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جستجوی مقالات مرتبط با کلیدواژه "behcet's disease" در نشریات گروه "پزشکی"

  • Simin Jahani, Neda Sayadi, Elham Rajaei
    Background

    Behçet's disease (BD) can severely impact the psychosocial and emotional aspects of patients, significantly disrupting their quality of life.

    Objectives

    The purpose of this study was to describe the psychosocial and emotional experiences of patients with BD and their coping strategies.

    Methods

    This study was conducted using a qualitative method with a hermeneutic phenomenological approach. Data were collected through unstructured and in-depth interviews (9 conducted face-to-face and 6 via WhatsApp video calls) until data saturation was reached. Face-to-face interviews took place at the rheumatology clinic and the rheumatologist’s office from April to July 2023. Data analysis was performed using Diekelmann's seven-step method. The trustworthiness of the study was ensured based on the criteria of Lincoln and Guba, as well as Sim and Sharp.

    Results

    By analyzing the data, 580 codes were extracted, leading to the identification of 12 sub-themes and 2 main themes. The first theme, "emotional, mental, and communicative instability," included the sub-themes of indecisiveness and uncertainty, moving away from desires and wishes, feelings of despair and demise, fears caused by the disease outcomes, sadness and mental anguish, isolationism, and reluctance to seek support. The second theme, "psychological and communicative strengthening strategies," comprised the sub-themes of having an occupation, seeking support, hoping for miracles and healing, doing mental exercises, and engaging in hobbies.

    Conclusions

    This study revealed numerous emotional, psychological, and communicative challenges faced by patients with BD, as well as the strategies they use to cope with these problems. It is recommended that the findings of this study be used to enhance the care team's understanding of the mental and communicative issues experienced by these patients and to improve their skills in teaching psychological strengthening strategies.

    Keywords: Behçet's Disease, Psychosocial Functioning, Emotional Depression, Coping Strategies, Qualitative Research
  • زهرا حسینی، سعید عابدیان*

    بیماری بهجت (Behcet’s disease:BD) یک اختلال التهابی سیستمیک مزمن و عود کننده است که عمدتا با زخم های مکرر دهان، یووئیت (التهاب چشم)، زخم های تناسلی و ضایعات پوستی مشخص می شود که با بهبودی و تشدید تظاهر پیدا می کند. آرتریت، ضایعات گوارشی، واسکولیت، اپیدیدیمیت، و ضایعات سیستم عصبی مرکزی نیز از دیگر تظاهرات در بیماران BD می باشد. این بیماری بیش تر در مناطق آسیایی مدیترانه ای و چین دیده می شود. سن شروع بیماری 20 تا 40 سالگی می باشد. گرفتاری چشمی یکی از علل عمده و مهم ناتوانی ناشی از این بیماری و یکی از عوارض مهم آن است، حتی احتمال ایجاد نابینایی کامل نیز وجود دارد. علائم چشمی بیماری بهجت در80-40 درصد موارد بیماری رخ می دهد. بهجت چشمی به صورت یووئیت (التهاب چشم) تظاهر می کند. یووئیت بیماری شایعی است که عمدتا جوانان را مبتلا می کند و در تعداد قابل توجهی باعث از دست دادن بینایی می شود. یووئیت یک بیماری التهابی چشمی شایع و تهدیدکننده بینایی است و شامل چندین عامل هتروژن بالینی است. شیوع انواع مختلف یووئیت به عوامل متعددی مانند سن، جنس، نژاد، توزیع جغرافیایی، عوامل محیطی، ژنتیک، محل آناتومیک فرآیند التهابی (یووئیت قدامی، میانی، خلفی، پان یووئیت)، هیستوپاتولوژی (گرانولوماتوز، غیرگرانولوماتوز)، نوع فرآیند التهابی (حاد، مزمن، عودکننده) و علت (عفونی، غیرعفونی) و عادات اجتماعی بستگی دارد. تحقیقات اپیدمیولوژیک یووئیت برای درک علت و ایمونوپاتوژنز این گروه از بیماری ها ضروری است. عوارض شایع چشمی تهدیدکننده بینایی یووئیت بهجت (BU) شامل ادم، غیرپرفیوژن، نئوواسکولاریزاسیون و آتروفی شبکیه و/یادیسک نوری می باشد. در بین مارکر های ژنتیکی متنوع، HLA ClassI، HLA-B*5 و زیر کلاس ان HLA-B*51 قوی ترین ارتباط را با بیماری بهجت دارد. اما نقش دقیق آن در یووئیت ایدیوپاتیک مشخص نیست. بسیاری از مطالعات ارتباط B*51 را با یووئیت تایید کردند. علاوه بر این، ژن های دیگری نیز در بروز بیماری نقش دارند. این بیماری با HLA-B5 و زیرگروه ان ژن B51، به ویژه آلل B*5101 مرتبط است. ارتباط HLA-B51 با تظاهرات مختلف BD متناقض گزارش شده است، که تا حدی به دلیل زمینه های مختلف منطقه ای و قومی در مطالعات متفاوت است. HLA-B27 شایع ترین نوع HLA در بیماران مبتلا به یووئیت به ویژه در یووئیت قدامی است و بیش تر پیش آگهی است زیرا بیماران را مستعد ابتلا به بیماری های عود کننده می کند. ژن های درگیر شامل ژن های بیان کننده اینترلوکین ها، کموکاین ها، گیرنده های کموکاین و فاکتور نکروز تومور و ژن های دخیل در سیستم کمپلمان هستند. توزیع جغرافیایی، اشکال خانوادگی، و منشا قومی بیماران مبتلا به بیماری بهجت (BD)، شواهد زیادی به نفع پیشینه ژنتیکی است. اگر چه BD معمولا به صورت پراکنده رخ می دهد، تجمع خانوادگی و شیوع بالاتری در خواهر و برادر و والدین بیماران BD مشاهده شده است. اگر چه پاتوژنز دقیق انواع یووئیت مشخص نیست، شواهد واضح و به دست آمده از این فرضیه حمایت می کند که یووئیت توسط عدم تعادل پاسخ ایمنی ناشی از فعل و انفعالات عفونی، تروما و عوامل محیطی در افراد با زمینه ژنتیکی خاص ایجاد می شود. در این فرآیند سلول های میلوئیدی ساکن بافت و لنفوسیت ها نقش مهمی در شروع و گسترش التهاب چشم بیماری دارند.

