Influence of Pathergy Test on the Accuracy of Different Diagnosis Criteria for Behçet’s Disease

Author(s):  
F. Davatchi ◽  
F. Shahram ◽  
A. Nadji ◽  
A.R. Jamshidi ◽  
C. Chams ◽  
...  
2013 ◽  
Vol 23 (1) ◽  
pp. 125-132 ◽  
Author(s):  
Fereydoun Davatchi ◽  
Bahar Sadeghi Abdollahi ◽  
Cheyda Chams-Davatchi ◽  
Farhad Shahram ◽  
Zahra Ghodsi ◽  
...  

2013 ◽  
Vol 8 (2) ◽  
pp. 205-211
Author(s):  
Mehmet Sayarlioglu ◽  
Sevil Kamali ◽  
Ayse Cefle ◽  
Murat Inanc ◽  
Lale Ocal ◽  
...  

1993 ◽  
Vol 14 ◽  
pp. 31s
Author(s):  
F. Davatchi ◽  
F. Shahram ◽  
M. Akbarian ◽  
F. Gharibdoost ◽  
A. Nadji ◽  
...  

2012 ◽  
Vol 69 (2) ◽  
pp. 168-174 ◽  
Author(s):  
Gordana Zlatanovic ◽  
Svetlana Jovanovic ◽  
Dragan Veselinovic ◽  
Maja Zivkovic

Bacground/Aim. Behcet's disease is genetically conditioned, immune-mediated multisystem occlusive vasculitis of small blood vessels, espesially venules, of unknown etiology. The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. Methods. In this study symptoms and signs of the disease were analyzed both prospectively and retrospectively during the active manifestation of the disease. The diagnosis was reached according to the International Criteria for Behcet's Disease (2006). The treatment effects were evaluated based on the presence of the best corrected visual acuity and the inflammation of the vitreous humour before and after the application of our therapeutic method. The applied therapeutic modality consisted of the primary application of corticosteroid therapy in the active stage of the disease complemented with the choice of drugs from the immunosuppressive group. In this study there drugs were cyclosporine or methotrexate. A treatment refractory patients with poor vision prognosis were treated with a third drug, the biological preparation infliximab, a tumor necrosis factor-alpha (TNF-?) antagonist. Results. The mean age of 11 patients with ophthalmologic manifestation of Behcet's disease was 50.6 years. HLA B-5(51) was positive in 81% of the patients while 36% of the patients had positive pathergy test. Changes in affected eyes included vitritis (100%), posterior uveitis (45%), panuveitis (54%), retinal vasculitis (54%), cystoid macular edema (54%), and cystoid degeneration (18%). Increased intraocular pressure was observed in 27% of the patients. There was no statistically significant variation in disease activity parameters in any of the patients (p > 0.05). A statistically significant improvement in visual acuity (p < 0.05) and a high statistically significant decrease of inflammation of the worst affected eyes (p = 0.001) were detected. Conclusion. Our therapeutic method is useful for producing the optimal therapeutic plan for the acute - chronic stage of the difficult ophtamological manifestation of Behcet's desease as well as the prevention of relapse. However the high cost of the therapy and the potential complications should be taken into consideration when prescribing this therapy, especially a TNF-? antagonist.


2019 ◽  
Vol 18 (04) ◽  
pp. 175-181
Author(s):  
Yaşar B. Turgut ◽  
Ayşe F. Tosun ◽  
Beste K. Yüzbaşı ◽  
Hasan Tunca

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.


Author(s):  
З.C. Алекберова ◽  
Т.А. Лисицына ◽  
Р.Г. Голоева ◽  
А.В. Волков ◽  
Р.А. Осипянц ◽  
...  

Введение. Болезнь Бехчета (ББ) – системный васкулит неизвестной этиологии, характеризующийся рецидивами язвенного процесса в ротовой полости и на гениталиях, частым поражением глаз и рядом проявлений, включающих патологию суставов, желудочно-кишечного тракта, нервной системы и сосудов. Цель исследования: определить частоту и локализацию сосудистых проявлений у больных с ББ, уточнить связь последних с полом, возрастом и другой клинической симптоматикой ББ. Материалы и методы. Обследовано 250 больных ББ, сосудистая патология выявлена у 63 (25,2%) пациентов. Возраст больных с сосудистыми проявлениями составил 34,8 ± 10,4 лет, соотношение мужчины:женщины – 3,5:1. Сосуды вовлекались в процесс чаще в течение первых трех лет болезни. Поражалось в основном венозное русло, доля больных с артериальной патологией составила 9,5%. Результаты. У пациентов с сосудистой патологией по сравнению с больными без тромбозов достоверно чаще выявлялись узловатая эритема (71% против 44%; р = 0,002) и эпидидимит (22,4 % против 10,9%; р = 0,04), а генерализованный увеит (17,4% против 32%; р = 0,002) и ангиит сетчатки (22% против 40,6%; р = 0,008) встречались достоверно реже. В системе коагуляции у больных ББ с и без сосудистой патологии достоверных различий не выявлено. Заключение. Сосудистые нарушения выявлены у ¼ больных (преимущественно мужчин молодого возраста), развивались чаще в первые три года болезни и ассоциировались с узловатой эритемой, эпидидимитом и положительным тестом патергии. Introduction. Behcet's disease (BB) is a systemic vasculitis of unknown etiology; it is characterized by recurrent aphthous stomatitis, genital ulcers, uveitis and other manifestations including arthritis, pathology of gastrointestinal tract, nervous system and blood vessels. The aim of study: to determine frequency and localization of vascular manifestations in patients with BB, to clarify the relationship of manifestations with sex, age and other clinical symptoms of BB. Materials and methods. We examined 250 patients with BB, vascular pathology was revealed in 63 (25,2%) patients. The age of patients with vascular manifestations was 34,8 ± 10,4 years, the ratio of men:women was 3,5:1. Vessels were involved in the process more often during the first three years of the disease. Mainly veins were affected, arterial pathology had 9,5% of patients. Results. In patients with vascular pathology erythema nodosum (71% vs. 44%, p = 0,002) and epididymitis (22,4% vs. 10.9%; p = 0,04) were significantly more frequent than in patients without thromboses, and panuveitis (17,4% vs. 32%; p = 0,002) and retinal vasculitis (22% vs. 40,6%; p = 0,008) were found significantly more rarely. There were no significant differences in coagulation system between patients with and without vascular pathology. Conclusion. Vascular disorders were detected in ¼ of patients (predominantly in men of young age), developed more often in the first three years of the disease and associated with erythema nodosum, epididymitis and a positive pathergy test.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
R Cherfi ◽  
N Mahieddine ◽  
K Mammeri ◽  
S Touri ◽  
F Sadaoui ◽  
...  

