Síndrome de Cogan: descripción de un caso con respuesta parcial a tocilizumab y revisión de la literatura / Cogan's syndrome: A case report with partial response to tocilizumab and a literature review

RESUMEN El síndrome de Cogan (SC) es una enfermedad inflamatoria crónica, caracterizada por queratitis intersticial y síntomas vestíbulo-auditivos similares al síndrome de Meniere, acompañado o no de vasculitis sistémica. Es una enfermedad poco frecuente y su diagnóstico es difícil y a menudo tardío, lo que aumenta el riesgo de secuelas como pérdida visual o cofosis. Además, su tratamiento no está bien establecido y se basa en reportes de casos donde se ha observado la eficacia de fármacos como la ciclofosfamida, anti-TNF o tocilizumab. Presentamos un caso de SC sin respuesta a metotrexato, ciclofosfamida ni anti-TNF, y respuesta parcial a tocilizumab.

ABSTRACT Cogan's syndrome is a chronic inflammatory disease typified by interstitial keratitis and Meniere-like auditory involvement, and it can be accompanied by systemic vasculitis. It's a rare disease and its diagnosis is difficult and often late, which increases the risk of sequelae, such as visual loss or deafness. In addition, its treatment is not well-established and is based on case reports that have observed efficacy of drugs such as cyclophosphamide, anti-TNF or tocilizumab. A case of Cogan's syndrome is presented that did not respond to methotrexate, cyclophosphamide, or anti-TNF, but with a partial response to tocilizumab.

Cogan Syndrome Accompanied with Meningitis / 대한평형의학회지

Cogan syndrome is a rare inflammatory disease characterized by intraocular inflammation and vestibulo-auditory dysfunction. The exact etiology of Cogan syndrome is still unknown, but is currently thought to be an autoimmune disease. Cogan syndrome can be accompanied with various conditions including fever, arthritis, skin rash, aortitis, central or peripheral nerve system involvement, lymphadenopathy, splenomegaly and diarrhea. We report a case of Cogan syndrome accompanied with meningitis.

Cogan's syndrome - A rare aortitis, difficult to diagnose but with therapeutic potential / Síndrome de Cogan - Uma aortite rara, de diagnóstico difícil, mas com potencial terapêutico

Summary The inflammation of aortic wall, named aortitis, is a rare condition that can be caused by a number of pathologies, mainly inflammatory or infectious in nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan's syndrome, making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control.

Resumo A inflamação da parede da aorta, denominada aortite, é uma condição clínica rara, que pode ser causada por diversas patologias, principalmente as de fundo inflamatório e/ou infeccioso. Nesse contexto, a ocorrência de sintomas vestibulares e oftalmológicos associados ao quadro remete ao diagnóstico de síndrome de Cogan, tornando o caso raro, mas passível de tratamento imunossupressor adequado e controle satisfatório da doença.

Ceratite intersticial em paciente com Síndrome de Cogan / Interstitial keratitis in patients with Cogan's Syndrome

RESUMO A Síndrome de Cogan é caracterizada pela ceratite intersticial não luética associada à vertigem, tinnitus e disacusia neurossensorial. Relatamos um caso que ilustra um modelo da intervenção multidisciplinar no diagnóstico e tratamento da doença.

ABSTRACT Cogan's syndrome is characterized by interstitial keratitis non-syphilis associated with vertigo, tinnitus and sensorineural hearing loss. We report a case that illustrates a model of multidisciplinary intervention in the diagnosis and treatment of disease.

Síndrome de Cogan típico: a propósito de un caso / Typical Cogan’s syndrome: report of a case

Introducción: El síndrome de Cogan es una entidad poco frecuente, de origen autoinmune.Se caracteriza por queratitis intersticial no sifilítica asociada a síntomasaudiovestibulares similares al síndrome de Ménière. Los primeros síntomas ocularesincluyen enrojecimiento y fotofobia, a los que se puede agregar conjuntivitis o uveítis.Objetivo: Presentar un caso de síndrome de Cogan típico y describir las principalescaracterísticas de la entidad.Caso clínico: Varón, 32 años, sin antecedentes mórbidos. Consulta por cuadro de cuatrodías de evolución caracterizado por dolor ocular e inyección conjuntival. Diagnosticadoen un comienzo con sinusitis y conjuntivitis aguda y se maneja como tal. Alpersistir sintomatología se hospitaliza a la semana evidenciándose fiebre, inyecciónconjuntival bilateral y a los pocos días hipoacusia. Los exámenes de laboratorio destacanleucocitosis, trombocitosis, PCR, VHS e IgA elevadas. Autoanticuerpos y estudiopara VIH y VHC negativos, TAC de cerebro y cavidades paranasales normales. Evaluadopor oftalmología y otorrinolaringología, describiéndose hiperemia conjuntival yquemosis, e hipoacusia neurosensorial de probable origen autoinmune, respectivamente.Evaluado por reumatología, se plantea síndrome de Cogan típico. Se indicaprednisona 1 mg/kg/día, metilprednisolona por dos días y metotrexato semanal, conbuena respuesta. Evoluciona con disminución de cefalea, de compromiso ocular ehipoacusia, afebril, dándose de alta con prednisona y metotrexato. Control al mesdel alta, paciente asintomático, se mantiene tratamiento. Control en oftalmología20 días después, objetivándose leve edema corneal, por lo que se indica tratamientotópico...