    کلید واژگان: بیماری بهجت, ژن های HLA, فاکتور ژنتیکی, پاتوژنز, یووئیت, یووئیت بهجت
    Zahra Hoseini, Saeid Abediankenari*

    Behçet's disease (BD) is a chronic multisystem inflammatory disorder characterized mainly by recurrent oral ulcers, ocular involvement, genital ulcers, and skin lesions, presenting with remissions and exacerbations. Arthritis, gastrointestinal lesions, vasculitis, epididymitis, and central nervous system lesions are also frequently observed disease manifestations in BD patients. This disease is more common in the Asian, Mediterranean, and China regions. The age of onset of the disease is 20 to 40 years. Eye involvement in uveitis is one of the main factors of disability caused by this disease and one of its important complications thatted to blindness. Ocular symptoms of Behcet's disease occur in 40-80% of the etiology of this disease. Ocular Behcet's manifests as uveitis (inflammation of the eye). Uveitis is a prevalent disease that mainly affects young people. It leads to a significant number of visual losses. Uveitis is a common, sight-threatening inflammatory ocular disease and includes multiple heterogeneous clinical entities. The prevalence of various types of uveitis depends upon multiple factors, such as age, sex, race, geographic distribution, environmental influence, genetics, and social habits. Epidemiologic research on uveitis is necessary to understand the etiology and immunopathogenesis of this group of diseases. The prevalence of different types of uveitis depends on several factors such as age, sex, race, geographical distribution, environmental factors, genetics, anatomical location of the inflammatory process (anterior, intermediate, posterior uveitis, pan uveitis), histopathology (granulomatous, non-granulomatous), type of inflammatory process ( acute, chronic, recurrent) and cause (infectious, non-infectious) and social habits. Common sight-threatening ocular complications of Behçet Uveitis (BU) include edema, non-perfusion, neovascularization, and atrophy of the retina and/or optic disc. Among various genetic markers, class I, HLA-B5, and its subclass B51 allele have been reported to have the strongest association with BD, but their roles in idiopathic uveitis are unclear. Many studies have confirmed the association of B*51 with uveitis. In addition to this gene, other genes also play a small role in the occurrence of the disease. BD is associated with the B51 subtype of the HLA-B5 gene, especially the B*5101 allele. The association of HLA-B51 with various manifestations of BD has been reported to be conflicting, partly due to different regional and ethnic backgrounds in various studies. HLA-B27 is the most common type of HLA in patients with uveitis, especially in the anterior uveitis, and it is mostly prognostic because it renders the patients prone to recurrent disease. Genes involved included genes expressing interleukins, chemokines, chemokine receptors, and tumor necrosis factor and genes involved in the complement system. The geographic distribution, the familial forms, and the ethnic origin of patients with Behçet's disease (BD) are many evidence in favor of a genetic background. Although BD usually occurs sporadically, familial aggregation and a higher prevalence in siblings and parents of BD patients have been observed. Although the exact pathogenesis of specific uveitis entities is not clear, accumulating evidence supports a hypothesis that uveitis is caused by an imbalanced immune response induced by an interplay of infectious, traumatic, and environmental factors in individuals with particular genetic backgrounds. In this process, the tissue-resident myeloid cells and the lymphocytes play an important role in both the initiation and the expansion of ocular inflammation.

    Keywords: Behcet's Disease, HLA, Genetic Factor, Pathogens, Uveitis, Behcet's Uveitis
  • سمانه حسینی*، علیرضا خبازی، مهران غفاری، سید حسین آقامیری

    مطالعه حاضر با هدف معرفی یک مورد بیماری نوروبهجت که در ابتدا به اشتباه با تشخیص سکته ایسکمیک حاد مغزی و سپس با تشخیص مالتیپل اسکلروزیس تحت درمان قرار گرفته، انجام شده است. بیمار مورد مطالعه آقای 29 ساله با شروع تحت حاد همی پارزی، دیزآرتری و آتاکسی است که ابتدا با تشخیص سکته ایسکمیک مغزی حاد و سپس با تشخیص حمله حاد مالتیپل اسکلروزیس تحت درمان قرار می گیرد، اما به دلیل عدم بهبودی به درمانگاه مالتیپل اسکروزیس دانشگاه علوم پزشکی تبریز مراجعه می کند. با گرفتن شرح حال دقیق از سابقه آفت های دهانی و تناسلی و رویت ضایعه تیپیک آبشاری در ساقه مغز در تصویربرداری رزونانس مغناطیسی، از نظر بیماری بهجت و درگیری ثانویه پارانشیم مغزی ارزیابی شده و بعد از تایید تشخیص، تحت درمان با اینفلیکسی ماب قرار گرفته و بهبودی بارز بالینی و تصویربرداری مشاهده می شود.

