THU0385 Cogan syndrome: A portuguese single center cohort report

Abstract

Background Cogan syndrome is a chronic inflammatory disease, of unknown origin, characterized by vestibuloauditory symptoms and inflammatory ocular manifestations with variable risk of developing into a systemic disease. Because it is a rare disease, with a variety of manifestations, the diagnosis, treatment and follow-up of patients with Cogan syndrome can be a challenge as is evident in the presented cases. Objectives To report the clinical experience of a single center in the disease course and manifestations of patients affected by Cogan syndrome. Methods Chart review, from 1985 to 2011, of all patients diagnosed as having Cogan syndrome at the Rheumatology outpatient clinic of Santa Maria Hospital, Lisbon. Results Nine patients were identified as having Cogan syndrome, 5 men (55,5%) and 4 women (45,5%), with mean age of 45.7 years [range 36-64 years]. The first disease manifestations started at a mean age of 32.7 years [range 17-46 years]. The patients were followed up for an average of 13,1 years [range, 3-21 years]. The most common initial disease manifestations were sudden hearing loss (77,8%) and vestibulary symptoms (77,8%), followed by ophthalmologic manifestations (66,7%). In most cases, both vestibuloauditory and ophthalmologic symptoms were present within a few weeks to months of the initial disease manifestation. The most common inflammatory ophthalmologic condition noted was interstitial keratitis (66,7%) and uveitis (anterior uveitis 44,4% and panuveitis 11,1%). Hearing loss (66.7%) and vertigo (66.7%) were the most common otolaryngologic symptoms. Hearing loss was bilateral in 8 cases (88.9%) and unilateral in 1 case (11.1%). Fever (50%) and arthralgia/arthritis (50%) were the most frequently encountered systemic manifestations. There was no evidence of neurological, cardiac or great vessel involvement. During the disease course most patients were treated with glucocorticoids (88,9%) and other immunosuppressive therapies (methotrexate 33.3% and oral cyclophosphamide 11.1%). Four patients needed high doses of prednisone (40 to 60 mg id) and one patient was admitted for glucocorticoids pulse therapy and plasmapheresis. This last patient died of cardiopulmonary arrest, attributed to the effects of Cogan syndrome, two years later. In the last follow up visit, 6 patients (75%) were under immunosuppressive therapy (22.2% methotrexate and 44,4% glucocorticoids), with a stable disease activity. The other two (without this treatment) showed no signs of disease progression. Complete hearing loss was noted in 33.3% of the affected patients, whereas permanent loss of any degree of vision was uncommon. A total of 2 patients underwent cochlear implantation. Conclusions In our small cohort of patients the major disease-related morbidities were due to vestibuloauditory manifestations. Aside from one patient death, there was a good response to the immunosuppressive treatment instituted. More advances in the understanding of Cogan syndrome different manifestations and appropriate treatments are vital to preven...