Clinical features and outcome of Cogan syndrome. It requires interdisciplinary clinical management and individualized treatment approaches by all specialists involved (24). Aortitis Secondary to Cogan's Syndrome: A Rare Cause of Fever of Unknown Origin. Has no currently accepted medical use in treatment in the United States. Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C. Rom J Ophthalmol. 2015 Jan-Mar;59(1):6-13. The patient showed spontaneous nystagmus beating leftward, downward, and counterclockwise (the upper poles of the eyes beating to the left shoulder). Cogan's syndrome is more than just keratitis: a case-based literature review. Successful treatment with tocilizumab in a case of Cogan's syndrome complicated with aortitis. Cogans syndrome (CS) is a very rare chronic autoimmune disease characterized by ocular inflammation and audiovestibular symptoms including tinnitus, vertigo and hearing loss (1). Pagnini I, Zannin ME, Vittadello F, Sari M, Simonini G, Cimaz R, et al. Choi, and X. Yang report no disclosures relevant to the manuscript. Additionally, CZP absorption by the infants via breast milk is unlikely because of its low oral bioavailability. -, Swiss Med Wkly. Drugs.com provides accurate and independent information on more than 24,000 prescription drugs, over-the-counter medicines and natural products. Diagnosis is by a doctor's evaluation and blood tests to rule out other diseases. Has a low potential for abuse relative to those in schedule 3. 'Royal Free Hospital'. Results of tone audiometric monitoring remained stable during follow-up. https://doi.org/10.1016/j.autrev.2014.01.002. Cogan's syndrome is more than just keratitis: a case-based literature List of 8 Cogan's Syndrome Medications Compared - Drugs.com BMJ Case Rep (2019) 12(10):e227917. Bithermal caloric tests showed bilateral canal paralysis (figure, B.b). 8600 Rockville Pike We are very happy to live a more or less normal life now as a family with two healthy and happy kids. Accordingly, CZP is considered safe for the use in pregnant women (5). Please enable it to take advantage of the complete set of features! Unauthorized use of these marks is strictly prohibited. Received: 13 October 2020; Accepted: 30 November 2020;Published: 18 January 2021. Though, we have no indication to expect results that are relevantly different to previously reported data in the CRIB and CRADLE studies. Cogan Syndrome - Eye Disorders - Merck Manuals Professional Edition We do not control or have responsibility for the content of any third-party site. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 4. The typical ocular picture is that of a non-syphilitic interstitial keratitis, while audiovestibular features resemble Meniere's disease. Ha, H.-J. Shivpuri A, Turtsevich I, Solebo AL, Compeyrot-Lacassagne S. Front Pediatr. Published by Elsevier B.V. In most breast milk samples no measurable CZP was detectable, indicating no to minimal CZP transfer from maternal plasma to breast milk. Disclaimer. Autoimmun Rev. The first-line treatment consisted of steroids alone (n=43; 70%) or associated with other immunosuppressive drugs (n=18; 30%). The fetal development was monitored by the patients gynecologists via ultrasound during every trimester of pregnancy. sharing sensitive information, make sure youre on a federal Cogan syndrome can present the features of benign paroxysmal positional vertigo (BPPV) initially, followed by sequential bilateral labyrinthitis, and finally ocular inflammation. NV and CG wrote the manuscript. Aortitis read more . At diagnosis, 61 patients (98%) had vestibulo-auditory symptoms, particularly bilateral hearing loss in 41% and deafness in 31%. Cogans syndrome a rare aortitis, difficult to diagnose but with therapeutic potential. Google Scholar. https://doi.org/10.1097/ICB.0000000000000201. The later development of ocular inflammation delayed the diagnosis of Cogan syndrome. Cogan Syndrome - an overview | ScienceDirect Topics Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Clowse ME, Frger F, Hwang C, Thorp J, Dolhain RJ, van Tubergen A, et al. 2022 Feb 24;12(3):306. doi: 10.3390/brainsci12030306. Mora P, Calzetti G, Ghirardini S, Rubino P, Gandolfi S, Orsoni J. Cogans syndrome: State of the art of systemic immunosuppressive treatment in adult and pediatric patients. Half of previously reported CS patients did not need any systemic immunosuppressive treatments throughout pregnancy, instead intermittent topical treatment with ophthalmic steroids was sufficient (1013). The mean age of all patients included in the CRIB and the CRADLE studies was approximately 30 years and therefore comparable to the CS patients reported here. NV, IJ, RV, and CG received payment (less than 5000) from UCB pharmaceuticals for advisory boards, lectures, or travel grants within the last five years. https://doi.org/10.1007/s10165-012-0691-0. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 3. Unable to load your collection due to an error, Unable to load your delegates due to an error. PubMed Careers. Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances. https://doi.org/10.1080/14712598.2019.1611779Comprehensive review of rising role of biologic agents in the treatment of Cogans syndrome. Dear Editor, We present the reported case of a 37-year-old male atypical Cogan's syndrome (CS) patient who was precisely diagnosed and clinically cured under our systematic therapeutic regime. J.-S. Kim designed and conceptualized the study, interpreted the data, and revised the manuscript. Web page addresses and e-mail addresses turn into links automatically. 2016 Jan;125(1):20-4. doi: 10.1177/0003489415595426. What triggers an autoimmune disorder is not known. Second-line treatment with TNF- blocking agent adalimumab was effective and resulted in normalization of CRP levels, however, was switched to CZP because of the patients desire to have children. Cogan's syndrome: new therapeutic approaches in the biological era Corticosteroids are the cornerstone of CS therapy at disease onset and during acute phases of the disease. However, immunosuppressive agentslike MTX, cyclophosphamide and mycophenolate are contraindicated for their teratogenicity during pregnancy. Cogan's Syndrome - Vasculitis Foundation Urgent evaluation by an ophthalmologist and otolaryngologist is indicated when Cogan syndrome is suspected. Allison S. Young, Brent McMonagle, David V. Pohl et al.Neurology, November 07, 2019, DOI: https://doi.org/10.1212/WNL.0000000000007733, Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome, Evidence of serum antibodies against inner ear tissues in the blood of patients with certain sensorineural hearing disorders, Cogan syndrome: pathogenesis, clinical variants and treatment approaches, Cogan's syndrome: 18 cases and a review of the literature, Cogan's syndrome: audiovestibular involvement and prognosis in 18 patients, Cogan syndrome: a retrospective review of 60 patients throughout a half century, Refractory positional vertigo with apogeotropic horizontal nystagmus after labyrinthitis: surgical treatment and identification of dysmorphic ampullae, Sudden sensorineural hearing loss with simultaneous positional vertigo showing persistent geotropic direction-changing positional nystagmus, Meniere's disease: prevalence of contralateral ear involvement, Comparison of caloric and head-impulse tests during the attacks of Meniere's disease, Superior semicircular canal dehiscence presenting with recurrent positional vertigo, Ictal downbeat nystagmus in Mnire disease, Neurology: Neuroimmunology & Neuroinflammation. Initially topical and sometimes systemic corticosteroids for ocular involvement. Typical and atypical Cogan's syndrome: 32 cases and review of the 2002 Sep 21;360(9337):915-21. doi: 10.1016/S0140-6736(02)11028-2. Federal government websites often end in .gov or .mil. 2002;360:91521. Mariette X, Frger F, Abraham B, Flynn AD, Molt A, Flipo RM, et al. Lines and paragraphs break automatically. J Matern Fetal Neonatal Med (2009) 22:92830. PubMed Epub 2009 Aug 4. The uncomplicated pregnancies and the normal development of the children afterwards, without any infectious or other complications, support our hypothesis that the infants were exposed to relevant CZP doses during pregnancy or lactation period. National Library of Medicine PubMed A novel therapeutic option in Cogan diseases? Vasculitis - NHS Background: sharing sensitive information, make sure youre on a federal Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C. Rom J Ophthalmol. Results: Cogans syndrome: new therapeutic approaches in the biological era. Treatment is typically with corticosteroids (topical and systemic). Cogan syndrome--pathogenesis, clinical variants and treatment approaches. There are now just over 300 cases of CS reported in the literature, although this is likely an underrepresentation of the true impact of this disease as it can easily go undiagnosed. Vestibuloauditory symptoms can include sensorineural hearing loss Pathophysiology , tinnitus Tinnitus Tinnitus is a noise in the ears. Management of inflammatory diseases in general is particularly challenging