    پیامدهای عملی:

     با توجه به شیوع بیماری بهجت در خاورمیانه، هر بیمار با نقص فوکال عصبی و شرح حال و تصویربرداری غیرمعمول برای بیماری های شایع مثل سکته مغزی و مالتیپل اسکلروزیس، حتما تشخیص نوروبهجت در ذهن باشد.

    کلید واژگان: بیماری بهجت, بیماری نوروبهجت, سکته مغزی, مالتیپل اسکلروزیس
    Samaneh Hosseini*, Alireza Khabazi, Mehran Ghaffari, Seyed Hossein Aghamiri

    This study aimed to investigate a case of neuro-Behçet’s disease misdiagnosed as acute ischemic stroke or multiple sclerosis (MS). The studied case was a 29-year-old man with subacute onset of hemiparesis, dysarthria, and ataxia who was diagnosed as acute ischemic stroke at first. Due to no significant improvement, the patient was managed as an MS case, but he did not experience any improvements again. We noticed a history of oral and genital aphthous and cascade sign appearance in his brain MRI. Then, Behçet’s disease with secondary parenchymal involvement of brainstem was confirmed. The patient received infliximab, which resulted in clinico-radiological recovery.

    Practical Implications.

     Given the prevalence of Behçet’s disease in the Middle East, the possibility of its diagnosis should be considered in patients with atypical history or imaging for ischemic stroke or MS.

    Keywords: Behçet’s disease, neuro-Behçet’s disease, stroke, multiple sclerosis
  • Hamidreza Jamaati, Raha Eskandari, Babak Sharif-Kashani, Roodabeh Haghgoo, Farzaneh Dastan *

    Behçet's disease (BD) is a multisystem, progressive, and inflammatory disorder of unknown etiology. Vasculitis is believed to underlie various clinical manifestations of BD and is known to be one of the main causes of death due to BD, in cases of large vessel involvement. The current study is done in order to examine the effects of rituximab on the patient’s debilitating clinical manifestations, as a result of not responding to the standard treatment regimens. The present case is a 28-year-old female patient with BD associated vasculitis. She was referred to the respiratory referral center, chiefly complaining of intermittent episodes of massive hemoptysis. She had also recurrent oral and genital ulcers, and difficulty in walking, despite considering the common treatment approaches for BD. Our patient received two courses of rituximab in combination with intravenous methylprednisolone. Over six months follow-up period from the date of treatment initiation with rituximab, symptoms of BD such as recurrent hemoptysis and aphthous ulcers were reduced in both frequency and severity. Lower limb weakness and difficulty in walking were improved as well. To summarize, rituximab appears to be an effective alternative for treatment-resistant vasculitis in BD patients.

    Keywords: Behçet's disease, Hemoptysis, Refractory cases, Rituximab, Vasculitis
  • Yousef Almheirat *, Khadija Kaddar, Lamis Elyamani, Siham Dikhaye, Nada Zizi
    Introduction

    Genital ulcers areamongthe main manifestations of Behçet’s disease (BD); however, large severe acute genital ulcers are not common.

    Case Presentation

    A 29-year-old unmarried woman with a history of recurrent oral aphthae presented with a genital ulcer developed 2 weeks before admission. On clinical examination, there were two large painful ulcerations on the right labia majora, painful oral ulcerations, non-follicular pustules on the face and limbs, and painful edema on the left leg with positive Homan’s sign. The D-dimer level was elevated, and a Doppler ultrasound showed deep venous thrombosis of the right common femoral, femoral, and popliteal veins. The BD was diagnosed according to the International Criteria for Behçet’s Disease. The patient was treated with prednisone 40 mg/day and colchicine 1 mg/day, and the lesions developed well and healed within 2 weeks, leaving scarring lesions in the genital area.

    Conclusions

    Behçet’s disease is an important nonvenereal cause of genital ulceration. A high index of suspicion in a patient with mucocutaneous lesions might result in the early diagnosis, management, and prevention of complications.

    Keywords: Genital Ulcer, Behçet’s Disease, Brutal Genital Ulcer
  • Zahra Heidari Sharifabad, Fahimeh Hajiabolhassan, Reza Hoseinabadi, Seyedeh Tahereh Faezi, Shohreh Jalaie
    Background and Aim

    Behcet’s disease (BD) is a multisystemic, chronic and progressive disorder with a relatively high prevalence in Iran. Therefore, this study aimed to compare the vestibular function between Iranian BD patients and normal subjects using cervical vestibular- evoked myogenic potentials (cVEMPs) and video head impulse test (vHIT).

