[Recurrent Corneal Erosions in Epithelial Corneal Dystrophies]. / Rezidivierende Hornhauterosion bei epithelialen Hornhautdystrophien.

The corneal epithelium is the most important structure of the ocular optical system. Recurrent corneal erosions can result from inflammation, trauma, degeneration and dystrophies. Epithelial basement membrane dystrophy (EBMD), epithelial recurrent erosion dystrophy (ERED) and Francheschetti and Meesmann's epithelial corneal dystrophy (MECD) can all - besides other signs and symptoms - result in more or less frequent corneal erosions. The pathomechanisms involved however are different. In EBMD, corneal erosions are facultative and clinical signs are often subtle. Aberrant basement membrane structures are associated with thinning of the epithelium and can be clinically identified as maps or fingerprints. In ERED, recurrent corneal erosions are - predominantly in the first decades of life - always present. A defect in the COL17A1 gene results in a dysfunctional hemidesmosome. In MECD, punctate corneal erosions are less frequent and result from intraepithelial microcysts which open spontaneously onto the ocular surface. Usually lubricants, therapeutic contact lenses and sometimes epithelial debridement and phototherapeutic keratectomy are the mainstay for treating corneal erosions in these three dystrophies.

Cogan syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review.

BACKGROUND: Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. PATIENTS AND METHODS: We conducted a French nationwide retrospective study of patients with Cogan syndrome (n=40) and a literature review of cases (n=22) and analyzed the efficacy of disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor α (TNF-α) antagonists. RESULTS: We included 62 patients (31 females) (median age 37years [range 2-76]. At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Ocular signs were present in 57 patients (92%), with interstitial keratitis in 31 (51%). The first-line treatment consisted of steroids alone (n=43; 70%) or associated with other immunosuppressive drugs (n=18; 30%). Overall, 13/43 (30%) and 4/18 (22%) patients with steroids alone and with associated immunosuppressive drugs, respectively (p=0.8), showed vestibulo-auditory response; 32/39 (82%) and 15/19 (79%) ocular response; and 23/28 (82%) and 10/14 (71%) general response. Overall 61 patients had used a total of 126 lines of treatment, consisting of steroids alone (n=51 lines), steroids with DMARDs (n=65) and infliximab (n=10). Vestibulo-auditory response was significantly more frequent with infliximab than DMARDs or steroids alone (80% vs 39% and 35%, respectively), whereas ocular, systemic and acute-phase reactant response rates were similar. Infliximab was the only significant predictor of vestibulo-auditory improvement (odds ratio 20.7 [95% confidence interval 1.65; 260], p=0.019). CONCLUSION: Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary.

COGAN'S SYNDROME.

OBJECTIVES: The objective of our study was to review the current knowledge on Cogan's syndrome, including etiology, diagnosis and treatment. Systematic review methodology: Relevant publications on Cogan's syndrome from 1945 to 2014 were studied. CONCLUSIONS: Cogan's syndrome is a rare autoimmune vasculitis, with unknown pathogenesis. Infection was thought to have played a role in the pathogenesis of the disease, but now the autoimmunity hypothesis is considered more likely to be true. Cogan's syndrome is characterized by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. Approximately 70% of the patients have systemic disease, of which vasculitis is considered the pathological mechanism. Corticosteroids are the first line of treatment; multiple immunosuppressive drugs were also used with varying degrees of success. The novelty in the treatment of the disease is tumor necrosis factor (TNF)-alpha-blockers, but more studies are necessary to establish their efficacy.

Cogan syndrome: an analysis of reported neurological manifestations.

BACKGROUND: Patients with Cogan syndrome typically present with nonsyphilitic interstitial keratitis and acute onset of sensorineural hearing loss. Neurological manifestations have been reported, but various frequencies and mechanisms have been proposed. REVIEW SUMMARY: We critically reviewed the English literature of Cogan syndrome to determine the nature, frequency, and most likely mechanisms of its neurological manifestations. CONCLUSIONS: On the basis of our review, we believe that Cogan syndrome can be associated with neurological manifestations. Our conclusion is based on reported tissue evidence of vasculitis involving the dura, brain, optic nerve, cochleovestibular nerve, and muscle, in patients with referable symptoms. However, we believe that the frequency of neurological manifestations may have been over reported due to lack of confirmatory testing in many of these cases.