Cogan's syndrome – A rare aortitis, difficult to diagnose but with therapeutic potential

Colodetti, Raiza; Spina, Guilherme Sobreira; Leal, Tatiana de Carvalho Andreucci Torres; Oliveira, Múcio Tavares de; Soeiro, Alexandre de Matos

doi:10.1590/1806-9282.63.12.1028

Cited by 6 publications

(2 citation statements)

References 5 publications

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“…Cogan's syndrome, a rare systemic disease of young Caucasian adults (mean age 30 years) of either sex defined by nonsyphilitic interstitial keratitis, in association with audio‐vestibular involvement that may lead to complete deafness . The exact incidence of Cogan's syndrome is unknown, with an estimated 300 cases have been reported in literature by 2017 . Cogan's syndrome may present in an atypical form where different types of inflammatory eye diseases occur other than interstitial keratitis or if there is a wide interval between visual and auditory involvement usually more than 2 years .…”

Section: Discussionmentioning

confidence: 99%

Aortic valve perforation in the setting of Cogan's syndrome

et al. 2019

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Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio‐vestibular manifestations. Systemic manifestations are quite unusual with pan‐vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56‐year‐old woman presented with ocular, audio‐vestibular, and systemic manifestations with medium‐sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome.

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Section: Discussionmentioning

confidence: 99%

Aortic valve perforation in the setting of Cogan's syndrome

et al. 2019

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“…Pathognomonic manifestations include intraocular inflammation (nonsyphilitic interstitial keratitis) and vestibulo-auditory dysfunction (neurosensory deafness, tinnitus, and vertigo) (9). Cases of aortitis with Cogan's syndrome have been reported (10).…”

Section: Differential Diagnosismentioning

confidence: 99%

All in the Family: A Curious Case of Aortopathy

McBride

Stern

Bilal

2021

Arthritis Care & Research

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The patient had a history of intermittent headaches and presented at a hospital outside of our institution with a few days history of severe pain and swelling of the right leg and dyspnea. A venous ultrasound of the right leg revealed a large occlusive deep vein thrombosis (DVT) extending from the right common femoral vein to the right popliteal vein. A computed tomography (CT) angiogram of the chest revealed a mid ascending thoracic aortic aneurysm measuring 5.9 cm, a pulmonary artery dilatation measuring 3.2 cm, and no evidence of pulmonary embolus. The patient was started on a regimen of anticoagulation with intravenous heparin and was transferred to our institution for surgical management of the aortic aneurysm and evaluation for large vessel vasculitis.

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