    Methods

    In this cross-sectional study, 44 patients with definitive BD in the inactive stage of disease and 30 age and sex matched normal subjects were evaluated via cVEMP and vHIT tests and dizziness handicap inventory (DHI). Then the parameters of the tests were compared between the two groups by statistical methods. Moreover, the effects of DHI scores and other contextual variables on the test results were examined.

    Results

    In terms of the cVEMP test, the response rate and mean latency of p13 in the left ear were significantly lower, the amplitude of the cVEMP wave in the left ear and the amplitude asymmetry ratio were significantly higher in BD patients compared to normal subjects (p<0.05). Fifteen BD patients had abnormal amplitude and nine patients had abnormal latency of cVEMP responses. In terms of the vHIT test, the mean vestibule-ocular reflex gain of the left posterior semicircular canal and the mean gain asymmetry ratio of lateral canals were significantly higher in BD patients than in the controls (p<0.05).

    Conclusion

    the vestibular system of some patients with BD is disturbed and the results of this study indicate the presence of unilateral vestibular weakness in BD. So vestibular assessment can be helpful for these patients.

    Keywords: Cervical vestibular evoked myogenic potentials, video head impulse test, saccule, semicircular canals, behcet’s disease
  • Maryam Masoumi, Alireza Sharifi, Sepideh Rezaei, Sima Rafiei, Hossein Hosseinifard, Saghar Khani, Maryam Doustmehraban, Mona Rajabi, Zahra Beiramy Chomalou, Parisa Soori, Akbar Javan Biparva, Afsaneh Dehnad, Fatemeh Pashazadeh Kan, Ahmad Ghashghaee*
    Background

    Behcet’s disease is a chronic fatal illness with a relapsing remitting nature and significant organ-threatening morbidity and mortality. The aim of this research was to examine studies which were conducted on investigation of prevalence of quality of life among patients with Behcet’s disease.

    Methods

    A total of 13 articles were extracted from four main databases including PubMed, EMBASE, Scopus, and Web of Science from the onset of 2000 to January 2021. All studies published in English with the purpose of examining quality of life (QOL) among patients with BD or investigating its main determinants were included.

    Results

    Totally, 1137 BD patients participated in 13 studies. Based on random effect analysis, the total score of physical health-related QOL was 46.7 (95% CI=41.26 to 52.13) and the total score of mental health-related QOL was 49.01 (95% CI=43.83 to 54.18) representing a moderate level of QOL among BD patients. Furthermore, weighted effect size analyses showed a significant correlation between QOL and variables such as patients’ age, gender, disease duration and depression (pvalue: 0.00).

    Conclusion

    As the symptoms of BD worsen over time, patients confront with more severe body pain, mobility restrictions, and difficulties in chewing, eating, speaking and swallowing which negatively affect social interactions of patients and reduce their QOL. Furthermore, depression was proved to act as a deteriorating factor for HRQOL among BD patients. Thus, patients need to be psychologically supported by a specialized team and be informed during the course of treatment to gain useful information about the disease, treatment approaches and coping strategies.

    Keywords: Global, health-related quality of life, Behcet’s disease, systematic review
  • Alireza Khabbazi, Ali Reza Shafiee-Kandjani *, Hadise Kavandi, Morteza Asadi, Maryam Panahzadeh
    Background

    Behcet’s disease (BD) is a chronic disease with adverse effects on sexual function and some likely effects on marital satisfaction, thereby posing some problems in family dynamics.

    Objectives

    This study aimed to determine the effects of BD on sexual function in females and males and determine the relationship between the clinical manifestations of BD and sexual function.

    Methods

    Fifty-one patients with BD and 50 healthy controls were included in this study. Inclusion criteria were: being married, aged 18 - 55 years, being sexually active, and having sex during the past four weeks. Sexual function was assessed for the groups using the female sexual function index (FSFI) for women and the international index of erectile function (IIEF) for men.

    Results

    Sexual dysfunctions were observed in 82.9% of the females with BD and 14.3% of the healthy females. The total score of sexual performance and its subscales were significantly lower in the female patients with BD than the controls. No significant difference was observed between the males with BD and the controls regarding the total score of the IIEF and its subscales.

    Conclusions

    Sexual dysfunction in BD patients seems to pose a significant problem; hence, it is recommended to be assessed by physicians during routine examinations.

    Keywords: Behcet’s Disease, Female Sexual Function Index (FSFI), International Index of Erectile Function (IIEF), Sexual Dysfunction
  • Sara Vossoughian, Seyedeh Tahereh Faezi *, Farhad Shahram, Hoda Kavosi, Mostafa Qorbani, Mohammad Nejadhosseinian, Hoda Haerian, Yasaman Ahmadzadeh, MohammadAli Mozaffari, Fereydoun Davatchi

    Behçet’s disease (BD) is a chronic multisystem disorder with the principal pathological finding of vasculitis, which may involve vessels of different sizes. The concurrence of small and large vessel involvement in BD patients is undetermined. The current study aims to evaluate small vessel involvement in BD patients with large vessel involvement. The study population comprised 35 BD patients with large vessel involvement (cases) and 35 BD patients without large vessel involvement (controls). Small vessel involvement was evaluated in all patients by capillaroscopy. Capillaroscopic findings were compared between the two groups. According to the capillaroscopic findings, all of our BD patients had small vessel involvement. The most prevalent abnormality was tortuosity (87.1%), followed by enlarged loops (58.6%) and avascular areas (51.4%). Capillaroscopy findings between the case and the control groups were not statistically different. There was a significant association between microbleeding and history of erythema nodosum (P-value = 0.015), tortuosity and a history of skin aphthosis (P-value = 0.015), architectural derangement and history of uveitis (P-Value = 0.029), the number of avascular areas and active oral aphthosis (P-value = 0.021), and architectural derangement and increased ESR (P-value = 0.011). There was no difference in nail fold folder involvement between BD patients with and without large vessel involvement; however, some capillaroscopic features were associated with some disease manifestations.

