients with BD. Therefore, we conclude that right and left ventricular function is impaired in patients with BD.