Cogan's syndrome: A systemic vasculitis

Cheson, Bruce D.; Bluming, Avrum Z.; Alroy, Joseph

doi:10.1016/0002-9343(76)90722-1

Cited by 115 publications

(42 citation statements)

References 21 publications

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“…Systemic manifestations occurred in 63% of the cases of Yamanishi 26 and 72% for Cheson. 35 In 37% and 28% [respectively 26,35], the disease is only localized to the eyes and inner ear, as in our cases 1 and 3. The systemic manifestations can occur from 2 months to 7 years 32,33,36 after the first signs of Cogan's syndrome.…”

Section: Systemic Manifestationssupporting

confidence: 58%

“…Cardiovascular manifestations are the most serious and represent 10% of cases of Cogan's syndrome. 17,35 Aortic insufficiency, coronary stenosis, 34,38 pericarditis, and arrhythmia are the most common cardiac lesions. According to Haynes et al, 3 aortic insufficiency is most common in typical Cogan's syndrome, whereas systemic vasculitis occurs in the atypical form.…”

Section: Systemic Manifestationsmentioning

confidence: 99%

“…This would suggest that typical Cogan's syndrome should not be considered as a disorder strictly limited to the eyes and inner ear and that in a long-term follow-up a regular cardiac examination should be performed to promptly detect such complications for appropriate treatment. 35 Respiratory symptoms-including thoracic pain, http://www.pediatrics.org/cgi/content/full/109/2/e38dyspnea, and hemoptysis or pleurisy-may occur. 33 None of our patients had cardiovascular or respiratory problems.…”

Section: Systemic Manifestationsmentioning

confidence: 99%

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Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

2002

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ABSTRACT. Cogan's syndrome is a rare, chronic inflammatory disorder that typically targets the eyes and vestibuloauditory apparatus, but it may also involve other organs. Three pediatric cases of Cogan's syndrome (ages 5, 13, and 18 years) are reported with long-term follow-up and complete and regular cochleovestibular functional evaluation and ophthalmologic and neurologic examinations. One case was a typical form (characterized by an interstitial keratitis and cochleovestibular impairment), whereas the other 2 cases were atypical forms with uveitis and polyarthritis. In all 3 cases, the first clinical sign was nonspecific eye redness misdiagnosed as a banal conjunctivitis, initially or secondarily associated with bilateral endocochlear sensorineural hearing loss and complete bilateral peripheral vestibular deficit. During the acute phase, early steroid treatment (prednisone, 1 mg/kg/day) was effective in treating the ocular lesions (3 of 3 cases) and improving hearing (2 of 3 cases) but less effective for the vestibular loss (2 of 3 cases). Adverse effects and dependence on the steroid occurred in 2 cases, and immunosuppressive drugs were necessary to avoid recurrences in 1 case. Over the longterm, the disease was controlled in 2 cases but continued to progress in the other. Cogan's syndrome in childhood should be suspected in cases of conjunctivitis associated with inner-ear symptoms; a prompt steroid treatment can avoid progressive impairment of multiple sensorineural functions (vision, balance, hearing). Long-term management involves limiting disease recurrences by adaptive therapies, screening for complications (aortitis in particular), and planning rehabilitation for the sensorial deficits. Pediatrics 2002;109(2). URL: http://www. pediatrics.org/cgi/content/full/109/2/e38; children, Cogan's syndrome, sudden hearing loss, imbalance, vertigo, conjunctivitis.ABBREVIATIONS. EVAR, earth vertical axis rotation; OVAR, off vertical axis rotation test; VOR, vestibuloocular responses; SNHL, sensorineural hearing loss; NSAID, nonsteroidal antiinflammatory drugs; MRI, magnetic resonance imaging; EBV, Epstein-Barr virus. C ogan's syndrome is a relatively rare inflammatory disease characterized by nonsyphilitic ocular interstitial keratitis associated with sudden hearing loss and vestibular impairment. However, its early diagnosis and treatment with steroids can prevent loss of inner-ear sensorial function and limit ophthalmologic aftereffects. In 1934, Morgan and Baumgartner 1 published the first case of the syndrome. In 1945, Cogan 2 published the full description of the syndrome as an inner-ear disorder in its typical form. Several authors later reported atypical forms 3,4 that differed from the typical presentation by the type of ocular lesions, which can include superficial keratitis, scleritis, episcleritis, iritis, or choroiditis, as well as variable hearing impairment and often systemic inflammation. 5 Cogan's syndrome is generally considered an autoimmune disease despite the frequent lack of direct immunologic...

