S-123 1 Department of Otolaryngology, Head & Neck Surgery, University Hospital Limerick, Dooradoyle, Limerick, Ireland; 2 Department of Rheumatology, University Hospital Limerick, Dooradoyle, Limerick, Ireland; 3 Department of Otolaryngology, Head & Neck Surgery, Royal Victoria Eye and Ear Hospital, Dublin, Ireland; 4 University of Limerick, Graduate Entry Medical School, Limerick, Ireland. Conall W.R. Fitzgerald, MD, FRCSI Fahd Adeeb, MRCPI Conrad V. Timon, MD, FRCSI Neville P. Shine, FRCSI Alexander D. Fraser, MD, FRCSI Joseph P. Hughes, FRCSI Please address correspondence to: Alexander Duncan Fraser, Consultant Rheumatology, University Hospital Limerick, Dooradoyle, Limerick, Ireland. E-mail: alexander.fraser@hse.ie Received on June 26, 2015; accepted in revised form on August 31, 2015. Clin Exp Rheumatol 2015; 33 (Suppl. 94): S123-S128. © Copyright CliniCal and ExpErimEntal rhEumatology 2015. Key words: Behçet’s disease, otolaryngology, laryngeal changes, ear, nose, throat Competing interests: none declared. ABSTRACT Objective. Behçet’s disease (BD) is a multisystem autoimmune disease of unknown origin typically affecting the triad of oral and genital mucosa and the eye. Limited data are available in the literature regarding the otolaryngology- related manifestations of BD, particu- larly in northern Europeans. This is a novel study detailing surprising and sig- nifcant laryngeal structural changes in a northern European cohort of BD. Methods. Patients meeting the Interna- tional Study Group for Behçet’s Disease (ISGBD) and the International Criteria for Behçet’s Disease (ICBD) criteria for diagnosis were identifed from an institutional database. Patients under- went examination with an otolaryngol- ogist, including fexible laryngoscopy. Intra-oral, pharyngeal and laryngeal manifestations of BD were documented and characterised. Patients underwent hearing assessment with pure-tone au- diometry. Results. Fifteen patients with BD were identifed (4 male, 11 female; median age 36 years). 60% (n=9) showed evidence of disease on examination and fexible laryngoscopy. 33% (n=5) showed laryngeal changes related to BD. 13% (n=2) demonstrated bilateral sensorineural hearing loss. The 5 cases demonstrating laryngeal manifesta- tions of disease are described in detail with photographic records. Conclusion. Limited data has been pub- lished regarding the laryngeal manifes- tations of BD, particularly in a north- ern European population. Our cohort of BD patients demonstrate signifcant laryngeal structural changes. It would appear that these clinically relevant changes may be more common than was previously thought. Raised aware- ness of the risk of laryngeal pathology in BD patients, often in the absence of overt clinical symptomatology, may re- sult in earlier diagnosis and treatment. Rheumatologists and otolaryngologists should consider closer multi-discipli- nary co-operation in the management and follow up of patients with BD. Introduction Behçet’s disease (BD) is a complex, infammatory, systemic disease of un- known aetiology. Although Hippocrates frst described a patient with a triad of mouth ulcers and symptoms affect- ing the genitals and eyes 2,500 years ago, Professor Hulusi Behçet, a Turk- ish dermatologist, in 1937 provided the frst characterisation of the trisymptom ‘occulo-buccal-genital’ complex (1, 2). The clinical manifestations of BD are believed to be attributable to vasculi- tis, which could involve blood vessels of any size from arterial or venous cir- culation. The prevalence of BD shows signifcant geographical variation with a reported prevalence ranging from 80–400/100,000 in Turkey and Middle- Eastern countries, to less than 1/100,000 in western Europe (3, 4). Pathogenesis of the disease is not well understood, however, environmental and genetic predisposition may play a role in the de- velopment of the disease (3, 4). No pathognomonic laboratory test ex- ists to diagnosis BD. As such, the di- agnosis of BD is guided by validated criteria, most commonly used are the International Study Group for Behçet’s Disease (ISGBD) or the International Criteria for Behçet’s Disease (ICBD). In the ISGBD criteria, which were pub- lished in 1990, recurrent oral aphthosis three or more times in a year is man- datory, with the presence of any two of the following: genital ulceration, ocu- lar or cutaneous manifestation, or skin pathergy. While having excellent specifcity, the ISGBD criteria lacked sensitivity, and for this reason the ICBD criteria were developed and frst presented in 2006 (5, 6). For this classifcation, which is Signifcant laryngeal destruction in a northern European cohort of Behçet’s disease patients C.W.R. Fitzgerald 1 , F. Adeeb 2,4 , C.V. Timon 3 , N.P. Shine 1 , A.D. Fraser 2,4 , J.P. Hughes 1