Background/Purpose:

We aimed to describe the Cogan Syndrome, the efficacy of DMARDs and biological targeted agents.

Methods:   A national multicenter retrospective study in France including patients from 2001 to 2015. Data were recorded at the time of each treatment initiation, at 6 months, and at the end of each treatment. Efficacy outcomes were the intention-to-treat rates of systemic, ocular and audiovestibular responses during the first 6 months of exposure, the evolution of the steroid doses and the number of relapses. Typical Cogan Syndrome was defined if ocular and audiovestibular involvements and the presence of interstitial keratitis were observed within less than 2 years. The efficacy of biologics was compared to DMARDs with Kaplan-Meier curves.

Results:   Sixty two patients were included with median age 37 [2-76] and 31/62 (50%) women. At the diagnosis, 61 (98%) had audiovestibular involvement, with bilateral hypoacousia/deafness, 19 (31%), 38 (95%) 57 (92%) with ophtalmological symptoms (keratitis 54%, scleritis 35%), 42 (68%) systemic symptoms. The time between the appearance of audiovestibular and ophtalmological symptoms was 2 months [ranges; 0-180]. Median ESR and CRP levels were 26 [3-125] mm and 15 [1-355] mg/l. The median follow-up was 34 [0-228] months. Comparing typical Cogan Syndrome to atypical cases (n=31 each), women (68% versus 32%, p<0.05), the presence of scleritis/episcleritis (10% versus 55%, p<0.05), significantly differed between groups. The first-line treatment was used in 61 (98%) patients and consisted in steroids alone (n=42), with median dose 60 mg/day [20-120], combined with other immunosuppressive drugs in 19 patients (31%)(methotrexate=8; azathioprine= 3; cyclophosphamide=8). Although ocular, constitutional signs and acute phase reactants were improved in nearly 80% of patients, only 28% had complete audiovestibular response. A second-line treatment was used in 44 cases, for relapses (n=27), steroid dependence (n=5), adverse effects or non-response (n=12). Overall, 129 lines of treatment were used in 61 patients and consisted in steroids alone (n=50), with steroids associated with DMARDs (n=69) and biological-targeted drugs (n=10). No difference was noted between 3 treatment regimens for the number of audiovestibular, ocular, systemic involvements, neither for the median dose of steroids, although the number of previous lines of treatment was more important for patients using biologics (1 (1-3) for steroids, 2 (1-5) for DMARDs and 2.5 (2-6) for biologics, p<0.05). Although the response rates were similar for ocular and systemic signs, audiovestibular improvement was significantly more frequent under biologics in comparison to steroids alone and DMARDs (80% versus 39% and 35%, respectively, p<0.05).

Conclusion: Audiovestibular involvement has a poor prognosis, with less response to treatment than ophtalmological symptoms. Biological targeted drugs seem to improve the audiovestibular impairment, whereas the addition of DMARDs does not improve the audiovestibular outcome.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/cogan-syndrome-treatment-and-outcome-from-french-nationwide-retrospective-study-and-literature-review-of-62-patients/