Background/Purpose: Cogan syndrome (CS) is a rare inflammatory disorder typically characterized by interstitial keratitis and audiovestibular symptoms, and patients may also develop other inflammatory ocular symptoms, sensorineural hearing loss and systemic manifestations including aortitis, aortic insufficiency. We herein aimed to investigate the diagnostic and therapeutic characteristics of five CS patients followed-up in a tertiary referral center.

Methods: We retrospectively reviewed the charts of the five patients diagnosed with CS according to their clinical manifestations. All of the clinical findings, laboratory and imaging results and treatment responses were recorded using a standard form. Because of the sample size, no statistical analysis was carried out, and results are summarized as descriptive findings.

Results: The mean age of the patients diagnosed with CS was 34 years, and the mean follow-up duration was 7.6 years. Demographic and clinical characteristics of CS patients are summarized in Table 1. Sensorineural hearing loss and intraocular findings were present in all patients. We observed variability in ocular findings, which included typical interstitial keratitis in three, and scleritis in two patients. Although aortitis has rarely been reported previously, diagnostic work-up included PET-CT imaging in three patients, and it revealed positive findings compatible with aortitis. One of our patients needed aortic valve replacement due to severe aortic regurgitation. All patients responded well to high dose corticosteroids, but complete remission was achieved in 1 patient with a mild disease course with methotrexate. Remaining 4 patients required biologic agents to control inflammatory activity. One patient with polyarthritis responded adalimumab and methotrexate combination. However, no response or even exacerbation of findings were observed in two patients with anti-TNF, and a partial or near-complete remission could be achieved with IL-1 blockade.

Table 1. Demographic and clinical features of Cogan syndrome patients.

SNHL: sensorineural hearing loss ,MP: methylprednisolone; MTX: methotrexate; ANK: anakinra; CAN: canakinumab; ETN: etanercept; ADA: adalimumab; TCZ: tocilizumab.

Conclusion: Patients with CS comprise a heterogeneous group, which may include some yet undiagnosed hereditary autoinflammatory conditions. Aortic involvement has rarely been reported in CS patients, but PEY-CT imaging may help diagnosing asymptomatic patients with inflammatory imaging findings in aorta. Early and effective treatment may prevent organ damage, and a differential response to IL-1 blocking biologic agents requires further investigation for both understanding its pathogenesis and better management of refractory patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cogan-syndrome-differential-response-to-biologic-agents-and-role-of-pet-ct-in-the-increased-diagnosis-of-aortitis/