    Keywords: Behçet’s disease, Vascular involvement, Nail fold capillaroscopy, vasculitis
  • Leila Alizadeh Ghavidel, Masood Bagheri*, Farideh Mousavi, Leila Rezaei, Somayyeh Hazeri, Hesam-Sadat Hashemi
    Purpose

    To investigate the pattern of ocular involvement in Behcet’s disease (BD) with predictors of patients’ final state of vision.

    Methods

    This historical cohort encompassed the clinical records of 200 patients diagnosed according to the International Criteria for BD (ICBD), over a period of 17 years between 2004 and 2021.

    Results

    The prevalence of Behcet’s uveitis (BU) was more common in females and patients in the fourth decade of life. Ninety-five patients (47.5%) had evidence of ocular involvement in the initial ophthalmologic evaluation, and 171 patients (85.5%) manifested evidence of BU during the follow-up visits of which bilateral non-granulomatous panuveitis was the most common anatomical pattern of involvement (32.9%) followed by posterior (27.6%), anterior (26.5%), and intermediate (13.8%) uveitis. The prevalent accompanying signs were oral aphthous (67%), skin lesions (29%), and genital ulcers (19.5%). Cystoid macular edema (CME) was the most frequent ocular complication (62%), followed by cataract (57.5%) and epiretinal membranes (ERM) (36.5%). Univariate analysis showed the following determinants: male gender, younger age at onset, panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis as poorer visual prognostic factors of the disease. Multivariate analysis demonstrated a higher chance of poor visual prognosis of BD in patients with panuveitis, posterior uveitis, retinal vasculitis, and longer duration of uveitis.

    Conclusion

    This cohort study demonstrated an overview on epidemiological patterns of BU along with the visual prognostic factors in Iranian patients.

    Keywords: Behcet’s Disease, Behcet’s Syndrome, Behcet’s Uveitis, Iran, Prognosis, Uveitis
  • Seyedeh Maryam Hosseini, Negar Morrovatdar, Armin Hemati, Mozghan Dolatkhah, Elham Bakhtiari, Zahra Mirfeizi, Mahdieh Azimizadeh*
    Purpose

    To assess the subfoveal choroidal thickness (SFCT) in patients with Behçet disease (BD) and compare the SFCT in patients with and without ocular BD (OBD) and between patients with active and quiescent phases of the Behçet’s posterior uveitis.

    Method

    This prospective cross-sectional study was conducted on patients with BD (n = 51) between October 2016 and October 2018. Complete ocular examinations including slit lamp biomicroscopy and fundus examination with dilated pupils were performed for all patients. The SFCT values were compared between patients with and without OBD. Enhanced depth imaging optical coherence tomography (EDI–OCT) was done to measure the SFCT, and wide field fundus fluorescein angiography (WF-FAG) was performed to evaluate the ocular involvement and determine the active or quiescent phases of the Behçet’s posterior uveitis. The correlation between the changes of SFCT and the WF-FAG scores was assessed.

    Results

    One hundred and two eyes of 51 patients with BD, aged 29 to 52 years were studied. Of these, 23 patients were male. The mean age ± standard deviation in patients with OBD and patients without ocular involvement was 38.71 ± 7.8 and 36.22 ± 10.59 years (P = 0.259) respectively. The mean SFCT in patients with OBD was significantly greater than in patients without OBD (364.17 ± 93.34 vs 320.43 ± 56.70 µm; P = 0.008). The difference of mean SFCT between the active compared to quiescent phase was not statistically significant when only WF-FAG criteria were considered for activity (368.12 ± 104.591 vs 354.57 ± 58.701 µm, P = 0.579). However, when the disease activity was considered based on both WF-FAG and ocular exam findings, SFCT in the active group was higher than the inactive group (393.04 ± 94.88 vs 351.65 ± 58.63 µm, P = 0.060). This difference did not reach statistical significance, but it was clinically relevant.

    Conclusion

    Choroidal thickness was significantly increased in BD patients with ocular involvement; therefore, EDI-OCT could be a noninvasive test for evaluation of ocular involvement in patients with BD. The increased SFCT was not an indicative of activity in OBD; however, it could predict possible ocular involvement throughout the disease course.

