Elsevier

The Journal of Pediatrics

Volume 160, Issue 2, February 2012, Pages 303-307.e1
The Journal of Pediatrics

Original Article
Clinical Features and Outcome of Cogan Syndrome

https://doi.org/10.1016/j.jpeds.2011.07.051Get rights and content

Objective

To review the clinical features of Cogan syndrome, a rare vasculitis characterized by systemic, ocular, and audiovestibular symptoms.

Study design

Clinical records of patients with Cogan syndrome followed at 2 pediatric rheumatology institutions and those from a database search were reviewed. Data included clinical features at onset and during the disease course, treatments, and outcomes.

Results

Twenty-three children with Cogan syndrome (15 males; mean age, 11.4 years [range, 4-18 years]) were included in the analysis. Eleven patients (47.8%) exhibited systemic features at disease onset, including fever, arthralgias-arthritis or myalgias, headache, and weight loss. Twenty-one patients (91.3%) had ocular symptoms, due mainly to interstitial keratitis, uveitis, or conjunctivitis/episcleritis. Vestibular symptoms (39.1%) included vertigo, vomiting, and dizziness. Auditory involvement (65.2%) consisted of sensorineural hearing loss, tinnitus, and deafness. Four patients had cardiac valve involvement, and 3 had skin manifestations. After a median 2 years of follow-up, 30.4% of the patients were in clinical remission, but all others had irreversible complications (deafness, 21.7%; sensorineural hearing loss, 13.0%; vestibular dysfunction, 4.3%; ocular complications, 13.0%; cardiac valve damage, 17.4%).

Conclusion

Audiovestibular and ocular involvement have a major impact on prognosis in children with Cogan syndrome.

Section snippets

Methods

Since 1995, we have followed 3 patients with Cogan syndrome who had at least 1 year of follow-up. Two of these patients have been seen at the Pediatric Rheumatology Unit of Padua, and the other has been treated at the Pediatric Rheumatology Unit of the Meyer Children’s Hospital in Florence. To investigate the clinical course and prognosis of the Cogan syndrome presenting in childhood, we conducted a MEDLINE search of all articles published in the English language during the last 30 years. The

Results

This study included 23 children with Cogan syndrome, 20 from previously published reports and 3 from our own series (Table; available at www.jpeds.com). The cohort comprised 15 males and 8 females, with a median age at disease onset of 11 years, 4 months, (range, 4-18 years), a median age at diagnosis of 12 years (range, 4-21 years), and a median follow-up period after diagnosis of 2 years (range, 1-16 years).

Eleven patients (47.8%) demonstrated systemic features at disease onset (Figure),

Discussion

Since its first description published in 1945,28 few cases of Cogan syndrome with pediatric onset have been reported.4, 8, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 29, 30, 31 Cogan syndrome is a very rare disorder, particularly in children. Its diagnosis is essentially clinical and based mainly on ocular and ENT symptoms often associated with systemic manifestations, including fever, musculoskeletal complaints, headache, and weight loss. These systemic symptoms were related to a good

References (38)

  • I.C. Ndiaye et al.

    Cochleovestibular impairment in pediatric Cogan’s syndrome

    Pediatrics

    (2002)
  • M. Gaubitz et al.

    Cogan’s syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature

    Clin Exp Rheumatol

    (2001)
  • J.J. Dekker et al.

    Cogan’s syndrome as an extra-articular manifestation of rheumatoid arthritis

    Clin Rheum

    (1996)
  • P. Casoli et al.

    Cogan’s syndrome: a new possible complication of antiphospholipid antibodies

    Clin Rheumatol

    (1995)
  • Y. Yamanishi et al.

    Atypical Cogan’s syndrome associated with antineutrophil cytoplasmic antibodies

    Br J Rheumatol

    (1996)
  • H.J. Son et al.

    Course of auditory impairment in Cogan’s syndrome

    Am J Otolaryngol

    (2009)
  • B. Bachynski et al.

    Cogan’s syndrome: a treatable cause of neurosensory deafness

    Can J Ophathalmol

    (1984)
  • C.F.J. Garcìa et al.

    Inner ear autoimmune disorder: Cogan’s syndrome

    An Esp Pediatr

    (2001)
  • T. Inoue et al.

    Low-dose oral methotrexate for the management of childhood Cogan’s syndrome: a case report

    Clin Rheumatol

    (2007)
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      Citation Excerpt :

      We will not discuss diseases for which vestibular symptoms have been rarely described, may be purely coincidental, or are predominantly due to central nervous system involvement. Cogan syndrome predominantly affects young adults between the ages of 20 and 40 years, but can occur in a pediatric population (Cogan, 1945; Pagnini et al., 2012). Typical Cogan syndrome is defined by nonsyphilitic interstitial keratitis associated with audiovestibular symptoms (Cogan, 1945).

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    The authors declare no conflicts of interest.

    Contributed equally to this work.

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