14Th International Conference on Behçet's Disease
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14th International Conference on Behçet’s Disease London, United Kingdom, 8-10 July 2010 Executive Committee – International Society for Behçet’s Disease President Sungnack Lee (Korea) - Dermatology Past-President Hasan Yazici (Turkey) - Rheumatology Vice-President Kenneth Calamia (USA) - Rheumatology Secretary Dongsik Bang (Korea) - Dermatology Treasurer Samir Assaad Khalil (Egypt) - Internal Medicine President of 14th International Congress Dorian Haskard (UK) - Rheumatology President Past International Congress Michael Schirmer (Austria) - Rheumatology Member – Dermatology Eun-So Lee (Korea) - Dermatology Member – Internal Medicine Petros Sfikakis (Greece) - Internal Medicine Member – Scientific Affairs Haner Direskeneli (Turkey) - Rheumatology Member – Publication Affairs Graham Wallace (UK) - Immunology Hon. Life Presidents – International Society for Behçet’s Disease Colin G. Barnes (UK) - Rheumatology Nihat Dilsen (Turkey) - Rheumatology George Ehrlich (USA) - Rheumatology Thomas Lehner (UK) - Immunology Desmond O’Duffy (USA) - Rheumatology Local Organising Committee Honorary President Dorian Haskard (UK) - Rheumatology International Affairs Secretary Colin G. Barnes (UK) - Rheumatology Honorary General Secretary Graham Wallace (UK) - Immunology Farida Fortune (UK) - Oral Medicine Robert Moots (UK) - Rheumatology Miles Stanford (UK) - Ophthalmology Representatives – Behçet’s Syndrome Society Jan Mather (UK) - BSS Chris Phillips (UK) - BSS Abstracts page Oral Presentation index S-107 Oral Presentations S-108 Poster Presentations index S-115 Poster Presentations S-119 14th International Conference on Behçet’s Disease Oral Presentations ORAL PRESENTATION INDEX Board Ref. no. Topic Title Date Time Number O-001 74 Immunology Altered Expression of Costimulatory Molecules in Behçet’s disease According 08.07.2010 10:15-10:30 to Clinical Activity O-002 111 Immunology Expression of pro-inflammatory protein S100A12 (EN-RAGE) in Behçet’s 08.07.2010 10:30-10:45 disease and its association with disease activity O-003 159 Immunology Identification of JAK1 as a candidate inflammatory signalling pathway by 08.07.2010 10:45-11:00 genome-wide expression profiling in monocytes from patients with Behçet’s disease O-004 263 Immunology A novel murine model of Behçet’s-like eye disease associated with proteoglycan 08.07.2010 11:00-11:15 induced arthritis in the absence of gamma interferon. O-005 314 Immunology The functional analysis of MICA polymorphism with an emphasis on Behçet’s 08.07.2010 11:15-11:30 disease O-006 9 Vasculitis Management of thrombosis in 62 patients with Behçet syndrome over 15 years 08.07.2010 15:00-15:15 O-007 194 Vasculitis Pulmonary Artery Hypertension in Behçet’s syndrome 08.07.2010 15:15-15:30 O-008 219 Vasculitis Cutaneous Vasculitis in Behçet’s disease 08.07.2010 15:30-15:45 O-009 309 Vasculitis Pulmonary Perfusion Scintigraphy Findings in Behçet’s disease 08.07.2010 15:45-16:00 O-010 316 Vasculitis Efficacy of Adalimumab in patients with Behçet’s disease unsuccessfully 08.07.2010 16:00-16:15 treated with Infliximab O-011 16 Regional Effects of infliximab in the treatment of refractory posterior uveitis of Behçet‘s 09.07.2010 10:15-10:30 disease after withdrawal of infusions O-012 62 Regional AIN457, a fully human monoclonal anti-interleukin-17A monoclonal antibody, 09.07.2010 10:30-10:45 for the adjunctive treatment of posterior segment uveitis secondary to Behçet’s disease: The SHIELD study O-013 97 Regional Methotrexate in Ocular lesions of Behçet’s disease; a Longitudinal Study 09.07.2010 10:45-11:00 O-014 104 Regional Neuro-Behçet’s disease in Japan: a multicenter retrospective survey 09.07.2010 11:00-11:15 O-015 298 Regional A Comparative Study of Clinical, Endoscopic and Histologic Findings in 09.07.2010 11:15-11:30 Patients with Gastrointestinal Behçet’s disease and Crohn’s disease O-016 98 Paediatric Ethnic and gender patterns of age distribution and duration of Adamantiades- 09.07.2010 15:00-15:15 Behçet’s disease (ABD) course in Germany O-017 166 Paediatric Systemic involvements and currently preferred immunosuppressive agents in a 09.07.2010 15:15-15:30 large population composed of relatively young Behçet’s patients from countrywide O-018 250 Paediatric Cross-sectional survey of neurologic and psychiatric symptoms in North 09.07.2010 15:30-15:45 American patients with Behçet’s syndrome. O-019 264 Paediatric Eye disease in juvenile patients with Behçet’s syndrome 09.07.2010 15:45-16:00 O-020 267 Paediatric Work disability in Behçet’s syndrome 09.07.2010 16:00-16:15 O-021 60 Genetics Methylenetetrahydrofolate reductase (MTHFR) genetic polymorphism in 10.07.2010 10:00-10:15 patients with Behçet’s disease O-022 129 Genetics Impact of HLA-B51(5) on