Background: Cogan’s syndrome is a rare entity of autoimmune origin. It is characterizedby non-syphilitic interstitial keratitis associated with audiovestibular symptomssimilar to Meniere’s syndrome. Early symptoms include eye redness and photophobia,which can be added conjunctivitis or uveitis.Objective: To report a case of typical Cogan’s syndrome and describe the main featuresof the entity.Case report: Male, 32, no morbid history. Consults for story of 4 days of evolutioncharacterized by eye pain and conjunctival injection. Initially diagnosed with sinusitisand acute conjunctivitis and handled as such. By persisting symptoms is hospitalizeda week later demonstrating fever, bilateral conjunctival injection and in a few dayshearing loss. Laboratory tests include leukocytosis, thrombocytosis, and elevatedPCR, VHS and IgA. Autoantibodies, HIV and HCV study were negative, brain and sinusesCT normal. Reviewed by ophthalmology and otolaryngology, describing conjunctivalhyperemia and chemosis, and sensorineural hearing loss of probable autoimmuneorigin, respectively. Evaluated by rheumatology, typical Cogan’s syndrome arises. It isindicated prednisone 1 mg/kg/day, methylprednisolone for 2 days and weekly methotrexate,with good response. Evolves with decreased in headache, eye commitmentand hearing loss, afebrile, was discharged with prednisone and methotrexate. Controla month after discharge, asymptomatic, treatment is maintained. Control 20 days laterin ophthalmology, objectifying mild corneal edema, so topical treatment indicated...

Vasculite sistêmica da síndrome de Cogan associada ao PR3-ANCA. Relato de caso / Systemic vasculitis of Cogan's syndrome associated with PR3-ANCA. Case report

JUSTIFICATIVA E OBJETIVOS: A síndrome de Cogan (SC)caracteriza-se pela presença de ceratite intersticial não luética associada a manifestações de disfunção vestibulococlear. Este artigo tem como finalidade dar continuidade ao relato de caso da SC publicado nesse periódico em 2009, que mostra, agora em 2014, o acompanhamento ambulatorial durante 60 meses, os resultados laboratoriais, o tratamento realizado e faz uma breve revisão bibliográfica dos marcadores imunológicos. Também, como objetivo principal, apresentar essa rara entidade nosológica que, quando não tratada no início dos sintomas com imunossupressores, pode causar anacusia em 50% e amaurose em 10% dos pacientes e, em sua forma atípica, cursar com vasculite sistêmica. Como não encontramos citações na literatura mundial dessa associação - vasculite da SC com positividade de anticorpos dirigidos contra citoplasma de neutrófilos (c-ANCA) direcionados especificamente contra o antígeno serinaproteinase 3(PR3) - consideramos prudente novos artigos serem publicados no sentido de confirmar ou não esses achados clínico-laboratoriais. Ressaltamos que o paciente evoluiu satisfatoriamente para a cura, visto que permanece assintomático e com os exames de atividade inflamatória normais. RELATO DO CASO: Paciente do sexo masculino, 43 anos, branco, casado, comerciário,foi internado por 15 dias por apresentar hiperemia conjuntival, mialgias e febre com 30 dias de evolução. O diagnóstico foi realizado a partir do 11° dia, quando surgiram as seguintes manifestações: nistagmo, ataxia de marcha, tontura, náuseas e vômitos aos movimentos, dores articulares no punho, joelho e tornozelo esquerdos, acompanhadas de rubor e calor, acrescidas de sufusões hemorrágicas subungueais dolorosas em três dedos da mão esquerda, sugestivas de vasculite sistêmica. CONCLUSÃO: Este relato de caso apresenta aos profissionais médicos essa entidade rara, de difícil diagnóstico e de repercussões graves quando não bem tratada. Além disso,...

