Comparison of the distribution of clinical features between reported cases of typical Cogan's syndrome and atypical Cogan's syndrome shows that patients having atypical Cogan's syndrome present musculoskeletal and neurological manifestations and lymphadenopathy significantly more frequently, but this may well be a consequence of reporting bias in the literature. Malik MU, Pandian V, Masood H, Diaz DA, Varela V, Davalos-Balderas AJ, et al. However, other infectious or inflammatory causes of ocular symptoms or vestibular symptoms need to be excluded (Table 2) [27,28,29,30,31,32]. Anti-68 kDa antibodies in autoimmune sensorineural hearing loss: are these autoantibodies really a diagnostic tool? Neurology. In summary, CS should be part of the differential diagnosis of keratitis, especially in the absence of predisposing factors to infection. Treatment of Cogan's syndrome with FK 506: a case report. In the same year, sudden hearing loss (50 decibels, high frequency) occurred in the left ear, accompanied by dizziness, nausea, and vomiting. Introduction Cogan's syndrome was first described in 1945 by an ophthalmologist, Dr David G. Cogan, who reported on a syndrome of non-syphilitic interstitial keratitis (IK) and vestibuloauditory symptoms that resembled Meniere's disease [1]. Cogan syndrome - Wikipedia An unusual etiology of urticarial vasculitis. WebCogan's Syndrome. https://doi.org/10.1161/circulationaha.117.030423. Antibodies directed against a corneal antigen or the constituents of the inner ear have been detected by some authors [4, 16, 46, 52]. COGAN'S SYNDROME - PubMed The hallmark features include: Interstitial keratitis (blood vessels grow into the cornea). Cogan syndrome: Descriptive analysis and clinical [2] published in 1980, which reported on 111 patients with Cogan's syndrome (typical, n = 78; atypical, n = 33), we were able to identify in the English and French literature 79 additional patients with either typical (n = 35) [1439] or atypical (n = 44) [411, 4075] Cogan's syndrome with sufficient details to be analysed. The presenting manifestations were ocular in seven patients (four typical Cogan's syndrome, three atypical Cogan's syndrome), audiovestibular in 15 (seven typical Cogan's syndrome, eight atypical Cogan's syndrome) and articular in two (one typical Cogan's syndrome, one atypical Cogan's syndrome). [3], with a mean follow-up duration of 22 months, an unfavourable disease course was observed in 49 (63%): 34 patients developed deafness, 12 diffuse vasculitis, 11 aortic insufficiency and six blindness, and seven patients had died. Webthe chronic eye inflammation with scarring of the cornea. Slit lamp examination showed signs of keratitis. 2003;290(14):187583. Privacy Two patients (patients 7 and 21) were tested for anti-cochlear antibodies and one had a positive result. Part of Treatment with prednisone (1 mg/kg/day) elicited a marked clinical response. Allen NB, Cox CC, Cobo M et al. Boulinguez S, Bernard P, Bedane C, Lasudry J, Labrousse F, Bonnetblanc JM. M, male; F, female; IK, interstitial keratitis; SK, superficial keratitis; Me, Meniere-like syndrome; 1, unilateral; 2, bilateral. DMARDs and biologics have been used to treat ocular and systemic symptoms of CS. Autoantibodies to inner ear and endothelial antigens in Cogans syndrome. Article Tofacitinib for the treatment of antineutrophil cytoplasm antibody-associated vasculitis: a pilot study. Buge A, Chamouard JM, Michon C et al. In the remaining eight patients (five typical Cogan's syndrome, three atypical Cogan's syndrome) the ocular and audiovestibular manifestations were concomitant. How is it diagnosed? Temporal bone findings in Cogan's syndrome. Colodetti R, Spina G, Leal T, Oliveira M, Jr., Soeiro A. Cogan's syndrome - A rare aortitis, difficult to diagnose but with therapeutic potential. Temporal bone pathology in a case of Cogan's syndrome. 2023 BioMed Central Ltd unless otherwise stated. Cundiff J, Kansal S, Kumar A, Goldstein DA, Tessler HH. Beltagy A, Eshak N, Abdelnabi MH, Almaghraby A, Magdy S, Shehata H. Aortic valve perforation in the setting of Cogans syndrome. Cogan syndrome: a retrospective review of 60 patients throughout a half century. PubMed Plasma exchange is frequently used for neuroimmune diseases such as multiple sclerosis, acute and chronic inflammatory demyelinating neuropathy, or refractory myasthenia gravis. One month later, her inflammatory indicators decreased, but her hearing and joint symptoms did not ease. Kamakura T, Lee DJ, Herrmann BS, Nadol JB Jr. Histopathology of the Human Inner Ear in the Cogan Syndrome with Cochlear Implantation. https://doi.org/10.1111/echo.14428. WebMaking a diagnosis There are no specific diagnostic tests. We report our experience of a multicentre series of 32 patients with either typical or atypical Cogan's syndrome, which we combined with a detailed review of the literature. WebDiagnosis Treatment Key Points Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. 