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Section: Systemic Manifestationssupporting

confidence: 58%

Section: Systemic Manifestationsmentioning

confidence: 99%

Section: Systemic Manifestationsmentioning

confidence: 99%

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Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

2002

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“…A etiologia pode ser considerada como uma resposta de hipersensibilidade a um ou mais agentes infecciosos, associada a vasculite. Alguns autores apontam a B. burgdorferi (agente etiológico da Doença de Lyme) e infecção por algumas espécies de Chlamydia como agentes causais, porém sem evidências conclusivas 6,7,8 . Transformação linfocitária tem sido relatada na exposição de linfócitos do paciente a antígenos da córnea, escleroproteínas e antígenos da orelha interna.…”

Section: Discussionunclassified

Síndrome de Cogan: apresentação de caso e diagnóstico diferencial

Aumond¹,

Leonhardt²,

Abreu³

et al. 2002

Rev. Bras. Otorrinolaringol.

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A síndrome de Cogan é uma doença de difícil diagnóstico pois não apresenta nenhum marcador laboratorial específico, assim como a neuronite vestibular. As características clínicas da síndrome de Cogan são: ceratite intersticial não-luética associada à vertigem, tinnitus e disacusia neurossensorial. Evidências de vasculite sistêmica são encontradas em 50% dos pacientes. As formas atípicas da síndrome de Cogan abrangem doenças oculares inflamatórias mais severas. As características clínicas da Neuronite Vestibular são: vertigem incapacitante de aparecimento abrupto e não responsiva ao tratamento clínico, sem associação com alterações cocleares. Exemplificamos essa dificuldade diagnóstica com o caso de um paciente que iniciou quadro de vertigem súbita incapacitante associada à febre e algia ocular unilateral. O paciente foi hospitalizado, sendo iniciada a terapêutica para síndrome vestibular, não respondendo à medicação. Após uma semana, evoluiu com lesão ocular caracterizada por esclerouveíte anterior. Com quinze dias de evolução o paciente apresentou quadro de disacusia neurossensorial rapidamente progressiva. O exame vestibular evidenciou arreflexia à esquerda, ipsilateral à disacusia neurossensorial. A ressonância magnética evidenciou sinais de microvasculite em sistema nervoso central sendo diagnosticada a síndrome de Cogan. Foi iniciada terapia com corticóide oral e pulsoterapia com ciclofosfamida, havendo melhora total da vertigem, do desequilíbrio e da alteração ocular e com melhora parcial da disacusia.

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“…To our knowledge, about 100 cases of typical or atypical Cogan's syndrome have been previously reported, most of them occurring in Caucasians [5,7], A striking temporal association between an upper respira tory tract infection and the onset of Cogan's syndrome has been noted in about 50% of cases [2, <S], Some authors have speculated that Cogan's syndrome might be a hyper sensitivity response to infectious agents that were present in eye and ear. Haynes et al [2] found that 9 of 13 Cogan's syndrome patients had significant IgG titers to C. tracho matis and that 2 of 3 active patients studied had significant IgM titers.…”

Section: ]-mentioning

confidence: 99%

Cogan’s Syndrome

Ochonisky¹,

Chosidow²,

Kuentz³

et al. 1991

Dermatology

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We report the case of an 18-year-old black woman with urticarial vasculitis, vestibuloauditory dysfunction and superficial keratitis. Oral steroid therapy was effective in reducing most clinical manifestations with high-dosage dependency. However, only intravenous pulses of methylprednisolone allowed slight improvement of hearing. In this case we emphasize urticarial vasculitis as a new manifestation of atypical Cogan’s syndrome. A significant titer of Chlamydia trachomatis antibodies was found in our patient: their role in the pathogenesis of Cogan’s syndrome is discussed.

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Cogan's syndrome: A systemic vasculitis

Cited by 115 publications

References 21 publications

Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

Síndrome de Cogan: apresentação de caso e diagnóstico diferencial

Cogan’s Syndrome

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