BACKGROUND AND OBJECTIVES: Cogan's syndrome (CS) is characterized by the occurrence of non-luetic intersticial keratitis associated with vestibulocochlear dysfunction signs. This article aims to give continuity to a report on the CS published in this journal in 2009, and proposes to show a 60-month outpatient follow-up, laboratory results and treatment prescribed, as well as to give a brief literature review on immunological markers, as far as 2014. It also has the primary aim of presenting this rare nosological entity which, if not treated with immunosuppressive drugs at the onset of symptoms, can lead to deafness in 50% of the patients and amaurosis in 10% of them, and occur concomitantly with systemic vasculitis in its atypical form. As international literature citations on the CS vasculitis association with positivity of antibodies against neutrophil cytoplasm (c-ANCA) specifically directed against cytoplasmic antigen serine proteinase 3 (PR3) have not been found, it is suggested that new articles should be published in order to either confirm or deny these clinical and laboratory findings. It is noteworthy that the patient progressed satisfactorily towards healing, and remains asymptomatic with negative inflammatory activity exam results. CASE REPORT: a 43 year-old married male Caucasian salesclerk from Piratini (RS) was hospitalized for a 15-day period showing conjunctival hyperemia, myalgia and fever following a thirty-day evolution period. Diagnosis was only reached on the 11th day when the following signs appeared: nystagmus, motor ataxia, dizziness, nausea and vomiting upon moving, joint pain in the left wrist, knee and ankle, accompanied by redness and heat, in addition to painful subungual hemorrhagic suffusions in three left-hand fingers compatible with systemic vasculitis. CONCLUSION: This case describes a rare syndrome of difficult diagnosis and serious implications when not properly treated...

Multiple Cerebral Infarction in Cogan's Syndrome / 대한평형의학회지

Cogan's syndrome is a rare chronic inflammatory disease Characterized by non-syphilitic keratitis and vestibuloauditory dysfunction. Although the precise pathogenesis of Cogan's syndrome is unknown, it is thought to develop from vasculitis involving multiple organ system. It can be accompanied with various systemic diseases including arthritis, lymphadenopathy, splenomegaly, and aortitis with insufficiency. We report a case of typical Cogan's syndrome with multiple cerebral infarctions.

Atypical Cogan's syndrome associated with coronary disease / 中华医学杂志(英文版)

Cogan’s syndrome (CS) is a rare inflammatory disorder characterized by interstitial keratitis and vestibuloauditory abnormalities often associated with various systemic manifestations. Involvement of cardiovascular system resembling systemic vasculitis may lead to severe complications and death. The present report describes a case of a female patient with atypical Cogan's syndrome presented with systemic manifestations and severe coronary and femoral artery stenosis. Despite the clinical improvement after glucocorticoids and cyclophosphamide, the patient required double aortocoronal bypass grafting one year letter. During three years follow-up, she was in stable condition, without stenocardial symptoms and claudication and her inflammatory parameters remain normal. This case highlights the rare involvement of coronary arteries without associated large-vessel vasculitis of the aortic arch in CS.

A Case of Typical Cogan's Syndrome with Aortitis / 대한류마티스학회지

Cogan's syndrome can accompany a variety of systemic vasculitides including aortitis. A 45-year-old woman with a history of typical Cogan's syndrome presented with orthopnea and exertional dyspnea. Echocardiography demonstrated severe aortic valve insufficiency. Computed tomographic angiography demonstrated active vasculitis affecting the ascending and descending aortas and also showed stenosis of the left subclavian artery, both renal arteries, the celiac axis, the superior mesenteric artery, and the right common iliac artery. She received high dose corticosteroid and then underwent an aortic valve replacement. This is the first case of Cogan's syndrome with aortitis in Korea.

Congenital Ocular Motor Apraxia: Clinico-Radiological Analyses

BACKGROUND: Congenital ocular motor apraxia (COMA) is characterized by impaired voluntary saccades and abnormal head thrusts. However, mechanism of this disorder remains to be elucidated. METHODS: This study analyzed the eye movements and imaging findings in 16 patients with COMA, who had been recruited from 2003 to 2009 at the Neuro-Ophthalmology Clinic of Seoul National University Bundang Hospital. RESULTS: All the children showed impaired saccades and smooth pursuit in the horizontal direction. One of them also exhibited impaired vertical saccades and smooth pursuit. Eight children showed excessive blinks in association with an attempt to generate saccades. The typical head thrust usually developed around the age of eight months and had resolved by the age of 6-7 years. History of spasmus nutans was confirmed in seven children. Fourteen children showed cerebellar vermian hypoplasia, mostly in the inferior portion, and five of them also had dysgenesis of the corpus callosum. The severity of cerebellar vermian hypoplasia was correlated with developmental delay, as determined by the age of independent walking. CONCLUSION: Cerebellar vermian hypoplasia is a frequent finding in COMA. Dysfunction of the oculomotor vermis may responsible for the impaired saccades and smooth pursuit in COMA. The occasional association of COMA with spasmus nutans indicates a common pathophysiology of these benign developmental disorders.

