In the remaining eight patients (five typical Cogan's syndrome, three atypical Cogan's syndrome) the ocular and audiovestibular manifestations were concomitant. Moalla M, Bergaoui N, Hamza M et al. Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P, et al. 2013;32(3):2067. J Fr Ophtalmol. The Importance of FDG-PET/CT in Cogans Syndrome. https://doi.org/10.4274/mirt.349. The diagnosis of CS is based upon presence of characteristic Yamanishi Y, Ishioka S, Takeda M, Maeda H, Yamakido M. Atypical Cogan's syndrome associated with antineutrophil cytoplasmic autoantibodies. Service de Medecine Interne, Hopital Louis Mourier, Colombes, 1Service de Medecine Interne, Hopital Claude Huriez, Lille, 2Service de Medecine Interne, Hopital Foch, Suresnes and 3Service de Medecine Interne, Hopital Bichat, Paris, France. Treatment with prednisone (1 mg/kg/day) elicited a marked clinical response. Oxford University Press is a department of the University of Oxford. WebCogan syndrome is a rare autoimmune disease involving the eye and the inner ear. Malik MU, Pandian V, Masood H, Diaz DA, Varela V, Davalos-Balderas AJ, et al. [3], which included 78 patients with typical Cogan's syndrome, bilateral deafness affected 43.5% of patients and occurred a mean of 3 months after the onset of the initial symptoms. The best results seem to have been obtained with methotrexate, which led to an improvement in four published cases [16, 35, 36, 68] and stabilized hearing in two other cases [7, 65]. Livingston JZ, Casale AS, Hutchins GM, Shapiro EP. 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]. The most common causes of IK are contagious. Lunardi C, Bason C, Leandri M, Navone R, Lestani M, Millo E, et al. Hearing improved in 7 (30%) patients, unchanged in 13 (57%) and worsened in 3 (13%) patients [42]. The patient developed glucocorticoid side effects in January 2019. Most of the patients underwent extensive investigations looking for an infection at the time of disease onset. Mod Rheumatol. Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menire-like cochlear vestibular symptoms, which may We carried out a retrospective multicentre study. CS was diagnosed after excluding other diseases. Advertisement intended for healthcare professionals. Circulation. By definition, all 17 patients in the typical Cogan's syndrome group presented IK, which was bilateral in 16. Wolf J, Rose-John S, Garbers C. Interleukin-6 and its receptors: a highly regulated and dynamic system. https://doi.org/10.1093/rheumatology/keaa086. https://doi.org/10.1002/art.37715. Palpable purpura and uveitis precipitated by splenectomy in an atypical case of Cogan's syndrome. All but one patient presented with Meniere-like syndrome, and at the end of follow-up 11 were deaf and 19 additional patients had developed a significant decrease in auditory acuity. Cogans Syndrome Richardson B. Methotrexate therapy for hearing loss in Cogan's syndrome. Cogan's syndrome as an extra-articular manifestation of rheumatoid arthritis. The hallmark features include: Interstitial keratitis (blood vessels grow into the cornea). Some of the symptoms of Sandifer syndrome can be hard to distinguish from a neurological problem, such as epilepsy. 2020;11:616992. https://doi.org/10.3389/fimmu.2020.616992. Surveying for infections is usually negative, but evidence for Chlamydia infection has been reported in a few cases [2, 62]. CS has been classified as a primary variable vessel vasculitis. BMC Ophthalmology Cogans Syndrome: Symptoms and Treatments - All About PubMed Central 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Eur Arch Otorhinolaryngol. 2013;23(3):57781. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. Gtestam Skorpen C, Hoeltzenbein M, Tincani A, Fischer-Betz R, Elefant E, Chambers C, et al. Lancet. Cogans syndrome is more than just keratitis: a case-based literature review. Rheumatology (Oxford). Cogans syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. Curr Allergy Asthma Rep. 2020;20(9):46. https://doi.org/10.1007/s11882-020-00945-1. https://doi.org/10.1159/000477534. Blurry Vision Conjunctivitis - Pink Eye Corneal Abrasions Dry Eyes Eye Discharge Eye Pain Dilated Pupils Eye Infections Puffy Eyes More Eye Conditions More Eye Conditions Systemic manifestations in a series of 32 patients with Cogan's syndrome, and comparison between typical and atypical Cogan's syndrome in 222 cases of the literature. Since CS is a rare disease, only a few hundred cases have been reported in the literature since 1945. Correspondence to CNS, central nervous system; CSF, cerebral spinal fluid. It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide, This PDF is available to Subscribers Only. Josiah Harrell Diagnosed with Brain Disease Ahead of UFC 290 In 2016, she suffered from finger and knee pain, vomiting, and diarrhea. High resolution MRI indicators of activity. Diagnosed Orsoni JG, Lagan B, Rubino P, Zavota L, Bacciu S, Mora P. Rituximab ameliorated severe hearing loss in Cogans syndrome: a case report. The IK was isolated in 13 patients and associated with