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

• Published in: Autoimmunity reviews Vol. 16; no. 12; pp. 1219 - 1223
• Main Authors: Durtette, Charlotte, Hachulla, Eric, Resche-Rigon, Matthieu, Papo, Thomas, Zénone, Thierry, Lioger, Bertrand, Deligny, Christophe, Lambert, Marc, Landron, Cédric, Pouchot, Jacques, Kahn, Jean Emmanuel, Lavigne, Christian, De Wazieres, Benoit, Dhote, Robin, Gondran, Guillaume, Pertuiset, Edouard, Quemeneur, Thomas, Hamidou, Mohamed, Sève, Pascal, Le Gallou, Thomas, Grasland, Anne, Hatron, Pierre-Yves, Fain, Olivier, Mekinian, Arsène
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.12.2017; Elsevier
• Subjects: Adolescent; Adult; Aged; Antirheumatic Agents - therapeutic use; Child; Child, Preschool; Cogan Syndrome - drug therapy; Cogan Syndrome - epidemiology; Cogan's syndrome; Female; Humans; Immunology; Infliximab; Infliximab - therapeutic use; Keratitis - drug therapy; Life Sciences; Male; Middle Aged; Outcome; Retrospective Studies; Treatment; Treatment Outcome; Tumor Necrosis Factor-alpha - antagonists & inhibitors; Young Adult; Cogan's syndrome; Treatment; Infliximab; Outcome
• ISSN: 1568-9972; 1568-9972; 1873-0183
• DOI: 10.1016/j.autrev.2017.10.005

Cogan syndrome is mainly treated with steroids. We aimed to determine the place of DMARDs and biologic-targeted treatments. 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. 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). Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary. •Vestibulo-auditory response was similar with DMARDs and steroids alone.•Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome.•The 5 and 10-years incidence of relapse increased under steroids and DMARDS, while it stayed stable with infliximab.