Charlotte Durtette1, Eric Hachulla2, Matthieu Resche-Rigon3, Thomas Papo4, Thierry Zénone5, Bertrand Lioger6, Christophe Deligny7, Marc Lambert2, Cédric Landron8, Jacques Pouchot9, Jean Emmanuel Kahn10, Christian Lavigne11, Benoit De Wazieres12, Robin Dhote13, Guillaume Gondran14, Edouard Pertuiset15, Thomas Quemeneur16, Mohamed Hamidou17, Pascal Sève18, Thomas Le Gallou19, Anne Grasland19, Pierre-Yves Hatron2, Olivier Fain1, Arsène Mekinian20. 1. AP-HP, Hôpital Saint-Antoine, service de médecine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Sorbonne Universités, UPMC University, Paris 06, F-75012, Paris, France. 2. Service de Médecine Interne et Immunologie Clinique, CHU Lille, Centre national de référence maladies systémiques et auto-immunes rares, Université de Lille, 59037 Lille cedex, France. 3. AP-HP, Hôpital Saint-Louis, service de Biostatistiques, UPMC Univ, Paris 07, F-75011, Paris, France. 4. AP-HP, service de médecine interne, Hôpital Bichat-Claude Bernard, Paris, France. 5. Service de médecine interne, CH Valence, Valence, France. 6. Service de médecine interne, CHU Tours, Université de Tours, Tours, France. 7. Service de médecine interne, CHU Martinique, Fort de France, France. 8. Service de médecine interne, CHU Poitiers, Poitiers, France. 9. Service de Médecine Interne, Hôpital européen Georges-Pompidou, Assistance Publique Hôpitaux de Paris - APHP, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. 10. Service de médecine interne, Hôpital Foch, 92150 Suresnes, Université Versailles Saint Quentin en Yvelines, France. 11. Service de médecine interne et Maladies vasculaires, Centre de compétence de Maladies rares, CHU Angers, Université Angers, 49000 Angers, France. 12. Service de médecine interne, CHU Nîmes, Nîmes, France. 13. Service de médecine interne, Université Paris 13, AP-HP, Avicenne, 93000 Bobigny, France. 14. Service de médecine interne, CHU Limoges, Limoges, France. 15. Service de Rhumatologie, Centre Hospitalier René Dubos, Pontoise, France. 16. Service de médecine interne et de néphrologie, Hôpital de Valenciennes, Valenciennes, France. 17. Hôpital Hôtel Dieu, service de médecine interne, Université de Nantes, Nantes, France. 18. Service de médecine interne, Hôpital de la Croix-Rousse, Université de Lyon, Lyon, France. 19. AP-HP, service de médecine interne, Hôpital Louis Mourier, Colombes, France. 20. AP-HP, Hôpital Saint-Antoine, service de médecine interne and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Sorbonne Universités, UPMC University, Paris 06, F-75012, Paris, France. Electronic address: arsene.mekinian@aphp.fr.
Abstract
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.
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.
Authors: José Luis Treviño González; German A Soto-Galindo; Rafael Moreno Sales; Josefina A Morales Del Ángel Journal: Ann Med Surg (Lond) Date: 2018-04-30
Authors: Nils Venhoff; Jens Thiel; Markus A Schramm; Ilona Jandova; Reinhard E Voll; Cornelia Glaser Journal: Front Immunol Date: 2021-01-18 Impact factor: 7.561