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Behcet’s Disease

A Bouarhroum, H Naouli, H Jiber
BACKGROUND: Behçet's disease (BD) is a multisystem chronic autoimmune inflammatory disorder that involves multiple organs. Arterial involvement in BD is rare. Aortic involvement is one of the most severe manifestations and is associated with a much higher mortality. In this article, we will present our experience in open surgical repair of abdominal aortic aneurysm combined with immunosuppressive therapy. METHODS: Between January 2010 and December 2017, 7 patients were treated for abdominal aortic aneurysms (5 infra-renal and 2 supra-renal, in 1 woman and 6 men)...
February 2019: Journal de Médecine Vasculaire
Janine Kemming, Emma Reeves, Katja Nitschke, Vanessa Widmeier, Florian Emmerich, Tobias Hermle, Emma Gostick, Andreas Walker, Jörg Timm, David A Price, Maike Hofmann, Robert Thimme, Edward James, Christoph Neumann-Haefelin
BACKGROUND & AIMS: Endoplasmic reticulum aminopeptidase 1 (ERAP1) polymorphisms are linked with human leukocyte antigen (HLA) class I-associated autoinflammatory disorders, including ankylosing spondylitis and Behçet's disease. Disease-associated ERAP1 allotypes exhibit distinct functional properties, but it remains unclear how differential peptide trimming in vivo affects the repertoire of epitopes presented to CD8+ T cells. The aim of this study was to determine the impact of ERAP1 allotypes on the virus-specific CD8+ T cell epitope repertoire in an HLA-B*27:05+ individual with acute hepatitis C virus (HCV) infection...
February 12, 2019: Journal of Hepatology
Justus G Garweg, Juerg Messerli
Acute posterior and panuveitis mostly affect younger patients and affect both eyes in more than half of cases. Because of the severe consequences in the clinical course, rapid and broad differential diagnosis are critical steps. Permanent loss of vision after a delay in starting therapy and the initiation of ineffective treatment are both serious risks. The initial diagnostic classification is based on clinical presentation (anatomical localisation and type of inflammation) and clinical course and, secondarily, on the response to acute therapy...
February 14, 2019: Klinische Monatsblätter Für Augenheilkunde
Lucien Chassin-Trubert, Giorgio Prousse, Baris Ata Ozdemir, Youcef Lounes, William Alonso, Myriam Clapiès, Pierre Alric, Ludovic Canaud
OBJECTIF: The aim of this paper is to report a case of filter associated inferior vena cava (IVC) thrombosis with perforation of the duodenum and penetration of a vertebral body by the filter struts. CASE REPORT: A 37 year old woman with a medical history of Behcet`s disease treated with corticosteroids, underwent placement of a retrievable IVC filter because of recurrent ileofemoral venous thrombosis regardless of therapeutic levels of anticoagulation. Despite a correct positioning of the filter, the second follow-up CT-scan, performed at 1 year, showed a complete thrombosis of the infra-renal IVC segment, with perforation of the vessel wall by the filter struts and penetration in the duodenum...
February 11, 2019: Annals of Vascular Surgery
Seohyuk Lee, Nicholas Czuzoj-Shulman, Haim Arie Abenhaim
Background Behcet's disease (BD) is a rare, multi-systemic inflammatory disorder for which only limited and contradictory data exists in the context of pregnancy. Our objective was to estimate the prevalence of BD in pregnancy and to evaluate maternal and fetal outcomes associated with pregnant women living with BD. Methods Using the 1999-2013 Healthcare Cost and Utilization Project-Nationwide Inpatient Sample from the United States, we performed a population-based retrospective cohort study consisting of pregnancies that occurred during this time period...
February 15, 2019: Journal of Perinatal Medicine
Christoph M Schempp, Franziska Schauer, Christian K Huhn, Nils Venhoff, Stephanie Finzel
Syndromes associated with concurrent skin and joint inflammation frequently pose a therapeutic challenge for both dermatologists and rheumatologists. In part 1 of this review, we discussed psoriatic arthritis as well as the autoinflammatory disorders SAPHO syndrome, Still's disease and Behçet's disease. Part 2 will address rheumatoid arthritis, reactive arthritis, Reiter's syndrome and Lyme borreliosis. In addition, we will discuss dermatomyositis and lupus erythematosus, two common autoimmune disorders that frequently present with both cutaneous and joint involvement...
February 2019: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Jing-Fen Ye, Jian-Long Guan
Background/aim: Differentiating intestinal Behçet’s disease (BD) from Crohn’s disease (CD) is highly challenging, as they often mimic each other in terms of clinical manifestations. Endoscopy is an important modality for distinguishing bowel lesions. The study was designed to identify clinical manifestations that are easily confused and to evaluate the efficacy of endoscopy for distinguishing intestinal BD from CD by several overlapping signs. Materials and methods: The data from 111 patients with intestinal BD and 81 patients with CD were retrospectively analyzed...