2013;23(3):57781. Maikap D, Pradhan A, Padhan P. A rare case of atypical Cogans syndrome presenting as encephalitis. Arthritis Rheum. Calopa M, Marti T, Rubio F, Peres J. Imagerie par resonance magnetique et syndrome de Cogan. A shifty diagnosis: Cogans syndrome. A case report and review of The dose of glucocorticoids was gradually reduced and a tumor necrosis factor (TNF)- inhibitor (etanercept 25mg, 2 times/week) was administered. J Fr Ophtalmol. Although successful responses can be achieved, relapses are common due to long-term glucocorticoid use, and side effects can occur. Computed tomographic scanning may provide evidence of localized cerebral ischaemia with infarction [20, 55] and MRI may also show multiple lesions of the white matter consistent with cerebral vasculitis [20]. Secondary neovascularization may frequently ensue [3]. Cogans syndrome is more than just keratitis: a case-based Your US state privacy rights, Durtette C, Hachulla E, Resche-Rigon M, Papo T, Zenone T, Lioger B, et al. The diagnosis of CS can be elusive due to the variability of clinical presentations. Only two patients (one typical Cogan's syndrome, one atypical Cogan's syndrome) experienced no hearing deficit. Vestibulo-auditory signs and symptoms include Matteson EL, Fabry DA, Facer GW, Beatty CW, Driscoll CL, Strome SE, et al. WebCogan syndrome is a rare autoimmune disease involving the eye and the inner ear. The microscopic lesions are diverse and non-specific. Late onset Cogan's syndrome. When vasculitis at other sites is suspected, it should be recommended to complete MRA, angiography, and even positron emission tomography combined with computed tomography (PET-CT) [34, 35]. Cogans Syndrome: Symptoms and Treatments - All About Cogan's syndrome. Patients may also present with myalgias, and muscle biopsy may reveal vasculitis or evidence of myositis [3, 4, 24, 69]. Ocular manifestations of CS tend to respond well to corticosteroids and disease-modifying anti-rheumatic drugs (DMARDs) [5]. 2019;93(1):3941. Cogan Syndrome - Eye Disorders - Merck Manuals Consumer Version Haynes et al. Wang, Y., Tang, S., Shao, C. et al. 2007;40(1):738. The diagnosis of CS is based upon presence of characteristic Since the literature review by Haynes et al. Cogan Syndrome - Eye Disorders - MSD Manual Professional Edition Ocular lesions did not progress. In contrast to the initial stage, routine diagnostic techniques sometimes fail to indicate progression in the chronic stage. CS should be involved in the differential diagnosis of keratitis. WebDiagnosis While the white blood cell count, erythrocyte sedimentation rate, and C-reactive protein tests may be abnormal and there may be abnormally high levels of platelets in the Most of the patients underwent extensive investigations looking for an infection at the time of disease onset. Ennouri A, Moalla K, Ferjaoui M, Marrekchi H, Atallah M. Le syndrome de Cogan. WebDiagnosis Treatment Cogan syndrome is a rare autoimmune disease that can affect the cornea. J Immunol Res. Covelli M, Lapadula G, Pipitone V. Cogan's syndrome: unsuccessful outcome with early combination therapy. Management of Cogan's syndrome. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Most commonly, after an initial flare lasting several weeks or months, the condition becomes chronic and progresses slowly. WebCogan syndrome (CS) is a chronic inflammatory disorder that most commonly affects young adults. The diagnosis is made on clinical examination and history where a combination of problems in the eyes and inner ears are Dekker JJ, Dinant HJ, Van Soesbergen RM. Cogan's syndrome Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. 2010;72(5):2759. In most cases, both eyes are affected during the disease course, with great variability in symptoms from one eye to the other and from day to day. Semeraro F, Russo A, Duse S, Romano V, Costagliola C. Retinal angiomatosis and cystoid macular oedema in Cogans syndrome. Benitez JT, Arsenault MD, Licht JM, Cohen SD, Greenberg RV. All authors read and approved the final manuscript. Andres T, Lapeyre G, Chan H, Saunier V, Coste V, Touboul D. Cogans syndrome. WebAbstract Objectives: Systematic review methodology: Relevant publications on Cogan's syndrome from 1945 to 2014 were studied. Syndrome de Cogan chez un adulte dage mur. The disease course of Cogan's syndrome is variable. Corticosteroids are first-line treatment. Since the initial description of Cogan's syndrome, its definition has been debated. She first developed earplugs and tinnitus in November 2009, which eased after rest. Cogan syndrome: Characteristics, outcome and treatment Abstract Summary: Cogan syndrome is an uncommon disorder of unknown etiology characterized by vestibuloauditory dysfunction and nonsyphilitic interstitial keratitis. The same antibodies also bound to connexin 26, which is implicated in congenital deafness. Lymphadenopathy in one patient with atypical Cogan's syndrome. The IK was isolated in 13 patients and associated with conjunctivitis in three patients or a superficial keratitis in two. Moreover, the presence of autoantibodies against inner ear and endothelial antigens found in some patients with Cogan's syndrome adds further evidence for the autoimmune nature of this disease [4, 16, 28, 46, 52, 77]. Ann Rheum Dis. Provided by the Springer Nature SharedIt content-sharing initiative. Immunol Lett. An audiogram revealed a pattern of sensorineural hearing loss. Lack of response to corticosteroids and pulse cyclophosphamide therapy in Cogan's syndrome. [2] and including our series, 28/52 (54%) and 22/59 (37%) with typical and atypical Cogan's syndrome respectively were deaf in both ears. Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P, et al. Am J Ophthalmol Case Rep. 2021;24:101215. https://doi.org/10.1016/j.ajoc.2021.101215. Reiter L, Szasz J, Wechsler IB, Walls RS. Meningism may occur in patients with Cogan syndrome. However, alopecia and poliosis do not. Granulomatosis with polyangiitis: Granulomatosis with polyangiitis often involves the ear and eye. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. https://doi.org/10.1007/s00405-004-0738-8. Article An overlap syndrome with features of atypical Cogan syndrome and Wegener's granulomatosis. https://doi.org/10.1016/j.amjoto.2005.07.006. There were no serious infections other than occasional mild cough and diarrhoea. Patients reported with atypical Cogan's syndrome had systemic manifestations more commonly than those with typical Cogan's syndrome. Surveying for infections is usually negative, but evidence for Chlamydia infection has been reported in a few cases [2, 62]. Cite this article. A propos dune observation. Infliximab is emerging as a potential first-line therapy in combination with corticosteroids. Optical coherence tomography (OCT) showed signs of optic disc oedema and macular degeneration. Kontorinis G, Giourgas A, Neuburger J, Lesinski-Schiedat A, Lenarz T. Long-term evaluation of Cochlear implantation in Cogan syndrome. A comparison of the clinical manifestations of typical and atypical Cogan's syndrome was also performed. 2022;70(5):40514. PubMed Central Peeters GJHCM, Cremers CWRJ, Pinckers AJLG, Hoefnagels WHL. Diagnosis A doctor diagnoses Cogan's syndrome after finding the typical combination of problems associated with this disease in the eye and inner ear. Fever of unknown origin Bomholt A, Knudsen JB, Permin H, Tommerup B, Gormsen J. Velut JG, Tonolli I, Demoux AL, Rossi P, Lafay V, Frances Y. The authors have declared no conflicts of interest. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. Curr Opin Rheumatol. Acute Limb Ischemia in Cogan Syndrome. De Righetti F, Nicole A, Meier D. Syndrome de Cogan atypique: a propos dun cas et revue de la litterature. Gran JT, Nordvag BY, Storesund B. proposed that a diagnosis of atypical Cogan's syndrome should be considered in the presence of ocular manifestations other than non-syphilitic IK or 2014;23(2):745. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. A 35-year-old female presented with fever, diarrhea, headache, bilateral sensorineural hearing loss, and tinnitus to the emergency department. 2021;46(10):2835. In February 2011, she first presented with ocular symptoms, including swelling and redness in her right eye. In an open-label prospective pilot study, 23 patients with bilateral immune-mediated cochlear vestibular disease or symptoms of bilateral Meniere's disease received etanercept (25mg twice weekly, subcutaneous injection) for 24weeks. Durtette et al. Morgan GJ Jr, Hochman R, Weider DJ. Finally, the role of vasculitis in the pathogenesis of the ocular and audiovestibular lesions of Cogan's syndrome remains to be demonstrated. Correspondence to Rituximab is a genetically engineered, chimeric murine/human monoclonal antibody directed against the cluster of differentiation (CD)-20 antigen found on the surface of normal and premalignant B cells and mature B cells. Symptoms recur during corticosteroid tapering. In the series reported by Vollertsen et al. Investigation of endolymphatic hydrops by electrocochleography in patients with Cogan's syndrome. Methods. She was prescribed with rituximab 500mg/week for 3months. [2] and including our series, 28/52 (54%) and 22/59 (37%) with typical and atypical Cogan's syndrome respectively were deaf in both ears. 2019;103(3):52734. The patients written consent for publication was obtained for this report. Of the remaining nine patients, seven presented uveitis, associated with conjunctivitis in two, superficial keratitis in two and descemetitis in one; the last two patients presented superficial keratitis with conjunctivitis. Once a diagnosis of CS has been made, patients need to be evaluated for vasculitis in addition to the degree of visual and hearing loss. Nineteen of the patients of our series were men. Twenty-five patients presented a single audiovestibular flare and seven patients (four typical Cogan's syndrome, three atypical Cogan's syndrome) had recurrent episodes of audiovestibular symptoms, occurring between 1 and 13yr after disease onset. WebCogan's syndrome is an idiopathic inflammatory disease which may present as interstitial keratitis, inflammation of other ocular structures, Meniere's-like attacks, or systemic vasculitis.

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