Lack of response to corticosteroids and pulse cyclophosphamide therapy in Cogan's syndrome

Vaiopoulos, George; Sfikakis, Petros P.; Skoumas, B.; Kavouklis, E.; Doukas, E.; Kaklamanis, Phaedon

doi:10.1007/bf02229876

Cited by 20 publications

(14 citation statements)

References 11 publications

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“…One of the pediatric patients had arthritis, [6,7] but arthralgia and myalgia had been reported in children and were also present in our case [5,8]. Fever, headache, maculopapular rash, abdominal pain, nausea, vomiting, and lymphadenopathy may be seen in children with CS [2,6,7,8].…”

Section: Discussionsupporting

confidence: 52%

“…Vestibuloauditory dysfunction usually starts suddenly with Meniere's-like attacks, but only four patients in the pediatric sector reported cases presenting with acute attacks [6,7]. However, sensorineural deafness of variable severity, whether transient or permanent, was found in all of the pediatric cases, including ours.…”

Section: Discussionmentioning

confidence: 73%

“…Interstitial keratitis was the most common ocular manifestation, seen in nine of the reported pediatric cases. The other patients, including ours, had other eye changes such as photophobia, conjunctivitis, scleritis, episcleritis, corneal opacity, iritis, and chalazion [6,8].…”

Section: Discussionmentioning

confidence: 92%

“…Since then, many similar cases have been reported, suggesting that this syndrome is a systemic illness involving other organs and leading to deafness, blindness, and death [3]. The eyes, ears, lymph nodes, gastrointestinal tract, heart and great vessels, joints, and nervous system are involved during the evolution of the disease [6].…”

Section: Discussionmentioning

confidence: 97%

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Cogan's syndrome in childhood

Olfat

Al‐Mayouf

2001

Rheumatol Int

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Section: Discussionsupporting

confidence: 52%

Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 97%

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Cogan's syndrome in childhood

Olfat

Al‐Mayouf

2001

Rheumatol Int

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“…12,45,46 However, these drugs have adverse effects, which limit their prescription in children, and their efficacy is controversial in Cogan's syndrome. For these reasons these agents should never be prescribed as a first treatment in children.…”

Section: Treatment and Prognosismentioning

confidence: 99%

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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Adult and Childhood Vasculitis of the Nervous System

Younger

2019

Clinical Neuroimmunology

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Lack of response to corticosteroids and pulse cyclophosphamide therapy in Cogan's syndrome

Cited by 20 publications

References 11 publications

Cogan's syndrome in childhood

Cogan's syndrome in childhood

Cochleovestibular Impairment in Pediatric Cogan’s Syndrome

Adult and Childhood Vasculitis of the Nervous System

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