    Keywords: Behçet’s Disease, Behçet’s Uveitis, Choroidal Thickness, Enhanced Depth ImagingOptical Coherence Tomography (EDI-OCT), Ocular Behçet, Wide Field Fluorescein Angiography
  • Farhad Shahram, Saied Ghorbani, Mahdi Mahmoudi, Maassoumeh Akhlaghi, Zohreh Jadali, Vahideh Imeni, Mehdi Norouzi, Seyedeh Tahereh Faezi, Seyed HamidReza Monavari, Shaya Mostafaei, Vahdat Poortahmasebi, Shahram Barfi, Seyed Moayed Alavian, Seyed Mohammad Jazayeri *
    Background

     Behcet's disease (BD) is a chronic multisystem vasculitis with an unknown etiology. During the past years, several reports are published on the occult hepatitis B infection (OBI), the presence of hepatitis B virus (HBV) DNA in the absence of HBsAg, in rheumatic diseases.

    Objectives

     The current study aimed to, firstly, investigate the prevalence of OBI in patients with BD, and, secondly, its potential association with the clinical and therapeutic status of BD.

    Methods

     HBV serological markers and HBV DNA were evaluated in 220 consecutive BD patients to detect OBI. Demographic and clinical data of OBI positive and negative groups were compared.

    Results

     The mean age of patients was 39.24 (± 10.57), and 134 (62.9%) were male. The mean disease duration was 14.13 (± 8.63) years. No HBsAg positive case was found, but HBV DNA was found in 19 (8.6%) patients. The median viral load value was 475.84 copy/mL. We compared clinical data of 10 OBI positive and 156 OBI negative BD patients with complete and accessible data. There was no difference between the two groups concerning demographic characteristics (age, sex, and disease duration), different clinical manifestations, or types of medications (immunomodulatory, cytotoxic, and corticosteroids).

    Conclusions

     This is the first study showing a rather high prevalence of OBI among BD patients. We did not find any correlation between OBI positivity and different clinical manifestations, medications, or HLA-B51. Further studies are needed on a larger group of patients and by molecular HBV evaluation (as well as serologic) regarding this possible association.
     

    Keywords: Occult Hepatitis B Infection, Behcet's Disease, Hepatitis B Virus Reactivation
  • Nasrin Zand*, Leila Ataie-Fashtami, Parvin Mansouri, Mohsen Fateh, Afshan Shirkavand
    Introduction

    Behçet’s disease (BD) is a debilitating and chronic vasculitis which can affect multiple organs. Recurrent oral aphthosis is the most common clinical feature of BD. The remarkable pain associated with painful oral ulcers can exert a major impact on the patients’ quality of life. Relieving pain of these oral ulcers is an essential approach in the management of mucocutaneous lesions of BD.Non-thermal CO2 laser therapy (NTCLT) as a novel photobiomodulative approach has recently been used for significant and immediate pain reduction of some types of painful oral lesions such as recurrent aphthous stomatitis, pemphigus vulgaris, and so on. In this procedure by some considerations, the conventional CO2 laser is used as a non-surgical, non- thermal, photobiomodulative laser without any visible thermal adverse effects. In this article, we report our experiences about the analgesic effects of NTCLT on painful oral aphthous ulcers of BD.

    Case Presentation

    We report four cases of BD, whose painful oral aphthous ulcers were irradiated with NTCLT. Immediately after NTCLT, the pain of the lesions was significantly relieved with no visible complications.

    Conclusion

    The results of this study suggest that NTCLT could be proposed as a hopeful procedure for significant and instant pain relief of oral aphthous ulcers of BD without any visible thermal adverse effects.

    Keywords: Behcet’s disease, Non-thermal CO2 laser therapy (NTCLT), Oral aphthous ulcers, Pain relief, Photobiomodulation
  • İlyas Dündar *, Ensar Türko
    Background

    In this study, we aimed to evaluate computed tomography(CT) findings of peripheral pulmonary artery aneurysms(PPAA) associated with Behcet Disease(BD), Hughes Stovin Syndrome(HSS), and idiopathic origin.

    Methods

    Contrast-enhanced CT scans of the patients were retrospectively reviewed regarding PPAA. The patients with PPA were classified into BD, HSS, and idiopathic groups according to the etiology. The groups were compared for demographical features including age and gender, multiplicity, distribution thrombosis and accompanying pulmonary artery embolism(PAE), and deep venous thrombosis(DVT) history.

    Results

    A total of 30 PPAA (25.4±13.4 (11-62) mm) were detected in 10(2.3%) (mean age 39.8±22-1.0[8-73] years, female/male:3/7) among 4391 patients reviewed. In 7 patients multiple aneurysms were detected while in 3 a solitary lesion was seen. Most commonly lower lobes (right 8-left 8, 53.2%) involvement was observed. A thrombosis was detected within 19(63.4%) aneurysms. Among 10 patients with PPAA 4(40%) ones have BD, 2(20%) HS and 4(40%) idiopathic origin. In 5(50.%) patients there was accompanying PAE and 3 (30%) DVT history. Patients with BD nad HSS tended to have multiple lesions than with idiopathic origin. Accompanying PAE was observed in 2(100%) of HSS, 2(50%) BD, and 1(25%) patient in the idiopathic group. A DVT history was recorded in 2(100%) of HSS, 1(25%) BD. None of in the idiopathic group had a DVT history. The only rupture was observed in the HSS group.

    Conclusion

    Vasculitic diseases lead to PPAA, including BD and HSS are more likely to be associated with complications and additional morbid conditions than idiopathic processes.

