Report of 2 pediatric cases with atypical Cogan's syndrome and a systematic review

KASAP CÜCEOĞLU M., BAŞARAN H. Ö., BATU AKAL E. D., Kaya Akca U., Atalay E., ŞENER S., ...More

International Journal of Rheumatic Diseases, vol.26, no.3, pp.544-550, 2023 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 26 Issue: 3
  • Publication Date: 2023
  • Doi Number: 10.1111/1756-185x.14531
  • Journal Name: International Journal of Rheumatic Diseases
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, MEDLINE
  • Page Numbers: pp.544-550
  • Keywords: aortitis, childhood vasculitis, cochlear implant, Cogan's syndrome, keratitis, sudden hearing loss, systematic review
  • Hacettepe University Affiliated: Yes

Abstract

© 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral. Although glucocorticoids are fundamental treatment options, in most cases, hearing loss gradually worsens. Herein we report 2 pediatric cases of CS who were treated with corticosteroids and methotrexate. One patient had a cochlear implant, and the hearing of the other patient improved with treatment. Also, a systematic literature review was conducted for articles including pediatric CS patients. In the literature, 34 articles describing 44 pediatric patients with CS were identified. Sudden hearing loss (95.3%) and ocular symptoms (92.5%) were the most common manifestations in these patients. Also, aortic involvement was present in 19.5% of patients in the literature. Otorhinolaryngologists, ophthalmologists, and pediatricians should collaborate to diagnose and manage CS to prevent progressive hearing loss and eye involvement.