in female patients of reproductive age. Clipboard, Search History, and several other advanced features are temporarily unavailable. Monoclonal anti-TNF--antibodies of IgG1 isotype are actively transported via the neonatal fragment crystallizable (Fc) receptor during the second and third trimester) (6). Scharl M, Frei P, Fried M, Rogler G, Vavricka SR. Association between Cogans syndrome and inflammatory bowel disease: a case series. This site needs JavaScript to work properly. Epub 2019 May 6. However, the evidence of clearly effective or preferable treatment options for CS remains lacking, mostly due to the rarity of the disease and to the consequent difficulty in organizing high-quality prospective trials. Areas covered: CZP-treatment was well tolerated without local reactions at the injection site or other clinically relevant adverse events. 0 Shop NowFind Eye Doctor Conditions Conditions Eye Conditions, A-Z Eye Conditions, A-Z Vasculitis can be triggered by certain infections or drugs or can occur for unknown reasons. On the one hand, adequate disease control at conception and during pregnancy is crucial to ensure maternal and fetal health, on the other hand, available treatment options are limited because of potential ovarian or fetal toxicity. Cogan's syndrome: an autoimmune inner ear disease - PubMed CRP values normalized, and tone audiograms showed stable or improved results compared to pre-CZP-treatment findings. I hope that this report will help and support other physicians and especially young female patients with Cogans syndrome. Brought to you by Merck & Co, Inc., Rahway, NJ, USA (known as MSD outside the US and Canada) dedicated to using leading-edge science to save and improve lives around the world. Cogan's Syndrome Symptoms, Doctors, Treatments, Advances & More - MediFind Cogan syndrome is a rare autoimmune syndrome affecting mainly the ocular and vestibuloauditory systems. All children were born healthy, at term, and developed regularly during follow-up. Lee analyzed and interpreted the data and wrote the manuscript. Copyright 2023 Merck & Co., Inc., Rahway, NJ, USA and its affiliates. doi:10.1007/s10067-007-0664-4. A potentially grave disorder of audiovestibulopathy with many faces. Cogan's syndrome can lead to vision difficulty, hearing loss and dizziness. Introduction: Accordingly, with its neglectable to minimal transplacental transfer in the third trimester of pregnancy (7), CZP was a particularly attractive second line therapy to avoid inflammatory flares and thereby protect mother and her unborn child. Deliveliotou A, Moustakarias T, Argeitis J, Vaggos G, Vitoratos N, Hassiakos D. Successful full-term pregnancy in a woman with Cogans syndrome: a case report. 2023 May 12;23(1):212. doi: 10.1186/s12886-023-02966-6. Data sources include IBM Watson Micromedex (updated 3 July 2023), Cerner Multum (updated 2 July 2023), ASHP (updated 11 June 2023) and others. Most people with Cogan's Syndrome will need treatment with moderately high doses of prednisolone or other types of steroids. Introduction. At admission, the body temperature was 38.4C. The https:// ensures that you are connecting to the Careers. This question is for testing whether or not you are a human visitor and to prevent automated spam submissions. 2017;16:38590. Treatment is typically with corticosteroids (topical and systemic). MeSH This study followed the tenets of the Declaration of Helsinki and was performed according to the guidelines of the Institutional Review Board of Seoul National University Bundang Hospital (B-1807-480-701). Pediatric uveitis: Role of the pediatrician. Before, during and after pregnancy the activity of CS was regularly monitored by CRP serum concentrations, along with clinical assessments including body weight, blood pressure and pulse rate, subjective hearing levels and tone audiometry, if necessary. 2021 Jan 18;11:616992. doi: 10.3389/fimmu.2020.616992. Sometimes people are treated with other drugs such as cyclophosphamide, methotrexate, cyclosporine, rituximab, or infliximab. Ocular symptoms include irritation, pain, photophobia, and decreased vision. J Pediatr. Eye pain, decreased vision, increased sensitivity to bright light, and redness of the eye are common symptoms. Symptoms come and go, or respond well to treatment, but usually the disease is prolonged or even lifelong. The appropriate timing of Infliximab treatment has yet to be thoroughly investigated, but it seems to be more effective when started at an early stage of the disease. antirheumatics, TNF alfa inhibitors, Drug class: Cogan's syndrome is more than just keratitis: a case-based literature review. Furthermore, TNF- inhibitors are often discontinued after the first trimester of pregnancy to limit placental transfer of the drug to the fetus (5). Greco A, Gallo A, Fusconi M, Magliulo G, Turchetta R, Marinelli C, et al. Cogan's syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. Kim, J.