February 11, 2019: Turkish Journal of Medical Sciences
Ahmed M Abu El-Asrar, Nele Berghmans, Saleh A Al-Obeidan, Priscilla W Gikandi, Ghislain Opdenakker, Jo Van Damme, Sofie Struyf
PURPOSE: Evidence exists that the interleukin (IL)-10 family of cytokines is involved in autoimmune diseases. The aim of this study was to analyse the levels of the IL-10 family cytokines IL-10, IL-19, IL-20, IL-22, IL-26, IL-28A and IL-29 in aqueous humour (AH) samples from patients with specific uveitic entities. In addition, we correlated their levels with the levels of the proinflammatory cytokines tumour necrosis factor-α (TNF-α) and IL-1β. METHODS: Aqueous humour (AH) samples from patients with active uveitis associated with Behçet's disease (BD; n = 13), sarcoidosis (n = 8), human leucocyte antigen (HLA)-B27-related inflammation (n = 12), Vogt-Koyanagi-Harada (VKH) disease (n = 12) and control subjects (n = 9) were assayed with the use of a multiplex assay...
February 13, 2019: Acta Ophthalmologica
Fatma Alibaz-Oner, Rabia Ergelen, Aydan Mutis, Zeynep Erturk, Ruslan Asadov, Gonca Mumcu, Tulin Ergun, Haner Direskeneli
Vascular involvement, especially in young males, is seen in up to 40% of the patients with Behcet's disease (BD) and is a major cause of mortality and morbidity. In this study, we investigated vessel wall thickness (VWT) and dilatation in lower extremity veins with Doppler ultrasound (US) in male BD patients. Sixty-one male patients with BD, 37 healthy male controls (HC) and 27 male patients, with ankylosing spondylitis (AS), were included in the study. Venous Doppler US was performed by an experienced radiologist blinded to cases...
February 13, 2019: Clinical Rheumatology
Emire Seyahi
Behçet syndrome (BS) is a multi-systemic complex disorder with unknown etiology and a unique geographic distribution. It could not be possible to include it into specific classification schemes and it is certainly not a uniform disease. Several cluster and association studies revealed that it has been composed of multiple phenotypes ascribing the principal problem such as skin-mucosa, joint, eye, vascular, neurological and gastrointestinal involvement. Each phenotype has its own characteristic demographic and clinical features as such their management strategies and prognosis differ substantially...
February 11, 2019: Internal and Emergency Medicine
Maria Carmela Padula, Pietro Leccese, Nancy Lascaro, Teresa Carbone, Michele Gilio, Angela Anna Padula, Giuseppe Martelli, Salvatore D'Angelo
The endoplasmic reticulum aminopeptidase protein 1 gene (ERAP1) is related to several human diseases, including Behçet syndrome (BS), a multisystemic disorder with unknown etiology. ERAP1 is involved in immune response and its role can be influenced by gene single nucleotide variations (SNVs). We genotyped the ERAP1 whole structure in 50 consecutive BS patients and 50 ethnically-matched healthy controls using both bioinformatics and molecular methodologies. We identified two novel heterozygous missense SNVs of ERAP1 exon3 responsible for the p...
February 8, 2019: Human Immunology
Aysegul Sevim Kecici, Zekayi Kutlubay, Server Serdaroglu, Yalcin Tuzun
Pathergy phenomenon is a non-specific tissue hyperreactivity reaction due to trauma and is a minor diagnostic criterion of Behcet's disease. In this study, 100 patients with a suspicion of Behcet's disease who were referred to Cerrahpasa Medical Faculty Dermatology department between 01.11.2014 and 31.01.2015 are included. Skin pathergy tests were applied to all the patients and results were evaluated by two dermatologists separately at 48th hour, each with naked eye and with dermatoscopy. Test results were scored on a scale of 0-6...
February 8, 2019: Internal and Emergency Medicine
Doaa Hassan Sayed Attia, Rasha A Abdel Noor, Shaimaa Salah
OBJECTIVES: The frequency of different vasculitides and their characteristics vary among different regions. The identification of geographic disparities of disease phenotypes helps the development of international criteria, allowing the classification of patients of different ethnicities. This study aimed to describe the frequency, characteristics, course, response to treatment, and outcome of the different adulthood vasculitides in Egypt. METHODS: This was a multicenter study in which the medical records of adult Egyptian patients diagnosed with vasculitis between 2002 and 2018 were retrospectively reviewed...
