Significant laryngeal destruction in a northern European cohort of Behçet's disease patients.
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, particularly in northern Europeans. This is a novel study detailing surprising and significant laryngeal structural changes in a northern European cohort of BD.


METHODS
Patients meeting the International Study Group for Behçet's Disease (ISGBD) and the International Criteria for Behçet's Disease (ICBD) criteria for diagnosis were identified from an institutional database. Patients underwent examination with an otolaryngologist, including flexible laryngoscopy. Intra-oral, pharyngeal and laryngeal manifestations of BD were documented and characterised. Patients underwent hearing assessment with pure-tone audiometry.


RESULTS
Fifteen patients with BD were identified (4 male, 11 female; median age 36 years). 60% (n=9) showed evidence of disease on examination and flexible laryngoscopy. 33% (n=5) showed laryngeal changes related to BD. 13% (n=2) demonstrated bilateral sensorineural hearing loss. The 5 cases demonstrating laryngeal manifestations of disease are described in detail with photographic records.


CONCLUSIONS
Limited data has been published regarding the laryngeal manifestations of BD, particularly in a northern European population. Our cohort of BD patients demonstrate significant laryngeal structural changes. It would appear that these clinically relevant changes may be more common than was previously thought. Raised awareness of the risk of laryngeal pathology in BD patients, often in the absence of overt clinical symptomatology, may result in earlier diagnosis and treatment. Rheumatologists and otolaryngologists should consider closer multi-disciplinary co-operation in the management and follow up of patients with BD.
Department of Otolaryngology, Head & Neck Surgey, University Hospital Limerick, Dooradoyle, Limerick, Ireland.
Conall Walter Robert
CW
Fitzgerald
Department of Rheumatology, University Hospital Limerick, Dooradoyle, Limerick; and University of Limerick, Graduate Entry Medical School, Limerick, Ireland.
Fahd
F
Adeeb
Department of Otolaryngology, Head & Neck Surgery, Royal Victoria Eye and Ear Hospital, Dublin, Ireland.
Conrad V
CV
Timon
Department of Otolaryngology, Head & Neck Surgey, University Hospital Limerick, Dooradoyle, Limerick, Ireland.
Neville P
NP
Shine
Department of Rheumatology, University Hospital Limerick, Dooradoyle, Limerick; and University of Limerick, Graduate Entry Medical School, Limerick, Ireland. alexander.fraser@hse.ie.
Alexander Duncan
AD
Fraser
Department of Otolaryngology, Head & Neck Surgey, University Hospital Limerick, Dooradoyle, Limerick, Ireland.
Joseph P
JP
Hughes
D000328:Adult; D000368:Aged; D001301:Audiometry, Pure-Tone; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D016208:Databases, Factual; D005260:Female; D006312:Hearing Loss, Bilateral / Q000175:diagnosis / Q000209:etiology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D007494:Ireland; D007818:Laryngeal Diseases / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D007828:Laryngoscopy; D007830:Larynx / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012307:Risk Factors; D055815:Young Adult