-J. Conventional immunosuppressive therapy, such as methotrexate, could be used in relapsing patients or as a glucocorticoid sparing agent, but efficacy is often modest. Curr Opin Otolaryngol Head Neck Surg. Cogan's syndrome is inflammation of the blood vessels in the inner ears and eyes. https://doi.org/10.1002/lio2.205Nicely delineated treatment options for autoimmune inflammation of the inner ear that is a hallmark of Cogans syndrome. Objective: Google Scholar. Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. Drugs used to treat Cogan's Syndrome The following list of medications are in some way related to or used in the treatment of this condition. The link you have selected will take you to a third-party website. Cite this article. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). Use to remove results with certain terms Google Scholar. During left Dix-Hallpike maneuver, he shows paroxysmal nystagmus beating upward and counterclockwise.Download Supplementary Video 1 via http://dx.doi.org/10.1212/007733_Video_1. This site needs JavaScript to work properly. Cogan's syndrome: new therapeutic approaches in the biological era. Submissions must be < 200 words with < 5 references. The https:// ensures that you are connecting to the Submissions should not have more than 5 authors. Neither the child nor the mother had any postpartum complications. To treat deeper ocular inflammation and to treat vestibuloauditory symptoms before they become permanent, prednisone 1 mg/kg orally once a day is begun as soon as possible and continued for 2 to 6 months. Results and conclusions: Cogan's syndrome is a rare autoimmune vasculitis, and its pathogenesis is unknown. Treatment consists of systemic glucocorticoids and other immunosuppressive agents including methotrexate, cyclophosphamide and TNF--inhibitors. 2018;4:1028. Cogan's syndrome (CS) is a rare chronic inflammatory disorder, classically characterized by interstitial keratitis and sensorineural hearing loss. Shahid FL, Mukherjee R, Knapp C. Cogans syndrome associated with orbital inflammation. 1945;33:1449. BMC Ophthalmol. 2012;160:3037. National Library of Medicine No further disease manifestations were detectable. He was discharged with a diagnosis of right labyrinthitis and concomitant BPPV involving the contralateral ear. Cogans Syndrome: Clinical Presentations and Update on Treatment. Cogan's syndrome is a rare autoimmune vasculitis, with unknown pathogenesis. 8600 Rockville Pike It is characterised by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. Menieres disease complicated by recurrent interstitial keratitis: excellent result following cervical ganglionectomy. Atypical Cogan's syndrome: a case report and summary of current treatment options. The original data generated and analyzed for this study are included in the published article. Cogans syndrome is a systemic chronic inflammatory disease which can lead to severe functional visual and hearing impairments. 2017 Sep;16(9):903-910. doi: 10.1016/j.autrev.2017.07.003. A diastolic heart murmur may be present when aortitis is significant. Some clinicians add cyclophosphamide, methotrexate, cyclosporine, infliximab, or rituximab for recalcitrant cases. higgs-boson@gmail.com. The patient shows persistent apogeotropic nystagmus after head turning to either side while supine, which is more intense when turning the head to the right, mimicking left horizontal canal cupulolisthiasis. Learn about its symptoms, causes and various ways to treat it. You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid Systematic review methodology: The CRIB study in pregnant women receiving CZP for approved indications showed no quantifiable CZP concentrations in the neonates at time of delivery and during follow-up, indicating zero to minimal placental transfer or fetal exposure during the third trimester (7). Untreated disease may lead to corneal scarring and vision loss and, in 60 to 80% of patients, permanent hearing loss Hearing Loss Worldwide, about half a billion people (almost 8% of the world's population) have hearing loss ( 1). Hazes JM, Coulie PG, Geenen V, Vermeire S, Carbonnel F, Louis E, et al.
Oak Hills Basketball Roster, Articles C