Behçet’s Disease Clinical Phenotype: Systematic 10.07.2010 10:15-10:30 Review and Meta-Analyses on Phenotype-Genotype Correlates O-023 185 Genetics Genome-wide association studies define two susceptibility loci for Behçet’s 10.07.2010 10:30-10:45 disease O-024 191 Genetics A twin study in Behçet’s syndrome 10.07.2010 10:45-11:00 O-025 224 Genetics A Genome-wide Association Study Identifies Common Variants of the IL10 and IL23R Genes that Contribute to Behçet’s Disease Susceptibility 10.07.2010 11:00-11:15 S-107 Oral Presentations 14th International Conference on Behçet’s Disease Oral Presentations Board No: O-003 Ref. No: 159 Topic: Immunology Board No: O-001 IDENTIFICATION OF JAK1 AS A CANDIDATE INFLAMMATORY Ref. No: 74 SIGNALLING PATHWAY BY GENOME-WIDE EXPRESSION PROFILING Topic: Immunology IN MONOCYTES FROM PATIENTS WITH BEHÇET’S DISEASE ALTERED EXPRESSION OF COSTIMULATORY MOLECULES IN 1H. Direskeneli, 4Jj. Boyle, 2Ft. Ozdemir, 3V. Yilmaz, 2E: Ekșioğlu-Demiralp, BEHÇET’S DISEASE ACCORDING TO CLINICAL ACTIVITY 4D. Haskard, 3G. Saruhan-Direskeneli 1Ji Hyun Sim, 1Mi Jin Park, 2Sun Park, 1Eun-So Lee 1Department of Rheumatology, Marmara University, Faculty of Medicine, Istanbul, Turkey, 2Department of Immunology, Marmara University, Faculty of Medicine, 1Department of Dermatology, Ajou University School of Medicine, 2Department of Istanbul, Turkey, 3Department of Physiology, Istanbul University, Istanbul Faculty Microbiology, Ajou University School of Medicine of Medicine, Istanbul, Turkey, 4Cardiovascular Sciences Centre, Imperial College, London, Uk Objectives: For a balanced immune response, key inhibitory costimulartory mol- ecules that critically affect peripheral T cell tolerance are important. To understand Aim: To obtain a global view of the immune responses in Behçet’s Disease (BD) the implication of costimulartory molecules in Behçet’s disease (BD), we investi- compared to Familial Mediterranean Fever (FMF), a systemic, auto-inflammatory gated the expression of CTLA-4 and PD-1 on T cell subsets and of their ligands, disorder. CD80, CD86 and PD-L1 on antigen presenting cells (APCs). Method: Twenty-eight BD (F/M: 9/19, mean age: 33.4), 13 FMF (F/M: 9/4, mean Methods: PBMCs of subjects (11 patients with active BD, 8 patients with inactive age: 30.4) patients and 8 controls (F/M: 4/4, mean age: 30.6) were enrolled. Whole- BD, 8 patients with recurrent aphthous ulcer as disease control and 10 healthy vol- genome microarray profiling was performed with human U133 (Plus 2.0) microar- unteers) were cultured and stained for analysis by flow cytometry. To measure the rays on an Affymetrix platform using isolated CD14+monocyte and CD4+T-lym- sCTLA-4 concentrations in culture supernatants, ELISA was performed. To investi- phocyte subsets. Data was analysed with Genespring (Version 10.0). RT-PCR was gate the mRNA expression level of PD-L1, real time PCR was performed. used for the validation of JAK1 expression. Results: Reduced expression of CTLA-4 on CD4+ T cells after stimulation and its Results: Among 28792 transcripts analysed, in CD14+ monocytes, 1188 transcripts ligand, CD86 on APCs on the resting state was shown in active BD group compared reached a significant difference level with a minimum 2 fold difference observed in with the HC group. There is significantly decreased frequency of PD-L1 express- 279 genes. In CD4+T-lymphocytes, 2880 transcripts showed significant difference ing APCs in the BD group compared with the other group on the resting state and with at least 2 fold difference in 109 genes. Among over-expressed genes in BD this was sustained after stimulation. There were no differences in concentration of CD14+ monocytes, oxysterol binding protein-like 8 (OSBPL8)(3.8 fold), cell-divi- sCTLA4 among each group in culture supernatants at 24 hours and 48 hours after sion-cycle-27 homolog (S. cerevisiae)(CDC27)(3.1 fold) and myeloid/lymphoid or stimulation. Decreased expression of PD-L1 mRNA in the active BD group was mixed-lineage leukemia 3 (MLL3)(3.1 fold) were among the highest. However, in demonstrated compared with the HC group. principal component analysis, Januse-kinase-1 (JAK1)(2.6 fold) and metallothionein Conclusion: This study suggests the possibility of impaired function of CTLA-4/B7 1X, (MT1X)(2.1 fold) are shown to be the major regulatory molecules of associated- pathway and PD-1/PD-L1 pathway as a part of pathogenesis in BD. signalling pathways. Validation by RT-PCR also showed an increased JAK1 expres- sion compared to FMF (BD: 9.5 vs. FMF: 5.1 fold, p=0.045). Discussion: Whole-genome microarray analysis demonstrated a selective activation of BD monocytes compared to FMF, suggesting their significant role between innate Board No: O-002 and adaptive immune responses. Activation of JAK1 through various cytokines such Ref. No: 111 as IL-2, IL-6, IL-15