glucocorticoids, Drug class: The disease appears to result from an autoimmune reaction directed against an unknown common autoantigen in the cornea and inner ear. and transmitted securely. Paediatric Cogans syndrome - review of literature, case report and practical approach to diagnosis and management. Rheumatology (2011) 50:195568. Use OR to account for alternate terms your institution, https://doi.org/10.1016/s0025-6196(12)61951-x, https://doi.org/10.1016/j.autrev.2017.02.009, https://doi.org/10.1016/j.autrev.2017.10.005, https://doi.org/10.1016/j.autrev.2012.07.012, https://doi.org/10.1016/j.autrev.2014.01.002, https://doi.org/10.1016/S0140-6736(02)11028-2, https://doi.org/10.1093/rheumatology/keh228, https://doi.org/10.1007/s11926-015-0499-y, https://doi.org/10.3109/01676830.2013.764324, https://doi.org/10.1097/ICB.0000000000000201, https://doi.org/10.1590/1806-9282.63.12.1028, https://doi.org/10.1016/j.crohns.2010.09.003, https://doi.org/10.1016/j.athoracsur.2010.12.068, https://doi.org/10.1016/j.ijporl.2009.06.025, https://doi.org/10.1016/jpeds.2011.07.051, https://doi.org/10.1080/14712598.2019.1611779, https://doi.org/10.1007/s10165-012-0691-0. Vestibuloauditory symptoms can include sensorineural hearing loss Pathophysiology , tinnitus Tinnitus Tinnitus is a noise in the ears. Gasparovic H, Djuric Z, Bosnic D, Petricevic M, Brida M, Dotlic S, et al. We do not control or have responsibility for the content of any third-party site. Bedside head impulse tests (HITs) were positive for right horizontal semicircular canal. If you are responding to a comment that was written about an article you originally authored: One month later, his dizziness and eye injection improved markedly, while the hearing impairments and abnormal HITs persisted in both ears. Frontiers | Case Report: Effective and Safe Treatment With Certolizumab Copyright 2023 Merck & Co., Inc., Rahway, NJ, USA and its affiliates. Examination showed spontaneous nystagmus beating rightward and upward, which changed its direction into left-beating during leftward gaze. 'Orthopedic Surgeon'. 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 syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review. After childbirth the Appearance, Pulse, Grimace, Activity, Respiration (APGAR) score was assessed, and umbilical cord pH, neonatal weight, length and head circumference were measured. An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. 2011;92:3401. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 4. CRP values normalized, and tone audiograms showed stable or improved results compared to pre-CZP-treatment findings. Monoclonal anti-TNF--antibodies of IgG1 isotype are actively transported via the neonatal fragment crystallizable (Fc) receptor during the second and third trimester) (6). Use for phrases Autoimmun Rev. MeSH Cogan syndrome: a retrospective review of 60 patients throughout a half century. We aimed to determine the place of DMARDs and biologic-targeted treatments. Copyright 2021 Venhoff, Thiel, Schramm, Jandova, Voll and Glaser. The schedule may depend on the exact dosage form or strength of the medication. Vollertsen RS, McDonald TJ, Younge BR, et al. Cogan's syndrome and other ocular vasculitides. Google Scholar. An official website of the United States government. Copyright 2017. Ocular involvement includes any combination of the following: Bilateral interstitial keratitis Interstitial Keratitis Interstitial keratitis is chronic, nonulcerative inflammation of the mid-stroma (the middle layers of the cornea) that is sometimes associated with uveitis. Article The erythrocyte sedimentation rate (ESR, 120 mm/h) and C-reactive protein (CRP, 23.4 mg/dL) were elevated. Cogan's syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. No use, distribution or reproduction is permitted which does not comply with these terms. . Treatment Key Points Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. 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. Tarney CM, Wilson K, Sewell MF. Cogan's syndrome is a chronic (long-lasting) disease. Treatment is with corticosteroid eye drops and corticosteroid pills. Gabriela Mabel Espinoza. Cogan's syndrome: new therapeutic approaches in the biological era. Choi, and X. Yang analyzed and interpreted the data and revised the manuscript. Disclaimer. 