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Shudan Wang, Noam Broder, Paula Marchetta, Johannes Nowatzky
A 46-year-old Hispanic man presented with fever, genital ulcers, left eye redness and chest pain. Physical examination was notable for a healed oral ulcer and scrotal ulcers, and bilateral superficial thrombophlebitis. He was found to have new-onset pancytopenia. CT of the chest showed pericardial and pleural effusions and rapidly progressing inflammation of the aortic arch and ascending vessels. Although the patient had Behcet's disease (BD)-like symptoms, pancytopenia could not be explained by the diagnosis, prompting a bone marrow biopsy which showed myelodysplastic syndrome...
March 15, 2018: BMJ Case Reports
Logan W Thomas, Ashley Elsensohn, Terese Bergheim, Jessica Shiu, Anand Ganesan
IMPORTANCE: Intramuscular (IM) steroids can be used to treat a wide variety of dermatologic diseases. Although seemingly effective and safe, this form of corticosteroid therapy may be underused amongst dermatologists. OBJECTIVE: The objective of this review is to determine the evidence regarding the efficacy and side effect profile of intramuscular triamcinolone in the treatment of dermatologic disease. EVIDENCE REVIEW: A PubMed search engine was used for this study...
March 1, 2018: Journal of Drugs in Dermatology: JDD
Eda Balkan, Handan Bilen, Nilnur Eyerci, Sadullah Keleş, Aslı Kara, Necmettin Akdeniz, Hasan Dogan
BACKGROUND To investigate the gene expression levels of interleukin 10 (IL10), IL18, interferon gamma (IFNG), IFN-gamma receptor (IFNGR), C-reactive protein (CRP), and heat shock protein 70 (HSP70) in patients with active Behçet's uveitis. MATERIAL AND METHODS Forty patients with Behçet's disease diagnosed according to the International Study Group criteria and 30 healthy individuals were included in the study. IL10, IL18, IFNG, IFNGR, CRP, and HSP70 gene expression levels were compared. RESULTS Expression levels of IL18, IFNG, IFNGR, and CRP were significantly higher in patients with active Behçet's uveitis than in control subjects (P<0...
March 13, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Cristian Herrera, Miguel Marcos, Cristina Carbonell, José Antonio Mirón-Canelo, Gerard Espinosa, Ricard Cervera, Antonio-Javier Chamorro
INTRODUCTION: The human glucocorticoid receptor gene (NR3C1) is considered to play a role in the differences and sensitivities of the glucocorticoid response in individuals with autoimmune diseases. The objective of this study was to examine by means of a systematic review previous findings regarding allelic variants of NR3C1 in relation to the risk of developing systemic autoimmune diseases. METHODS: Studies that analysed the genotype distribution of NR3C1 allelic variants among patients with systemic autoimmune diseases were retrieved...
March 8, 2018: Autoimmunity Reviews
Oguz Abdullah Uyaroglu, Emrah Seyhoglu, Abdulsamet Erden, Cevanşir Vahabov, Hakan Babaoglu, Berkan Armagan, Alper Sari, Levent Kilic, Olcay Tatar, Sule Apras Bilgen, Omer Karadag, Umut Kalyoncu
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...
March 9, 2018: Irish Journal of Medical Science
M Roriz, I Crassard, S Lechtman, D Saadoun, K Champion, B Wechsler, H Chabriat, D Sène
There is as yet no consensus on the treatment of cerebral venous thrombosis (CVT) in Behçet's disease, and the place of anticoagulation is also still being debated. This report is of a series of seven patients with Behçet's disease (BD)-associated CVT, for which anticoagulation was stopped, and discusses the possibility of stopping anticoagulation during follow-up while receiving optimal treatment for BD. The diagnosis of BD was established during follow-up, which lasted a median of 120 [range: 60-1490] days after CVT diagnosis...
March 7, 2018: Revue Neurologique
Guillaume Voiriot, Antoine Parrot, Martine Antoine, Aude Gibelin, Samuel Haddad, Marie-France Carette, Muriel Fartoukh, Antoine Khalil
Hemoptysis is a life-threatening complication of Behcet's disease that is likely related to pulmonary artery aneurysm (PAA). Vascular interventional radiology may offer effective emergency therapeutic option, but has not been thoroughly investigated in this setting. A case series of a French referral center for hemoptysis combined with a literature review of case reports was conducted. Between 1995 and 2016, 12 patients were referred to our center for hemoptysis revealing or complicating the course of Behcet's disease...
