Cogan syndrome is a syndrome of non-syphilitic interstitial keratitis associated with vestibuloauditory deficits. We report a 10 year-old male child who presented with fever, acute polyarthritis, and unilateral red eye and was diagnosed as acute rheumatic fever. Subsequently unilateral hearing loss was detected and the child was diagnosed to have atypical Cogan syndrome.

Key words: Acute rheumatic fever, Arthritis, Atypical Cogan’s syndrome, Polyarthritis.

A 10 years old male child was referred to our center as a case of acute rheumatic fever. There was history of low grade fever for 7 days, severe pain and swelling in multiple large joints for 4 days and redness of the right eye for 4 days. There was no history of any antecedent upper respiratory tract infection. On examination, there was redness, swelling, tenderness and restriction of movement in bilateral knee, ankle and wrist joints. On ophthalmologic evaluation, visual acuity in the right eye was 2/60 with presence of anterior uveitis and inflammatory hypopyon. Left eye was normal. Bilaterally pupillary reaction was normal. Except for mild hepatomegaly, rest of the systemic examination was unremarkable. Initial laboratory evaluation revealed mild polymorphonuclear leucocytosis, raised ESR and elevated C-reactive protein. His chest radio-graph, electrocardiogram and echocardiogram were unremarkable. A differential diagnosis of acute rheumatic arthritis, and autoimmune disorder was considered and he was started on high dose aspirin (75mg/kg/day). In addition topical steroids and mydriatics were given for the right eye. The joint symptoms responded dramatically to aspirin, which further tilted the diagnosis in favour of acute rheumatic arthritis.

On day 3 of admission, patient complained of right-sided hearing loss and tinnitus for last 7-8 days. There was no history of vomiting and vertigo. Local ear examination was normal. A BERA screen showed no response in right ear. Auditory Steady State Response (ASSR) assessment showed profound deafness of right ear with mild hearing loss of left ear. MRI of the petrous bone was suggestive of right sided labyrinthitis. Other laboratory investigations including antistreptolysin O, VDRL, RA factor and ANA were normal.

Cogan syndrome is a systemic vasculitis that mainly affects young adults but can appear from ages 3.5 month to 81 years [6-7]. In approximately 30% of the cases, the disease is preceded by an upper respiratory tract infection [4-7]. The disease generally begins with ocular or audiovestibular symptoms separated by months, or occasionally these can occur con-currently [4,5]. Audiovestibular symptoms of typical Cogan syndrome are similar to that of Meniere’s disease (sudden onset of nausea, vomiting, tinnitus and vertigo) that usually progress to complete deafness within several hours to days [7]. Various systemic features like fever, headache, myalgia, arthralgia, arthritis, rash, abdominal pain, gastrointestinal bleeding, pleuritis, lymphadenopathy, splenomegaly, encephalitis, cerebral infarction and other vasculitis may also be present [3-8]. Approximately 10% cases are complicated with aortic insufficiency associated with aortitis and inflammatory or myxoid degenerative valve changes [3-8].

The disease course is variable. Mostly it presents as an initial flare followed by a chronic and slowly progressive course. Some may have recurrent flare-ups secondary to intercurrent infections with remissions in between. Deafness is usually permanent. Death can occur due to cardiac failure. The patient should be kept under regular follow-up in view of possibility of life-threatening complications and possible side effects of immunosuppressive agents [5].

Contributors: All authors were involved in the case management. BC and RJ were involved in the review of literature and preparation of the manuscript. MJ and RJ drafted the final manuscript.

Funding: None.

Competing interests: None stated.

1. Cogan DG. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol. 1945;33:144.

2. Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan’s syndrome: studies in thirteen patients, long term follow up, and a review of the literature. Medicine (Baltimore). 1980;59:426-41.

3. Murphy G, Sullivan MO, Shanahan F, Harney S, Molloy M. Cogan’s syndrome: present and future directions. Rheumatol Int. 2009;29:1117-21.

4. Gluth MB, Baratz KH, Matteson EL, Driscoll CL. Cogan’s syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc. 2006;81:483-8.

5. Grasland A, Pouchot J, Hachulla E, Blétry O, Papo T, Vinceneux P; Study Group for Cogan’s Syndrome. Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology (Oxford). 2004;43:1007-15.

6. Cheson BD, Bluming AZ, Alroy J. Cogan’s syndrome: a systemic vasculitis. Am J Med. 1976;60:549.

7. Vinceneux P. Cogan’s syndrome. Orphanet Encyclopedia. 2003;134:1-7.

8. Vollertsen RS, McDonald TJ, Younge BR, Banks PM, Stanson AW, Ilstrup DM. Cogan’s syndrome: 18 cases and a review of the literature. Mayo Clin Proc. 1986;61:344-61.