Cogan syndrome

Changed by Mohammed Sultan, 12 Dec 2018

Updates to Article Attributes

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Cogan syndrome is a rare vasculitis of children and young adults which primarily characterised by 1,4,6:

However, it can potentially affect a multitude of other organs (classically aortitis) 4.

Cogan syndrome (CS) is the combination of episodic attacks of interstitial keratitis and audiovestibular disturbance due to autoimmune reaction to the eyes and the audiovestibular structures.

PathologyEpidemiology

CS is a rare disorder that can occur in people of any age and race, and it most frequently starts in young adults in their late 20’s or early 30’s.

Aetiology

Clinical presentation

  • Inflammation of the eyes
  • Audiovestibular dysfunction
    • nausea
    • vomiting
    • tinnitus
    • vertigo
    • hearing loss
  • Later on:
    • fatigue
    • headaches
    • joint pains
    • rashes

The exact aetiologyHearing loss progresses for 1 to 3 months and deafness occurs in about 60% of patients.

Auditory symptoms can precede or follow eye disease, usually within a short period of time.

Atypical Cogan syndrome (audiovestibular dysfunction with other types of inflammatory eye disease) is not well known although an autoimmune aetiologyassociated with vasculitis in 20% of cases and has been postulateda less favourable prognosis than typical Cogan syndrome.

TreatmentPathophysiology

Autoimmune reaction to the eyes and prognosis

The onset of

the disease is usually rapid with ocular findings preceding tinnitus and vertigo. If untreated the majority of patients will become deaf and 5% will be blind. Aortitis and aortic insufficiency develop in 10-15% of patients. Prognosis is good in patients without symptoms of aortitis and early steroid treatment may prevent deafness 6audiovestibular structures possibly triggered by upper respiratory infection.

History and etymology

It was initially described by David Glendenning Cogan, American ophthalmologist (1908-1993) in 1945 5.

Histopathology

Vestibule-cochlear structures demonstrate endolymphatic hydrops  with  perilymphatic compartments filled with loose fibrous tissue.

Radiographic features

MRI
  • post-contrast T1-weighted images of the middle ears show enhancement of the vestibule-cochlear, ampulla and semicercular canals
  • features of small and large vessel vasculitis

Differential diagnosis

  • lyme disease
  • syphilis
  • chlamydia
  • whipple’s disease

 other systemic inflammatory diseases

Crohn’s, Behcet’s, Relapsing polychondritis, rheumatoid arthritis, sarcoidosis, Sjogren’s syndrome, systemic lupus, vasculitis, and Vogt-Koyanagi-Harada syndrome

Treatment

  • glucocorticoid therapy
  • immunosuppressive therapy
  • anti-tumour necrosis factor (TNF) therapy

Prognosis

  • the course of the disease varies significantly from patient to patient.
  • in some patients there is an initial flare, which may last several weeks to months.
  • following this there may be a slowly progressive course in some patients while others have a course of complete remission with intermittent episodes of disease activity.
  • life-threatening aortic insufficiency develops in 10% of reported cases.
  • blindness occurs in less than five per cent of patients.
  • deafness is a frequent and debilitating outcome occurring in up to 54% of patients.

Practical points

  • prompt recognition and exclusion of other diseases with similar presentation.
  • prompt treatment with glucocorticoids and secondary line immunosuppressive therapy if not responding.
  • awareness of development of vasculitis and particularly aortitis with associated complications

History and etymology

It was initially described by David Glendenning Cogan, American ophthalmologist (1908-1993) in 1945 5.