March 7, 2018: Internal and Emergency Medicine
Gergely Bodis, Victoria Toth, Andreas Schwarting
Since the discovery of HLA 60 years ago, it has contributed to the understanding of the immune system as well as of the pathogenesis of several diseases. Aside from its essential role in determining donor-recipient immune compatibility in organ transplantation, HLA genotyping is meanwhile performed routinely as part of the diagnostic work-up of certain autoimmune diseases. Considering the ability of HLA to influence thymic selection as well as peripheral anergy of T cells, its role in the pathogenesis of autoimmunity is understandable...
March 7, 2018: Rheumatology and Therapy
Qi Zhang, Hua Li, Shengping Hou, Hongsong Yu, Guannan Su, Bolin Deng, Jian Qi, Chunjiang Zhou, Aize Kijlstra, Peizeng Yang
BACKGROUND: Protein tyrosine phosphatases (PTPs) play critical roles in human autoimmunity. Previous studies found that PTPN2 may be the key regulatory factor in the T-cell-mediated immune response. PTPN2 regulates the Janus kinase/signal transducers and activators of transcription pathway by inhibiting signalling via the interleukin (IL)-2 receptor (CD122). An association between genetic variations in PTPN2 and CD122 with ocular Behcet's disease (BD) has not yet been addressed and was therefore the purpose of this study...
March 3, 2018: British Journal of Ophthalmology
Maria Zerkaoui, Fatima Zahra Laarabi, Yousra Ajhoun, Bouchra Chkirate, Abdelaziz Sefiani
BACKGROUND: Familial Mediterranean fever is an autoinflammatory disease of unknown etiology, characterized clinically by recurrent attacks of sudden-onset fever with arthralgia and/or thoracoabdominal pain and pathogenetically by autosomal recessive inheritance due to a mutation in the MEFV gene. Behçet's disease is an inflammatory disease characterized by recurrent oral and genital aphthous ulcerations, uveitis, and skin lesions. Preliminarily, our literature review suggested that patients with familial Mediterranean fever who also have Behçet's disease have only a single mutated familial Mediterranean fever gene...
March 1, 2018: Journal of Medical Case Reports
Yingying Yue, Jun Zhang, Lu Yang, Shengyun Liu, Jian Qi, Qingfeng Cao, Chunjiang Zhou, Yao Wang, Aize Kijlstra, Peizeng Yang, Shengping Hou
Purpose: Long noncoding RNAs (lncRNAs) are emerging as important regulators of inflammatory immune responses, whereby genetic variants may affect this biologic function. This study aimed to investigate the association of 110 single nucleotide polymorphisms (SNPs) of lncRNAs, known to be associated with autoimmune disease, in patients with ocular Vogt-Koyanagi-Harada (VKH) disease, Behcet's disease (BD), and acute anterior uveitis (AAU) with or without ankylosing spondylitis (AS). Methods: A two-stage case-control study was performed on 1626 VKH patients, 384 BD patients, 624 AAU with AS, 751 AAU without AS, 720 AS without AAU, and 3305 healthy subjects...
February 1, 2018: Investigative Ophthalmology & Visual Science
Sameer Lakha, C Y Maximilian Png, Kevin Chun, Windsor Ting
OBJECTIVE: Vascular manifestations including pulmonary artery aneurysms and venous thrombosis are seen in up to 14% of patients with Behcet's Disease. We report a patient who had recurrent deep vein thrombosis (DVT) as the presenting symptom of Behcet's Disease. METHODS: A 19-year-old male who presented with acute iliofemoral DVT, confirmed by intravascular ultrasound (IVUS) and venogram. May-Thurner syndrome was also observed. Repeated catheter-based pharmaco-mechanical thrombolysis, thrombectomy and subsequent iliac vein stenting were performed...