conjunctivitis in three patients or a superficial keratitis in two. Roat MI, Thoft RA, Thomson AW, Jain A, Fung JJ, Starzi TE. Immune-mediated conditions affecting the brain, eye and ear (BEE Ollivaud L, Godeau B, Lionnet F, Abbou CC, Lejonc JL, Schaeffer A. Cogan's syndrome and Peyronie's disease: a non-fortuitous association. Cogan's syndrome Temporal bone pathology in a case of Cogan's syndrome. ORL J Otorhinolaryngol Relat Spec. Med Clin North Am. Neurology. Thank you for submitting a comment on this article. Two out of 20 patients tested positive for antineutrophil cytoplasmic antibodies (ANCA) and had low titres (1/40) (patient 8 had peripheral ANCA and patient 15 had cytoplasmic ANCA), but antigen specificity could not be elicited by enzyme-linked immunosorbent assay. Cogan's syndrome with refractory abdominal aortitis and mesenteric vasculitis. Mol Imaging Radionucl Ther. Eye pain, decreased vision, increased sensitivity to bright light, and redness of the Shah P, Luqmani RA, Murray PI, Honan WP, Corridan PGJ, Emery P. Posterior scleritis. In October 2016, she suffered multiple joint swelling and pain, severe hearing loss in both ears (left ear 120 decibels, right ear 80 decibels) with elevated inflammatory indicators (CRP, 69mg/L; ESR, 108mm/h). However, in most cases, both manifestations occurred closely or even simultaneously in five patients (mean 2 months, median 4 weeks, range 012 months). Cogan's syndrome: a rare systemic vasculitis. in 1980 [2], the 79 patients identified in the French and English literature since 1980 and the 32 patients in the present series. Nineteen of the patients of our series were men. Joint symptoms were relieved after treatment with a JAK inhibitor (tofacitinib), and hearing did not deteriorate further. Wang, Y., Tang, S., Shao, C. et al. Google Scholar. Cogans Syndrome Two out of 16 patients (patients 11 and 24) had a circulating anticoagulant antibody, and only one (patient 9) of the six patients for whom tests for anticardiolipin antibodies were performed tested positive. Mod Rheumatol Case Rep. 2022;6(2):3058. Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. Respiratory and tinnitus, dizziness symptoms eased, and inflammatory markers decreased (CRP, 39mg/L; ESR, 29mm/h). It is recommended to start with 1mg/kg/day of prednisone for 24weeks or until patient improves [23]. Reumatol Clin (Engl Ed). Cogan syndrome masquerading as corneal ectasia. Involvement of the branches of the aorta may result in claudication of the upper and lower limb [11, 12]. 2019;103(3):52734. A moderate and usually transient decrease in visual acuity is not uncommon in Cogan's syndrome, but amaurosis or blindness may also occur. Abstract. https://doi.org/10.1016/j.jfo.2020.11.004. Immunol Lett. The patients written consent for publication was obtained for this report. The patient still developed bilateral sensorineural hearing loss after receiving hormone, methotrexate, cyclophosphamide, and a variety of biological agents. Optical coherence tomography (OCT) showed signs of optic disc oedema and macular degeneration. Classic form. https://doi.org/10.1016/j.cyto.2014.05.024. Cogan's Syndrome: Clinical Presentations and Update on Treatment However, magnetic resonance imaging and computed tomographic scanning may show narrowing or obliteration of parts of the vestibular labyrinth that are related to the risk of permanent hearing loss [12, 13]. Velut JG, Tonolli I, Demoux AL, Rossi P, Lafay V, Frances Y. Syndrome de Cogan: a propos dun cas. Treatment failure indicates the need to add additional immunosuppressants [4]. 2021;44(7):e41921. Methods. Pearls & Oy-sters: Cogan syndrome: A potentially grave disorder of audiovestibulopathy with many faces. Inhibition of JAK-STAT Signaling Suppresses Pathogenic Immune Responses in Medium and Large Vessel Vasculitis. J Vestib Res. Syndrome de Cogan. Cogan syndrome: Descriptive analysis and clinical experience of 7 cases diagnosed and treated in two third level hospitals Sndrome de Cogan: Cogans syndrome (CS) is an inflammatory disease classified as variable vessel vasculitis. Arthritis Rheum. Cogan syndrome | Radiology Reference Article | Radiopaedia.org It is a powerful antagonist of TNF, binding to and inactivating the cytokine. Cogan syndrome - Wikipedia J Immunol Res. Specific involvement of large arteries has been reported in Cogan's syndrome. As maintenance therapy, prednisone was reduced to 5mg/day orally. Cogan's syndrome after finding the typical combination of problems associated with this disease in the eye and inner ear. Pyoderma gangrenosum complicating Cogan's syndrome. All authors read and approved the final manuscript. https://doi.org/10.1111/echo.14428. Although successful responses can be achieved, relapses are common due to long-term glucocorticoid use, and side effects can occur. 2011;88(10):E12626. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Diagnosis Corticosteroids are first-line treatment. https://doi.org/10.1001/archopht.1959.00940090697004. Helmchen C, Jager L, Buttner U, Reiser M, Brandt T. Cogan's Syndrome. Kontorinis G, Giourgas A, Neuburger J, Lesinski-Schiedat A, Lenarz T. Long-term evaluation of Cochlear implantation in Cogan syndrome. The association of non-syphilitic interstitial keratitis (IK) with audiovestibular symptoms resembling Meniere's disease was first recognized as a separate clinical entity by David Cogan in 1945 [1]. CT and contrast-enhanced MRI of the inner ear were normal. The aetiology of Cogan's syndrome remains unknown. Correspondence to: A. Grasland, Service de Medecine Interne, Hopital Louis Mourier, 178 rue des Renouillers, 92700 Colombes, France. Casoli P, Tumiati B. Cogan's syndrome: a new possible complication of antiphospholipid antibodies? 2016;75(5):795810. In the other patients, immunological studies were not contributory. Elkayam O, Caspi D, Geyer O, Yaron M. Atypical Cogan's syndrome in a 76-year-old woman. Orphanet J Rare Dis. 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]. PubMed Article Reported peripheral nerve involvement includes paraesthesia of the extremities, trigeminal neuralgia or motor deficits affecting the cranial nerves, the phrenic nerve or several nerve trunks, resulting in mononeuritis multiplex [3, 9, 24, 33, 38]. The diagnosis of Cogan syndrome was based on a combination of bilateral keratitis, sensorineural hearing loss, and systemic vasculitis manifestations. Rheumatology (Oxford). Cathi Bedy in "Take Care of Maya." Netflix - Insider In our series of patients with Cogan syndrome, sex ratio, age at diagnosis, and prevalence of vestibulo-auditory, ocular and systemic signs were similar to previous data from the Mayo Clinic and French nationwide series [2], [3]. Vestibular auditory dysfunction in CS with sudden onset of Menire-like vertigo, ataxia, tinnitus, nausea, vomiting, and sudden hearing loss [6]. Clin Rev Allergy Immunol. https://doi.org/10.1136/annrheumdis-2021-220484. Cogan's syndrome has been associated with various systemic manifestations, and the disease remained restricted to eye and ear in 17/52 (33%) of the patients with typical Cogan's syndrome and 7/59 (12%) (P<0.01) of those with atypical Cogan's syndrome published since the review by Haynes et al. Treatment of Cogan's syndrome with FK 506: a case report. Optom Vis Sci. Comparison of our series with the 190 reported cases does not reveal any significant difference regarding the prevalence of these systemic manifestations. Her condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. Thomas HG. 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. A comprehensive literature review using the Medline database and the reference lists of identified articles was performed. My Diagnosis Gave an Explanation for My Horrible Leg Cramps Cogans syndrome: a cause of progressive hearing deafness. The patient's body temperature gradually returned to normal. Springer Nature. In the typical Cogan's syndrome group, the delay between the onset of ocular and audiovestibular manifestations was, by definition, less than 2yr. Until both CAS The patient initially received intravenous methylprednisolone 40mg/day in combination with oral methotrexate 10mg/week. Renal manifestations in one patient with atypical Cogan's syndrome who developed a membranoproliferative glomerulonephritis followed by an end-stage renal failure 1yr later. Mitral valve involvement has been reported rarely [5, 6, 52]. [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. Une observation avec vascularite systemique. Vella JP, OCallaghan J, Hickey D, Walshe JJ. 2019;36(8):15903. 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. In addition to perfecting eye exams and pure tone audiograms for quantifying hearing loss and tracking disease progression, laboratory tests (including complete blood count, urinalysis, serum electrolytes, creatinine, hepatic transaminases, and erythrocyte sedimentation rate) are also required to confirm systemic involvement or other diagnoses [33]. Matteson EL, Choi HK, Poe DS, Wise C, Lowe VJ, McDonald TJ, et al. In February 2011, she first presented with ocular symptoms, including swelling and redness in her right eye. If deafness occurs, it is usually permanent although a secondary improvement in hearing has been observed in a few patients. Hocevar A, Tomsic M, Perdan PK. 2004;36(67):5247. Anyone you share the following link with will be able to read this content: Sorry, a shareable link is not currently available for this article. YQW collected the patients information, reviewed the literature, and drafted this manuscript; SCT and CS helped to analyze and interpret the patient data; YL checked all works and words. Neurological manifestations were present in 29 of the 222 published cases (13%). One patient had a moderate positive antinuclear antibody (ANA) titre (1/160) while in the others ANA was negative or present at only low titres. The audiovestibular manifestations of Cogan's syndrome are similar to those of Meniere's syndrome, with an abrupt onset including vertigo, instability, nausea, vomiting and tinnitus [13].
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