  • -</ul><p>However, it can potentially affect a multitude of other organs (classically <a href="/articles/aortitis">aortitis</a>) <sup>4</sup>.</p><h4>Pathology</h4><h5>Aetiology</h5><p>The exact aetiology is not well known although an autoimmune aetiology has been postulated. </p><h4>Treatment and prognosis</h4><p>The onset of the disease is usually rapid with ocular findings preceding tinnitus and vertigo. If untreated the majority of patients will become deaf and 5% will be blind. <a href="/articles/aortitis">Aortitis</a> and aortic insufficiency develop in 10-15% of patients. Prognosis is good in patients without symptoms of aortitis and early steroid treatment may prevent deafness <sup>6</sup>.</p><h4>History and etymology</h4><p>It was initially described by <strong>David Glendenning Cogan,</strong> American ophthalmologist (1908-1993) in 1945 <sup>5</sup>.</p>
  • +</ul><p>However, it can potentially affect a multitude of other organs (classically <a href="/articles/aortitis">aortitis</a>) <sup>4</sup>.</p><p><strong>Cogan syndrome (CS)</strong> is the combination of episodic attacks of interstitial keratitis and audiovestibular disturbance due to autoimmune reaction to the eyes and the audiovestibular structures.</p><h4>
  • +<strong> </strong>Epidemiology</h4><p>CS is a rare disorder that can occur in people of any age and race, and it most frequently starts in young adults in their late 20’s or early 30’s.</p><h4>
  • +<strong> </strong>Clinical presentation</h4><ul>
  • +<li>Inflammation of the eyes</li>
  • +<li>Audiovestibular dysfunction<ul>
  • +<li>nausea</li>
  • +<li>vomiting</li>
  • +<li>tinnitus</li>
  • +<li>vertigo</li>
  • +<li>hearing loss</li>
  • +</ul>
  • +</li>
  • +<li>Later on:<ul>
  • +<li>fatigue</li>
  • +<li>headaches</li>
  • +<li>joint pains</li>
  • +<li>rashes</li>
  • +</ul>
  • +</li>
  • +</ul><p>Hearing loss progresses for 1 to 3 months and deafness occurs in about 60% of patients.</p><p>Auditory symptoms can precede or follow eye disease, usually within a short period of time.</p><p><strong>Atypical Cogan syndrome</strong> (audiovestibular dysfunction with other types of inflammatory eye disease) is associated with vasculitis in 20% of cases and has a less favourable prognosis than typical Cogan syndrome.</p><h4>Pathophysiology</h4><p>Autoimmune reaction to the eyes and the audiovestibular structures possibly triggered by upper respiratory infection.</p><h4>Histopathology</h4><p>Vestibule-cochlear structures demonstrate endolymphatic hydrops  with  perilymphatic compartments filled with loose fibrous tissue.</p><h4>Radiographic features</h4><h5>MRI</h5><ul>
  • +<li>post-contrast T1-weighted images of the middle ears show enhancement of the vestibule-cochlear, ampulla and semicercular canals</li>
  • +<li>features of small and large vessel vasculitis</li>
  • +</ul><h4>Differential diagnosis</h4><ul>
  • +<li>lyme disease</li>
  • +<li>syphilis</li>
  • +<li>chlamydia</li>
  • +<li>whipple’s disease</li>
  • +</ul><p> other systemic inflammatory diseases</p><p>Crohn’s, Behcet’s, Relapsing polychondritis, rheumatoid arthritis, sarcoidosis, Sjogren’s syndrome, systemic lupus, vasculitis, and Vogt-Koyanagi-Harada syndrome</p><h4>Treatment</h4><ul>
  • +<li>glucocorticoid therapy</li>
  • +<li>immunosuppressive therapy</li>
  • +<li>anti-tumour necrosis factor (TNF) therapy</li>
  • +</ul><h4>
  • +<strong> </strong>Prognosis</h4><ul>
  • +<li>the course of the disease varies significantly from patient to patient.</li>
  • +<li>in some patients there is an initial flare, which may last several weeks to months.</li>
  • +<li>following this there may be a slowly progressive course in some patients while others have a course of complete remission with intermittent episodes of disease activity.</li>
  • +<li>life-threatening aortic insufficiency develops in 10% of reported cases.</li>
  • +<li>blindness occurs in less than five per cent of patients.</li>
  • +<li>deafness is a frequent and debilitating outcome occurring in up to 54% of patients.</li>
  • +</ul><h4>Practical points</h4><ul>
  • +<li>prompt recognition and exclusion of other diseases with similar presentation.</li>
  • +<li>prompt treatment with glucocorticoids and secondary line immunosuppressive therapy if not responding.</li>
  • +<li>awareness of development of vasculitis and particularly aortitis with associated complications</li>
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  • + <w:LsdException Locked="false" Priority="63" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium Shading 1 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="64" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium Shading 2 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="65" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium List 1 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="66" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium List 2 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="67" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium Grid 1 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="68" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium Grid 2 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="69" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Medium Grid 3 Accent 6"/>
  • + <w:LsdException Locked="false" Priority="70" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Dark List Accent 6"/>
  • + <w:LsdException Locked="false" Priority="71" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Colorful Shading Accent 6"/>
  • + <w:LsdException Locked="false" Priority="72" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Colorful List Accent 6"/>
  • + <w:LsdException Locked="false" Priority="73" SemiHidden="false"
  • + UnhideWhenUsed="false" Name="Colorful Grid Accent 6"/>
  • + <w:LsdException Locked="false" Priority="19" SemiHidden="false"
  • + UnhideWhenUsed="false" QFormat="true" Name="Subtle Emphasis"/>
  • + <w:LsdException Locked="false" Priority="21" SemiHidden="false"
  • + UnhideWhenUsed="false" QFormat="true" Name="Intense Emphasis"/>
  • + <w:LsdException Locked="false" Priority="31" SemiHidden="false"
  • + UnhideWhenUsed="false" QFormat="true" Name="Subtle Reference"/>
  • + <w:LsdException Locked="false" Priority="32" SemiHidden="false"
  • + UnhideWhenUsed="false" QFormat="true" Name="Intense Reference"/>
  • + <w:LsdException Locked="false" Priority="33" SemiHidden="false"
  • + UnhideWhenUsed="false" QFormat="true" Name="Book Title"/>
  • + <w:LsdException Locked="false" Priority="37" Name="Bibliography"/>
  • + <w:LsdException Locked="false" Priority="39" QFormat="true" Name="TOC Heading"/>
  • + </w:LatentStyles>
  • +</xml><![endif]--><!--[if gte mso 10]>
  • +<style>
  • + /* Style Definitions */
  • + table.MsoNormalTable
  • + {mso-style-name:"Table Normal";
  • + mso-tstyle-rowband-size:0;
  • + mso-tstyle-colband-size:0;
  • + mso-style-noshow:yes;
  • + mso-style-priority:99;
  • + mso-style-parent:"";
  • + mso-padding-alt:0cm 5.4pt 0cm 5.4pt;
  • + mso-para-margin-top:0cm;
  • + mso-para-margin-right:0cm;
  • + mso-para-margin-bottom:10.0pt;
  • + mso-para-margin-left:0cm;
  • + line-height:115%;
  • + mso-pagination:widow-orphan;
  • + font-size:12.0pt;
  • + mso-bidi-font-size:11.0pt;
  • + font-family:"Garamond","serif";
  • + mso-fareast-language:EN-US;}
  • +</style>
  • +<![endif]--></p><h4>History and etymology</h4><p>It was initially described by <strong>David Glendenning Cogan,</strong> American ophthalmologist (1908-1993) in 1945 <sup>5</sup>.</p>

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