February 23, 2018: Annals of Vascular Surgery
Kentaro Awaji, Ryota Inokuchi, Hiromu Maehara, Makoto Moriyama, Tomoya Omae, Makoto Okamoto, Yasuhiro Suyama
No abstract text is available yet for this article.
February 20, 2018: QJM: Monthly Journal of the Association of Physicians
Belén Atienza-Mateo, Vanesa Calvo-Río, Emma Beltrán, Lucía Martínez-Costa, Elia Valls-Pascual, Marisa Hernández-Garfella, Antonio Atanes, Miguel Cordero-Coma, Joan Miquel Nolla, Carmen Carrasco-Cubero, Javier Loricera, María C González-Vela, Nuria Vegas-Revenga, Carlos Fernández-Díaz, Rosalía Demetrio-Pablo, Lucía C Domínguez-Casas, José Luis Martín-Varillas, Natalia Palmou-Fontana, José L Hernández, Miguel Á González-Gay, Ricardo Blanco
Objective: To assess the efficacy of tocilizumab (TCZ) in refractory uveitis of Behçet's disease (BD). Methods: Multicentre study of patients with BD-associated uveitis. Patients were refractory to conventional and biologic immunosuppressive drugs. The main outcome measures were intraocular inflammation, macular thickness, visual acuity and corticosteroid-sparing effects. Results: We studied 11 patients (7 men) (20 affected eyes); median age 35 years...
February 19, 2018: Rheumatology
Ahmet Ilbay, Abdulsamet Erden, Alper Sari, Berkan Armagan, Burak Yasin Aktas, Ertugrul Cagrı Bolek, Umut Kalyoncu, Omer Karadag
No abstract text is available yet for this article.
February 22, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Yumi Motokura, Haruki Watanabe, Yuriko Yamamura, Yuzuki Kano, Yoshinori Matsumoto, Tomoko Kawabata, Ken-Ei Sada, Jun Wada
No abstract text is available yet for this article.
February 22, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
A Okano, M Ohana
No abstract text is available yet for this article.
March 2018: Journal of Gastroenterology and Hepatology
Sentaro Kusuhara, Atsuko Katsuyama, Wataru Matsumiya, Makoto Nakamura
PURPOSE: The purpose of this study was to describe the initial experience, efficacy, and safety of ripasudil hydrochloride hydrate (ripasudil), a Rho-associated kinase inhibitor eye drop, for uveitic glaucoma. METHODS: In this retrospective case series, we retrieved the clinical data of 21 eyes from 19 patients with open-angle uveitic glaucoma who were treated with ripasudil at Kobe University Hospital. We analyzed the median intraocular pressure (IOP) reductions after ripasudil treatment and collected the information on the adverse events that were encountered during the course of this treatment period...
February 21, 2018: Graefe's Archive for Clinical and Experimental Ophthalmology
Leandro L Prado, Celio R Goncalves, Vilma T Viana, Carla G S Saad, Eloisa Bonfa
OBJECTIVES: To assess IgM anti-alpha-enolase antibodies (AAEA) in systemic Behçet's disease (BD) and its possible association with clinical manifestations and disease activity. METHODS: Ninety-seven consecutively selected BD patients were compared to 36 enteropathic spondyloarthritis (ESpA) [24 Crohn's disease (CD) and 12 ulcerative colitis (UC)] patients and 87 healthy controls. IgM AAEA was detected by immunoblotting. Disease activity was assessed by standardised indexes, Brazilian BD Current Activity Form (BR-BDCAF) for BD and Harvey-Bradshaw Index (HBI) for CD and UC patients...
February 7, 2018: Clinical and Experimental Rheumatology
Ji Hwan Lee, Christopher Seungkyu Lee, Sung Chul Lee
BACKGROUND: To evaluate therapeutic outcomes of interferon alpha-2a (IFNα2a) treatment in patients with Behcet's disease who were refractory to immunosuppressive agents. METHODS: This retrospective case series reviewed the medical records of 5 patients with refractory Behcet uveitis from January 2011 to February 2017. IFNα2a was administered at a dose of 3 million IU 3 times per week. Clinical response, relapse rate, and change of visual acuity were evaluated...
February 20, 2018: BMC Ophthalmology
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