Abstract
Cogan’s syndrome is a rare systemic vasculitis of unknown origin. It is characterized by the presence of worsening audiovestibular and ocular symptoms that may manifest simultaneously or sequentially. No specific diagnostic laboratory tests or imaging studies exist. The diagnosis is clinical and should be established as early as possible so as to initiate prompt treatment with steroids and prevent rapid progression to deafness or blindness and potentially fatal systemic involvement. We report a case of association between Cogan’s syndrome and ileal Crohn’s disease which we believe deserves attention since, after an accurate review of the literature, we have found approximately 250 reports of patients with Cogan’s syndrome, only 13 of whom with concurrent chronic inflammatory bowel disease; of these 13 cases, none experienced improvement after therapy. In the light of the good outcome obtained in our case, we proposed a valid treatment option with boluses of steroids, combined with early systemic immunosuppression and intra-tympanic steroid injections.
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Introduction
Cogan’s syndrome (CS) is a rare disease named after the ophthalmologist David Cogan who first reported its clinical features in 1945 ; since then, fewer than 250 cases have been reported in the literature .
The classic form of CS affects young Caucasian individuals with an average age of 25 years, and is characterized, according to Cogan’s criteria, by the following triad: 1) rapidly progressive bilateral audiovestibular auditory involvement with features similar to Menière’s disease (vertigo, tinnitus, ataxia and progressive and fluctuating hearing loss, often leading to deafness) 2) ocular involvement (non-syphilitic interstitial keratitis), 3) less than 2 years’ interval between the onset of audio or/and vestibular and ocular symptoms .
In addition to the classic form, in 1980 Haynes et al. defined the criteria for an atypical form, namely: 1) inflammatory eye manifestations with or without interstitial keratitis, 2) eye symptoms associated with audiovestibular symptoms with characteristics other than those seen in Menière’s disease, 3) more than 2 years’ interval between the onset of ocular and audiovestibular symptoms. The atypical form is reported to affect 20% of cases . The vasculitic nature of the syndrome, hypothesized by Cody and Williams in 1960, is suggested by the finding that 70% of patients have systemic symptoms in addition to the ocular and audiovestibular manifestations . In particular, in 15%–21% of cases there may be involvement of the large vessels (Takayasu’s-like) and medium-size vessels (polyarteritis-like) resulting in manifestations affecting the cardiovascular system (aortitis with aortic failure), nervous system (hemiparesis and hemiplegia) and gastrointestinal tract (diarrhea, melena, abdominal pain) . Less typical systemic symptoms include headache (40%), joint pain (35%), fever (27%), arthritis (23%), myalgia (22%), and abdominal pain (13%) .
The autoimmune nature of the syndrome is also suggested by the fact that in 15% to 30% of cases there is an association with other autoimmune conditions such as interstitial nephritis, chronic inflammatory bowel disease (IBD), hypothyroidism and sarcoidosis . The literature to date contains reports of only 13 cases of association between CS and chronic IBD .
The diagnosis of CS is exclusively clinical and based on the Cogan or Haynes criteria. Laboratory tests are non-specific whereas in most cases imaging studies (computed tomography, CT, and magnetic resonance imaging, MRI) fail to reveal abnormalities except in advanced or complicated forms .
We report a case of a patient who developed CS shortly after receiving a diagnosis of Crohn’s disease, and was effectively treated with steroids (intravenous, oral and trans-tympanic) and azathioprine. In view of the paucity of reports of the coexistence of CS and IBD, we believe that the account of our experience may make a valuable contribution to the diagnosis and treatment of these syndromes.
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