ReviewCogan syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review
Introduction
Cogan syndrome was first described in 1945 by an ophthalmologist, David G. Cogan, who reported on a “syndrome of non-syphilitic interstitial keratitis and vestibulo-auditory symptoms” that resembled Meniere's disease [1]. Other ocular signs such as conjunctivitis, uveitis, scleritis and choroiditis have been described recently and are considered atypical forms in the absence of interstitial keratitis.
Since 1945, > 250 cases of Cogan syndrome have been reported, and the disease seems to have an autoimmune origin; no easily available autoantibodies have been clearly associated with this syndrome [2], [3]. Alleviating the vestibulo-auditory symptoms, and in particular the hearing loss, remains challenging. Because of the rarity of the disease, no controlled study has been performed, with only small reported series, and the value of immunosuppressive treatment, particularly the place of disease-modifying anti-rheumatic drugs (DMARDs), remains to be determined. The 2 largest case-series, of 32 and 60 patients, mainly described the efficacy of steroids alone [2], [3]. Among the 60 patients, 57 (95%) received steroids, with vestibulo-auditory and ophthalmological improvements in 58% [3].
However, no studies have compared the benefit of DMARDs added to steroids and the potential efficacy of various DMARDs. Recently, a few case reports demonstrated the benefit of biologic targeted treatments, mainly infliximab, but larger studies are needed to determine their benefit for vestibulo-auditory involvement [4], [5], [6], [7], [8] [9]. A recent literature review analyzed treatment strategies in 141 patients, including various DMARDs and biologics, suggesting the value of immunosuppressive drugs but could not compare the efficacy of various regimen [10].
In this largest French nationwide study and literature review, we report the long-term outcome of 62 patients with Cogan syndrome receiving steroids, DMARDs and/or tumor necrosis factor α (TNF-α) antagonists and compare their efficacy.
Section snippets
Materials and methods
We conducted a retrospective multicenter study in the French network of internal medicine (SNFMI) and the Club Rhumatisme and Inflammation (CRI) between July 2014 and 2016. Data were collected retrospectively from physicians in charge of patients. The physicians were asked to complete a standardized questionnaire online.
The inclusion criteria were 1) ocular signs consistent with non-syphilitic interstitial keratitis and/or conjunctivitis, episcleritis, scleritis or uveitis; 2) rapidly
Baseline characteristics of patients
We included 40 personal cases and 22 from the literature, for 62 patients (31 females; median age 37 years [range 2–76]). The median time from the first symptoms to diagnosis was 12 months [0 − 231]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, in particular, bilateral hearing loss for 41% and deafness for 31% (Supplementary Table 1). Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The median time between vestibulo-auditory and ocular
Discussion
In our series of patients with Cogan syndrome, sex ratio, age at diagnosis, and prevalence of vestibulo-auditory, ocular and systemic signs were similar to previous data from the Mayo Clinic and French nationwide series [2], [3]. Associated autoimmune diseases were present in 8% of patients from the Mayo Clinic and in 10% of our patients [21]. Only one previous large series compared features of typical and atypical Cogan syndrome, mainly showing more frequent joint and neurological involvements
Conclusions
In DMARDs and steroids-refractory Cogan syndrome, infliximab can lead to vestibulo-auditory response, with 80% frequency of improvement at 6 months. Prospective studies are warranted to confirm these data and determine the place of associated drugs in this rare condition.
The following are the supplementary data related to this article.
Acknowledgements
We thank the Club Rhumatismes et Inflammation (CRI) and the French National Society of Internal Medicine (SNFMI) for their help in the organization of this study.
Funding Sources
None.
Conflicts of interest
None.
Author contributions
All authors were involved in drafting the article. Arsene Mekinian had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of data analysis.
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