A Case of Cogan's Syndrome With Angina

Cogan's syndrome is a rare systemic inflammatory disease and can be diagnosed on the basis of typical inner ear and ocular involvement with the presence of large vessel vasculitis. We report a case of Cogan's syndrome with stable angina resulting from coronary ostial stenosis caused by aortitis.

Two Cases of Congenital Ocular Motor Apraxia Associated With Cerebellar Vermian Dysplasia

Congenital ocular motor apraxia is a rare syndrome characterized by rotational head thrusts that attempt to compensate for the lack of voluntary eye movements. We describe the clinical, oculographic and magnetic resonance imaging features of two children with congenital ocular motor apraxia.

A Case of Cogan's Syndrome with Antibodies to Ro (SS-A) and La (SS-B) Antigen / 대한류마티스학회지

Cogan's syndrome is a rare chronic inflammatory disease of unknown origin, characterized by nonsyphilitic interstitial keratitis, vestibuloauditory dysfunction and vasculitis. Cogan's syndrome is uncommon and few cases have been published. A case of Cogan's syndrome in a patient with anti-Ro and anti-La positivity is described. A 24-year-old woman visited to department of rheumatology with dry mouth, dry eye, and vertigo on August 2004, and interstitial keratitis had developed on October 2004. She was admitted to the otolaryngology department with rapidly progressive hearing loss on December 2004. The patient's audiogram revealed severe sensorineural deafness. The patient was treated with systemic corticosteroid and methotrexate. This case is the first to report a case of Cogan's syndrome associated with anti-Ro and anti-La positivity.

A Case of Atypical Cogan Syndrome with Late-Onset Corneal Involvement

PURPOSE: To report a case of atypical Cogan syndrome with late-onset corneal involvement. METHODS: Bilateral conjunctival injection with severe headache developed in a 38-years old woman who had suffered from tinnitus, vertigo and hearing difficulty. Inflammation in anterior chamber and vitreous cavity, macular edema, disc edema and retinal hemorrhages were found on ocular examination. RESULTS: After initiation of systemic steroid therapy, ocular inflammatory reaction and visual acuity improved, but hearing difficulty did not respond. After 7 years of lost follow-up, corneal stromal opacity occurred and she was diagnosed as atypical Cogan syndrome. Elevated ESR, multiple stenosis in renal and lower extremity arteries in angiography were found. CONCLUSIONS: Atypical Cogan syndrome with late-onset corneal involvement is rare and difficult to diagnose, which may delay in initiation of adequate treatment.

Syndrome of nonsyphilitic Interstitial Keratitis and Vestibulcauditory symptoms: Cogan's Syndrome

The syndrome of nonsyphilitic interstitial keratitis associated with vestibulcauditory symptoms (tinnitus, vertigo, nystagmus and progressive deafness) was described by Dr. Cogan in 1945. Since then, numerous cases of this clinical entity have been reported elsewhere in the world. Recently the author experienced one case of Cogan's syndrome in our ophthalmologic OPD for the first time in Korea. The 45 years old male patient, looking very well on gross appearance, was admitted to ENT ward with sudden onset of vertigo, tinnitus and progressive deafness under the diagnosis of Meniere's disease on May 14,1976. Two month later, on July 6, 1976, the visual acuity in his left eye had suddenly deteriorated from 1.2 to 0.6. Under the slit lamp microscopic examination, mild infiltration in the corneal stroma and almost total pcsterior synechia were detected, and a few inflammatory cells were found in anterior chamber but not observed agueous flares. Several fine keratic precipitates were adhered on the lower one half of the posterior corneal surface as a linear apperance. The patient was treated by local instillation of 1% Atropine, 10% Phenylephrine and 3%. Dexamethasone, and also 1% Depomedrol was injected subconjunctivally. Oral administration of Dexamethasone, Vitamine B complex, INH and Chloramphenicol were combined. Thereafter those ocular symptoms had gradually improved, and recently his visual acuity has recovered to normal level (OS 1.2). The vestibuloauditory symptoms just as tinnitus and vertigo were also disappeared, where as moderate deafness has been still remained.