    Keywords: peripheral pulmonary artery aneurysm, behcet’s disease, hughes stovin syndrome, pulmonary artery embolism, deep venous thrombosis, rupture
  • Hosein Eslami, Leila Alizadeh Ghavidel, AliReza Khabbazi, Homayun Dolatkhah, Mohammadreza Bonyadi, Kamal Nasiri, Niloofar Bonyadi, Sahar Khademnezhad*
    Introduction

    Behcet’s disease (BD) is a multi-systemic inflammatory disorder. Evaluating theproduction of cytokines such as interferon gamma,biomarkers such as heat shock protein-70(HSP70)is an important way to study the pathogenesis,development of BD. This study aimed tocompare the salivary level of interferon gamma,HSP70 between patients infected with BD andhealthy individual.

    Methods

    This case-control study was performed on 35 patients with Behcet’s syndrome,70 healthyindividuals as the control group,who were selected from those referring to the Department of OralMedicine of Tabriz University of Medical Sciences. The levels of interferon gamma,HSP70 weremeasured in the whole unstimulated saliva through enzyme-linked immunosorbent assay (ELIZA). Inorder to compare the quantitative variables between two groups,independent samples t-test or itsnonparametric equivalent,Mann-Whitney U test,was used in SPSS software version 16.0. In this study,a P value less than 0.05 was considered statistically significant.

    Results

    There was no significant difference between the study groups in terms of age,gender,aswell as salivary interferon gamma,HSP70 levels. Interferon gamma level was 15.16±3.38 pg,mg inthe case group,5.27±1.21 pg,mg in the control group,and salivary HSP70 level was found to be45.50±17 ng,mL,19.5±5.2 ng,mL in the case,control groups,respectively.

    Conclusions

    The results of this study showed that interferon gamma,HSP70 levels in patients withBehcet’s syndrome are high,can be evaluated as an important tool for the treatment,evaluationof disease development in future studies

    Keywords: Behcet’s disease, Interferon gamma, HSP70, Saliva
  • Elham Aflaki, Saman Farahangiz *, Alireza Salehi
    Background
    Behçet’s disease (BD) can negatively impact the quality of life (QoL) of the affected patients. The present study aimed to assess the QoL of BD patients using the Leeds BD-QoL and compare its results with the WHOQOL-BREF questionnaire.
    Methods
    In the present cross-sectional study, 179 BD patients and 304 healthy individuals (the control group) were recruited in Shiraz, 2017. The Persian version of the Leeds BD-QoL and WHOQOL-BREF questionnaires were used to assess the QoL of patients with BD. The QoL in the patient and control groups was compared after controlling the effect of some variables (age, sex, marital status, and educational level) using the multiple linear regression analysis. Spearman’s correlation coefficient was calculated for the Leeds BD-QoL and WHOQOL-BREF scores. Disease activity was measured using the Behçet’s Disease Current Activity Form. All the statistical analysis was performed using SPSS software (version 21.0). P<0.05 was considered statistically significant.
    Results
    The mean of the Leeds BD-QoL total score in the patient group was 12.3±8.7. The control group had significantly higher scores in the WHOQOL-BREF total score and the physical health and psychological health domains compared with the patient group; mean difference of 10.24, 10.8, and 4.62, respectively (P<0.001). The Spearman’s correlation coefficient for the Leeds BD-QoL score and WHOQOL-BREF total score and its domains (physical health, psychological health, social relationships, and environment) was -0.669, -0.713, -0.714, -0.536, and -0.550, respectively. The disease activity score was correlated with the Leeds BD-QoL score (r=0.361, P<0.001).
    Conclusion
    BD patients had a lower QoL than healthy individuals, specifically in the physical health and psychological health domains. An increase in disease activity and severity was associated with a reduced QoL. The Persian version of the Leeds BD-QoL questionnaire had an acceptable correlation with the WHOQOL-BREF questionnaire.
    Keywords: Behçet’s disease, Quality of life, Leeds BD-QoL
  • Seyedeh Tahereh Faezi, Mohammad Nejadhosseinian*, Farhad Shahram, Nahid Sadighi, Masoumeh Banihashemian, Pedram Paragomi, Fereydoun Davatchi
    Background

    Osteonecrosis is death of bone tissue due to interruption of the blood supply. It can occur in autoimmune disease due to multiple mechanisms. It occurs rarely in some kind of multisystem disease such as Behcet’s disease (BD). The aim of this article is to present a case of BD with multifocal osteonecrosis.

    Case Presentation

    A 26-year-old woman with oral aphthosis, panuveitis, and retinal vasculitis was diagnosed to have BD, and oral treatment of prednisolone (0.5 mg/kg/day) together with azathioprine (2 mg/kg/day) and intravenous (IV) cyclophosphamide (CYC) (0.75 g/m2) was administered for her. Prednisolone was tapered to 15 mg/day after 3 months. Because of unresponsiveness of eye involvement to traditional immunosuppressive therapy, infliximab (5 mg/kg)was started for her and IV CYC was discontinued. 4 months after initial therapy, she complained of her right shoulder pain. Regarding to shoulder pain, magnetic resonance imaging (MRI) showed osteonecrosis of right shoulder. After diagnosis of osteonecrosis, prednisolone was discontinued by herself. 3 months later, she complained of both knees, both hips, and right ankle pain. According to previous diagnosis of osteonecrosis of herright shoulder, MRI was done for all involved joints, which revealed osteonecrosis of all of them. After diagnosis of multifocal osteonecrosis, the following tests were done that all results were normal: anticardiolipin antibody (ACA) [immunoglobulin G (IgG), immunoglobulin M (IgM)], lupus anticoagulant (LA), anti-beta-2 glycoprotein 1 (antiB2GP1) (IgG, IgM), coagulation tests, and lipid profiles.