2016 Jan;125(1):20-4. doi: 10.1177/0003489415595426. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. It can lead to deafness or blindness if untreated. 'Royal Free Hospital'. 1986;61:34461. Autoimmun Rev. Published by Elsevier B.V. -, Swiss Med Wkly. Espinoza GM, Wheeler J, Temprano KK, Keller AP. Subscribe to Drugs.com newsletters for the latest medication news, new drug approvals, alerts and updates. Proposed guidelines for the use of infliximab in CS are found in the last table of the review, in an attempt to define the proper timing for initiating infliximab treatment in order to avoid permanent disability. (C) Five weeks later, the patient shows a conjunctival and episcleral injection and diffuse stromal edema with keratic precipitates (black box) in both eyes suggesting interstitial keratitis. Arch Ophthalmol. 2018;4:1028. With a diagnosis of Cogan syndrome, he was placed on aspirin 100 mg, azathioprine 100 mg, and methylprednisolone 30 mg per day. The most frequent indications for CZP treatment in CRIB/CRADLE were rheumatoid arthritis, followed by Crohns disease, psoriatic arthritis and axial spondyloarthritis (7, 14). o [teenager OR adolescent ], , MD, FACS, Sidney Kimmel Medical College at Thomas Jefferson University. Has a currently accepted medical use in treatment in the United States. calcineurin inhibitors, Drug class: The .gov means its official. Furthermore, we have no data on CZP pharmacokinetics in the three infants and their mothers. Rcklov K, von Kalle T, Koitschev A, Gekeler K, Scheltdorf M, Heinkele A, Blankenburg F, Ktter I, Hospach A. Pediatr Rheumatol Online J. Infliximab could lead to vestibulo-auditory response in DMARDS and steroid-refractory Cogan syndrome, but prospective studies are necessary. Matteson EL, Choi HK, Poe DS, Wise C, Lowe VJ, Mcdonald TJ, et al. Bookshelf Retin Cases Brief Rep. 2015;9:3159. Screening for antinuclear antibodies (ANA), antiphospholipid antibodies (APA), rheumatoid factor (RF), anti-neutrophil cytoplasmic antibodies (ANCA) was negative at time of initial diagnosis and during follow-ups. Has a currently accepted medical use in treatment in the United States. The typical ocular picture is that of a non-syphilitic interstitial keratitis, while audiovestibular features resemble Meniere's disease. This article will review the varying clinical presentations of CS and emerging information of systemic disease associated with CS. The positional nystagmus did not respond to repeated CRMs. The patient also reported intermittent febrile sensation and myalgia for 2 months. Tax calculation will be finalised during checkout. However, one limitation of our report is the low number of patients included, with CS being a very rare disease. While the establishment of the diagnosis of Cogan's syndrome is challenging, so is its treatment. Authors Paolo Mora 1 , Giacomo Calzetti 2 , S Ghirardini 2 , P Rubino 2 , S Gandolfi 2 , J Orsoni 2 Affiliations Latent tuberculosis was ruled out by chest X-ray and interferon-gamma release assay (IGRA) prior to initiation of adalimumab. The .gov means its official. Google Scholar. doi:10.1136/annrheumdis-2017-212196. Recurrent episodes of inner ear disease might result in deafness. From the Department of Neurology (S.-U.L. Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C. Rom J Ophthalmol. Janatov D, Zelenk K, Komnek P, Matouek P. Int J Pediatr Otorhinolaryngol. No further disease manifestations were detectable. Mendis S, Longley N, Morley S, Korres G, Kaski D. Brain Sci. Epub 2022 Apr 14. Clowse ME, Frger F, Hwang C, Thorp J, Dolhain RJ, van Tubergen A, et al. Lancet. Accessibility Cogan's syndrome: an autoimmune inner ear disease - PubMed Cogan syndrome - UpToDate Front Pediatr. Epub 2019 May 6. Branislava I, Marijana T, Nemanja D, Dragan S, Maja Z. Atypical Cogans syndrome associated with coronary disease. Front. Cogan's Syndrome: Symptoms and Treatments - All About Vision Given the wide spectrum of audiovestibular findings and later development of ocular inflammation in our patient, Cogan syndrome should be suspected even in unilateral audiovestibulopathy of unknown cause, especially in the presence of systemic symptoms, elevated serologic markers for inflammation, and bilateral labyrinthine enhancements on MRIs. A novel therapeutic option in Cogan diseases? Cogan's syndrome (CS) is a rare chronic inflammatory disorder, classically characterized by interstitial keratitis and sensorineural hearing loss. Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience, and the risks involved in use in pregnant women clearly outweigh potential benefits. *Correspondence: Nils Venhoff, nils.venhoff@uniklinik-freiburg.de, Autoimmune Vasculitis: Advances in Pathogenesis and Therapies, View all Subjective tinnitus is perception of sound in the absence of an acoustic stimulus and is heard only by the read more , vertigo Dizziness and Vertigo Dizziness is an imprecise term patients often use to describe various related sensations, including Faintness (a feeling of impending syncope) Light-headedness Feeling of imbalance or unsteadiness read more , and/or a sensation of fullness or pressure. Vasculitis can be triggered by certain infections or drugs or can occur for unknown reasons. Unauthorized use of these marks is strictly prohibited. Cogan syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review. Otherwise, given the paroxysmal nature of the positional nystagmus during Dix-Hallpike maneuver, it may be ascribed to secondary BPPV due to labyrinthine inflammation. About 10 to 30% of patients also have severe systemic vasculitis, which may include life-threatening aortitis Aortitis Aortitis is inflammation of the aorta, sometimes causing aneurysm or occlusion. Curr Rheumatol Rep. 2015;17:24. https://doi.org/10.1007/s11926-015-0499-y. 2006;81:4838. The trusted provider of medical information since 1899, Reviewed/Revised Aug 2022 | Modified Sep 2022. Cogan Syndrome - an overview | ScienceDirect Topics The cornea is the clear layer in front of the iris and pupil. Is not subject to the Controlled Substances Act. The aorta is the largest artery of the body. 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. doi:10.1136/bcr-2018-227917, 10. A rare case of atypical Cogan's syndrome presenting as encephalitis. The most widely used treatment consists in oral prednisone, administered at 1-2 mg/kg/day, during the first 2 weeks (Vollertsen et al. An official website of the United States government. Two weeks before referral to our hospital, he developed hearing loss and fluctuating tinnitus in the right ear, and the positional vertigo changed into recurrent spontaneous vertigo lasting about 2 hours. Other pregnant patients in remission were successfully treated with hydroxychloroquine, or a combination therapy of either cyclosporine or intravenous immunoglobulin application (IVIG) with azathioprine and prednisolone (8, 9, 12). Recent findings: This article will review the varying clinical presentations of CS and emerging information of systemic . Cogan's syndrome (CS) is a rare autoimmune disease, characterized by ocular and vestibulo-auditory involvement. eCollection 2020. 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. Cogan's syndrome is inflammation of the blood vessels in the inner ears and eyes. Mora P, Calzetti G, Ghirardini S, et al. PDF Cogan's Syndrome: Clinical Presentations and Update on Treatment Re-start of CZP lead to complete serological and clinical remission without relevant side effects, which demonstrates the long-term efficacy and safety of CZP in this patient. He was discharged with a diagnosis of right labyrinthitis and concomitant BPPV involving the contralateral ear. 2019 Jul 8. Management of inflammatory diseases in general is particularly challenging in female patients of reproductive age. Received: 13 October 2020; Accepted: 30 November 2020;Published: 18 January 2021. Clin Exp Rheumatol. At the age of 27, patient (A) experienced several episodes of steroid-sensitive hearing loss accompanied by vertigo and tinnitus, as well as recurrent bilateral conjunctivitis and keratitis (Table 1). Life-threatening aortitis sometimes occurs. 