February 9, 2019: Clinical Rheumatology
Fatmazohra Djaballah-Ider, Abdelhamid Djaballah, Zineb Djeraba, Samia Chaib, Chafia Touil-Boukoffa
BACKGROUND AND AIMS: Behçet disease (BD) is a chronic multisystem disease. It stands at the crossroads between the auto-immunity and auto-inflammatory disorders. Our study aims to evaluate corticosteroids therapy effects on serum immunoglobulin isotypes and anti-phospholipid auto-anti-body production in Algerian BD patients with different clinical manifestations. METHODS: We evaluated serum immunoglobulin isotypes and anti-phospholipid (anti-cardiolipin, anti-β2glycoprotein I, anti-prothrombin) auto-anti-body production using Turbidimetric or Luminex platform assays...
February 5, 2019: Inflammopharmacology
C Papadopoulou, E Omoyinmi, A Standing, C E Pain, C Booth, F D'Arco, K Gilmour, M Buckland, D Eleftheriou, P A Brogan
Objectives: Monogenic autoinflammatory disorders (AID) and primary immunodeficiencies can present early in life with features that may be mistaken for Behçet's disease (BD). We aimed to retrospectively describe the clinical and laboratory features of 11 paediatric cases referred for suspected BD who turned out to have an alternative, monogenic disease mimicking BD. Methods: Retrospective, paediatric BD specialist multicentre case series. Next generation sequencing (NGS) or conventional candidate gene screening approaches were utilized, facilitated in some cases by functional assays...
January 30, 2019: Rheumatology
Tomohiro Koga, Atsushi Kawakami
Autoinflammatory diseases are characterized by recurrent systemic inflammation due to dysfunction of the innate immune system, and they are originally the hereditary recurrent fever syndromes that develop in early childhood. Many cases are thus diagnosed in the pediatric field, but there are many cases that are not diagnosed until adulthood, including adult-onset cases. Accordingly, not only pediatricians but also rheumatologists and primary care physicians play a major role in the diagnosis and treatment of autoinflammatory diseases...
February 3, 2019: Immunological medicine
Sinan Emre, Suzan Güven-Yılmaz, Mahmut Oğuz Ulusoy, Halil Ateş
PURPOSE: Retinal vasculitis and vein occlusions are common causes of serious visual loss in Behçet's disease. We aimed to evaluate the optical coherence tomography angiography (OCTA) findings of Behcet uveitis (BU) patients. METHODS: We evaluated 32 eyes of 16 patients with BU and 30 eyes of 15 healthy controls. Superficial capillary plexus (SCP) and deeper capillary plexuses (DCP) were evaluated using OCTA RTVue XR AVANTI. RESULTS: On SCP, nonperfusion/hypoperfusion areas (NPA) were seen in 10 eyes, perifoveal capillary arcade disruption (PCAD) was seen in 10 eyes, capillary network disorganization (CND) was seen in 7 eyes and intraretinal cystoid spaces (ICS) were seen in 2 eyes...
February 1, 2019: International Ophthalmology
T A Lisitsyna, D Yu Veltishchev, O F Seravina, O B Kovalevskaya, M N Starovoytova, O V Desinova, A A Abramkin, P S Ovcharov, V I Vasil'ev, Z S Alekberova, V N Krasnov, E L Nasonov
AIM: Research objective - comparative analysis of incidence and structure of anxiety-depressive spectrum disorders (ADD) in patients with various rheumatic diseases (RD). MATERIALS AND METHODS: 613 patients with RD were enrolled in the study: 180 with a reliable diagnosis of systemic lupus erythematosus (SLE), 128 with rheumatoid arthritis (RA), 110 with systemic sclerosis (SSc), 115 with Behcet's disease (BD), 80 with primary Sjögren's syndrome (pSS). Female prevailed in all groups (95% of patients with pSS, 88,2% - SSc, 87,2% - RA, 85,5% of SLE) except BD patients (70% male)...
May 11, 2018: Terapevticheskiĭ Arkhiv
Jui-Ming Liu, Cheng-Ping Yu, Heng-Chang Chuang, Chun-Te Wu, Ren-Jun Hsu
BACKGROUND: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases. METHODS: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases...
January 28, 2019: Prostate Cancer and Prostatic Diseases
Ali Nazmi Çalık, Kazım Serhan Özcan, Banu Mesci, Tufan Çınar, Yiğit Çanga, Barış Güngör, Mukaddes Kavala, Aytekin Oğuz, Osman Bolca, Ömer Kozan
BACKGROUND: The main objective of the current study is to find out if any association exists between specific inflammatory markers such as homocysteine (Hcy) and pentraxin-3 (PTX-3) and cardiac involvement determined by means of echocardiographic parameters in patients with Behçet disease (BD). METHODS: From January 2011 to January 2012, a total of 62 Behçet's patients were enrolled in the study. Thirty-two healthy subjects constituted the control group. The diagnosis of BD was made as proposed by International Study Group of BD...
January 28, 2019: Acta Cardiologica
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