    Conclusions

    Bone infarction should be considered in differential diagnosis of patients with joint pain and without the evidence of arthritis; however, it rarely occurs in patients with BD.

    Keywords: Behcet’s Disease, Osteonecrosis, Avascular Necrosis of Bone
  • Yasamin Pahlavan, Naser Samadi, Khalil Ansarin, Alireza Khabbazi*
    Purpose

    Survivin is critical for proliferation, maturation, homeostasis and differentiation of effector and memory lymphocytes. In this study the baculoviral inhibitors of apoptosis proteins (IAPs) repeat containing 5 (BIRC5) mRNA, survivin, and phosphorylated survivin expression were evaluated in peripheral blood mononuclear cells (PBMCs), and plasma of patients with Behcet’s disease (BD).

    Methods

    In this study, 26 Iranian Azari patients diagnosed with BD and 30 healthy controls were recruited. Total RNA was extracted from PBMCs. The expression level of survivin was measured by quantitative real-time polymerase chain reaction (PCR). Survivin plasma levels were measured using survivin Enzyme-linked immunosorbent assays. Also, western blotting analysis was performed to measure phosphorylated-survivin and survivin levels in PBMCs and plasma of patients with BD.

    Results

    In a pilot study, we showed that BIRC5 gene expression increased in BD patients compared with healthy controls (P<0.05). Western blotting analysis indicated that there was an increase in phosphorylated survivin expression in PBMCs of BD patients. Our data from western blot analysis showed survivin level in plasma samples of BD patients was similar to healthy controls. No significant differences were observed between plasma survivin levels in the BD patients compared with control group (P>0.05). The expression of phosphorylated survivin at Thr34 in PBMCs of BD patients with active disease was increased. Plasma phosphorylated survivin levels in having BD patients were also downregulated compared to healthy individuals.

    Conclusion

    Analysis of PBMCs indicated increasing expression level of phosphorylated survivin in PBMCs of BD patients. There was also a downregulation in phosphorylated survivin levels in plasma of BD patients.

    Keywords: Autoimmunity, Apoptosis, Lymphocytes, Inhibition of apoptosisproteins (IAPs), Behcet’s disease
  • Shahrzad Amizadeh, Nadereh Rashtchizadeh, Alireza Khabbazi *, Amir Ghorbanihaghjo, Ali Asghar Ebrahimi, Amir Mansour Vatankhahc, Aida Malek Mahdavi, Mohsen Taghizadeh
    Objective

    Behcet's disease (BD) is a chronic inflammatory disorder characterized by recurrent oral and genital aphthous ulcers, uveitis and skin lesions. Oxidative stress and inflammation have important role in the pathogenesis of BD. The aim of this study was to assess the effect of Nigella sativa (NS) oil administration on malondialdehyde (MDA), total anti-oxidant capacity (TAC), tumor necrosis factor-α (TNF-α), IL-10 and high sensitivity C-reactive protein (hs- CRP) levels in patients with BD.

    Materials and Methods

    In this randomized, double-blind and placebo-controlled clinical trial, 96 BD patients were randomly assigned to NS or placebo groups. Study groups received 1000 mg/day NS oil and placebo soft gels for 8 weeks. Serum levels of TNF-α, IL-10, hs-CRP, MDA and TAC were measured before and after treatment.

    Results

    Eighty-nine individuals completed the study. Significant decreases in the serum levels of MDA and increases in the serum levels of TAC were found in the NS group. However, differences in the changes of MDA and TAC in the NS and placebo groups were not significant. Pre- and post-intervention changes of TNF-α, IL-10 and hs-CRP levels in the NS group were non-significant.

    Conclusion

    NS 1000 mg per day is probably not effective in reducing the inflammatory and oxidative markers in BD.

    Keywords: Nigella Sativa, Behcet’s disease, IL-10, TNF-α, Oxidative stress
  • Nasrin Zand*, Parvin Mansouri, Samad Rezaee Khiabanloo, Mohsen Fateh, Leila Ataie Fashtami, Zahra Safaee Naraghi, Afshan Shirkavand

    Behçet’s disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology. Oral and genital aphthous ulcers are considered as the hallmarks of BD. The genital ulcers of BD may be extremely painful and often refractory to multiple treatments. In addition, they exert a negative impact on the patient’s quality of life. Some investigations have demonstrated the significant and immediate pain-relieving effects of NTCLT (non-thermal CO2 laser therapy) on some oral lesions with no visible adverse effects. In this paper, we report a case of BD whose painful genital ulcers were irradiated with NTCLT and the patient’s pain caused by the ulcers relieved immediately and significantly with no visible complications.

    Keywords: Behcet’s disease, Genital ulcers, CO2 laser, Non-Thermal CO2 Laser Therapy (NTCLT), Pain-relieving effects, Photobiomodulation, Low-level laser therapy
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