Abstract Background Behçet's disease is a rare systemic vasculitis in children. The most frequent manifestations are orogenitalaphthosis and uveitis. Neurological, digestive, vascular, joint, and skin involvement are possible. We aimed to describe the clinical, diagnostic criteria, and therapeutic features of pediatric-onset Behcet's disease Methods Study carried out based on files collected over 12 years (2007–2018) concerning patients under the age of 15 hospitalized in the pediatric department of CHU de Blida for Behcet disease Results For 12 years (2007–2018), we hospitalized five children for various reasons and whose diagnosis of Behcet's disease was retained. These are 03 boys (2 of the theme are brothers) and 02 girls. The average age at admission was 13.5 years (12–15 years), and the average duration of follow-up is 4.2 years (15 months-8 years). The mean age of onset of first symptoms was 11 years, and the meantime to diagnosis was 2 years (12 months-3 years) The main diagnostic criteria were: mouth ulcers in five patients (100%), genital ulcers in three patients (60%), ophthalmic manifestations in two patients (40%): one had bilateral uveitis, and the other had papillitis, the pathergy test was positive in 3 patients (60%). The other manifestations were as follows: polyarthralgia in 3 patients (60%), family history in one patient (20%). Neurological manifestations were present in 3 patients (60%): one child presented two episodes of aseptic meningitis and thrombosis deep cerebral venous; one child with diffuse cerebral demyelination lesions, and the third with HIC syndrome and massive lateral sinus thrombosis. Three children have a vascular involvement (60%): pulmonary embolism with thrombosis of the superior vena cava (20%), bilateral thrombosis of the jugular veins (20%), and thrombosis of the right ventricle complicated by pulmonary embolism (20%) Initial symptoms included recurrent aphthous stomatitis (100%), arthralgia (60%), prolonged fever (20%), behavioral disturbances and dysarthria (20%), weight loss (40%), and headache (60 %). The histocompatibility antigen (HLAB 51) was found in 2 children (40%). The treatment included corticosteroids for all patients (3 boluses of 1 g/m2/day of methylprednisolone then relayed by prednisone 1 mg/kg/day), azathioprine 2.5 mg/kg/day in 5 children (100%), and colchicine for 2 patients only one patient had received anticoagulant therapy (LMWH followed by VKA) Relapses were observed in a single patient (20%) and mortality in one patient (20%.) Conclusion Behcet's disease is a rare disease whose diagnosis remains difficult in children. However, early diagnosis and aggressive treatment are mandatory to avoid complications and functional sequelae.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Melaku Getachew ◽  
Nebiyou Seyoum ◽  
Finot Debebe

Introduction. Behcet’s disease is a multisystem disease. In sub-Saharan Africa, the prevalence of this disease is not known, with only one case report from Ethiopia. Case Presentation. We describe a case of a 29-year-old Ethiopian male who presented to the emergency room of Tikur Anbessa specialized hospital with 4 days history of back pain, recurrent history of oral and genital ulcers, right eye blindness, chronic cerebral vein thrombosis, gastrointestinal bleeding, aortic aneurysm with dissection, and positive pathergy test. He is retrospectively diagnosed with Behcet’s disease according to both the International Criteria for Behcet’s Disease (ICBD) and the International Study Group (ISG) consensus. Conclusion. Even if Behcet’s disease is rare in sub-Saharan Africa, it is important to know the clinical presentation for timely diagnosis and urgent management.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Fereydoun Davatchi

Historical Background. The ISG criteria for Behcet's, created in 1990, have excellent specificity, but lack sensitivity. The International Criteria for Behcet's Disease (ICBD) was created in 2006, as replacement to ISG. The aim of this study was to compare their performance. ISG and ICBD Criteria. For ISG oral aphthosis is mandatory. The presence of any two of the following (genital aphthosis, skin lesions, eye lesions, and positive pathergy test) will diagnose/classify the patient as BD. For ICBD, vascular lesions were added, while oral aphthosis is no more mandatory. Getting 3 or more points diagnose/classify the patient as BD (genital aphthosis 2 points, eye lesions 2 points, and the remaining each one point). Performance and Comparison of ISG and ICBD. Their sensitivity, specificity, and accuracy (percent agreement), were tested in three independent cohort of patients from Far-East (China), Middle-East (Iran), and Europe (Germany). The sensitivity for ISG was respectively 65.4%, 78.1%, 83.7% and for ICBD 87%, 98.2%, and 96.5%. The specificity for ISG was 99.2%, 98.8%, 89.5% and for ICBD 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, 85.5% and for ICBD 88.9%, 97.3%, and 89.5%. Conclusion. ICBD has better sensitivity, and accuracy than ISG.


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