2018:1498640. doi: 10.1155/2018/14986, PubMed Abstract | CrossRef Full Text | Google Scholar, 3. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. The anti-TNF monoclonal antibody Infliximab appears to be the most frequently used, leading to an improvement in hearing loss in 89% of the cases and allow corticosteroid tapering in 86% of the patients. Submissions must be < 200 words with < 5 references. It is composed of cells, protein, and fluid. Learn more about the MSD Manuals and our commitment to Global Medical Knowledge. https://doi.org/10.1093/rheumatology/keh228. Bithermal caloric tests showed right canal paresis (figure, A.b). The CRADLE study investigated CZP concentrations in human breast milk to estimate the daily dose of maternal CZP transferred to the infant by breast feeding (14). Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menire-like cochlear vestibular symptoms, which may also have systemic effects. Use to remove results with certain terms Federal government websites often end in .gov or .mil. 8600 Rockville Pike Data sources include IBM Watson Micromedex (updated 3 July 2023), Cerner Multum (updated 2 July 2023), ASHP (updated 11 June 2023) and others. Scharl M, Frei P, Fried M, Rogler G, Vavricka SR. Association between Cogans syndrome and inflammatory bowel disease: a case series. Cogan Syndrome - Eye Disorders - MSD Manual Professional Edition Results: Expand current row for information about methotrexate, for methotrexate to treat Cogan's Syndrome, Expand current row for information about prednisone, Expand current row for information about infliximab, Expand current row for information about cyclosporine, Expand current row for information about azathioprine, Expand current row for information about etanercept, Expand current row for information about cyclophosphamide, Expand current row for information about mycophenolate mofetil. COGAN'S SYNDROME - PMC - National Center for Biotechnology Information Cogan's syndrome: State of the art of systemic immunosuppressive treatment in adult and pediatric patients. Expert Opin Biol Ther. Cogan's Syndrome Symptoms, Doctors, Treatments, Advances & More - MediFind Careers. 2021 Jan 18;11:616992. doi: 10.3389/fimmu.2020.616992. ), Korea University Anam Hospital; Department of Neurology (S.-U.L., J.-S.K., J.-Y.C. DATA SOURCES: Studies reviewed were English language original articles ranging from 1990 to 2016 reporting data for subjects with CS (typical and atypical) undergoing any systemic treatment other than steroids alone. Medicine. Padoan R, Cazzador D, Pendolino AL, Felicetti M, De Pascalis S, Zanoletti E, Bovo R, Martini A, Schiavon F. Expert Opin Biol Ther. PMC The easiest way to lookup drug information, identify pills, check interactions and set up your own personal medication records. Cogan syndrome can affect different parts of the eye, including the cornea. Cogan's Syndrome - Harvard Health Select drug class All drug classes Alkylating agents (1) Antimetabolites (1) Antirheumatics (4) Antipsoriatics (1) Glucocorticoids (1) Calcineurin inhibitors (1) Tnf alfa inhibitors (2) Selective . https://doi.org/10.1016/j.autrev.2014.01.002. Indeed, persistent geotropic or apogeotropic nystagmus may be observed in labyrinthitis8,9 and has been ascribed to heavy or light cupula due to changes in the specific gravity of the endolymph from inflammation. Expanded Access is a potential pathway for a patient with a serious or immediately life-threatening disease or condition to gain access to an investigational medical product (drug, biologic, or medical device) for treatment outside of clinical trials when no comparable or satisfactory alternative therapy options are available. Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C. Rom J Ophthalmol. Case Report: Effective and Safe Treatment With Certolizumab Pegol in Pregnant Patients With Cogan's Syndrome: A Report of Three Pregnancies in Two Patients. The aorta is the largest artery of the body. Abstract Purpose of ReviewCogan's syndrome (CS) is a rare systemic vasculitis that can severely affect vision and hearing, which mayalso have significant systemic effects. Urgent evaluation by an ophthalmologist and otolaryngologist is indicated. Typical and atypical Cogan's syndrome: 32 cases and review of the Areas covered: Expert commentary: 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. Disclaimer. Of relevance, none of the patients in these two studies had Cogans syndrome or any other form of systemic vasculitis. The cause is usually infectious. read more , ocular redness, optic nerve edema, proptosis, or a combination of these symptoms. Background: antirheumatics, other immunosuppressants, For professionals: Use to remove results with certain terms What is the mechanism of action of infliximab? CZP-treatment was well tolerated without local reactions at the injection site or other clinically relevant adverse events. Bakalianou K, Salakos N, Iavazzo C, Danilidou K, Papadias K, Kondi-Pafiti A. https://doi.org/10.1007/s11882-020-00945-1, DOI: https://doi.org/10.1007/s11882-020-00945-1. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). 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. Symptoms vary depending read more that causes inflammation of the eyes, the ears, and sometimes the blood vessels (vasculitis Overview of Vasculitis Vasculitic disorders are caused by inflammation of the blood vessels (vasculitis). What triggers an autoimmune disorder is not known. HHS Vulnerability Disclosure, Help Shamriz O, Tal Y, Gross M. Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogans Syndrome. PubMed Central The site is secure. doi:10.1007/s10067-007-0664-4. Your role and/or occupation, e.g. Cogan syndrome is mainly treated with steroids. Department of Ophthalmology, Saint Louis University Eye Institute, Saint Louis University School of Medicine, 1755 South Grand Blvd., St. Louis, MO, 63104, USA, Gabriela Mabel Espinoza&Angela Prost Keller, Department of Internal Medicine, Saint Louis University School of Medicine, 1402 South Grand Blvd. Written informed consent was obtained from the individual for themselves and their child/children for the publication of any potentially identifiable images or data included in this article. Epub 2017 Feb 14. The patient also showed persistent apogeotropic nystagmus after head turning to either side while supine, more intense while turning the head to the right. Cogan's syndrome (CS) is a rare autoimmune disorder characterized by audiovestibular dysfunction and ocular inflammation. doi: 10.1055/a-1349-7475. Online ahead of print. Mycophenolate mofetil as a treatment of steroid dependent Cogan's syndrome in childhood. The https:// ensures that you are connecting to the Gluth MB, Baratz KH, Matteson EL, Driscoll CL. https://doi.org/10.1016/j.athoracsur.2010.12.068. Clipboard, Search History, and several other advanced features are temporarily unavailable. It has a currently accepted medical use in treatment in the United States. Clin Rheumatol. Accordingly, CZP is considered safe for the use in pregnant women (5). Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. ), Dizziness Center, Clinical Neuroscience Center, and Department of Neurology (J.-S.K., J.-Y.C. Please enable it to take advantage of the complete set of features! The original data generated and analyzed for this study are included in the published article. Unauthorized use of these marks is strictly prohibited. PubMed Nonspecific systemic complaints include fever, headache, joint pain, and myalgia. FOIA Currently, the patient is still under treatment with CZP to prevent relapse of disease. Venhoff N, Thiel J, Schramm MA, Jandova I, Voll RE, Glaser C. Front Immunol. your institution. The link you have selected will take you to a third-party website. If untreated, permanent vision and hearing loss can result. Before . PubMed Colodetti R, Spina G, Leal T, et al. Unauthorized use of these marks is strictly prohibited. The slightly overweight patient had stopped smoking years before; a mild hypothyroidism was adequately treated. HHS Vulnerability Disclosure, Help Hyon, Y.-J.
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