Significant laryngeal destruction in a northern European cohort of Behçet's disease patients

Clin Exp Rheumatol. 2015 Nov-Dec;33(6 Suppl 94):S123-8. Epub 2015 Oct 19.

Abstract

Objectives: 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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Audiometry, Pure-Tone
  • Behcet Syndrome / complications*
  • Behcet Syndrome / diagnosis
  • Behcet Syndrome / immunology
  • Behcet Syndrome / therapy
  • Databases, Factual
  • Female
  • Hearing Loss, Bilateral / diagnosis
  • Hearing Loss, Bilateral / etiology
  • Hearing Loss, Sensorineural / diagnosis
  • Hearing Loss, Sensorineural / etiology
  • Humans
  • Ireland
  • Laryngeal Diseases / diagnosis
  • Laryngeal Diseases / etiology*
  • Laryngeal Diseases / immunology
  • Laryngeal Diseases / therapy
  • Laryngoscopy
  • Larynx / immunology
  • Larynx / pathology*
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Risk Factors
  • Young Adult