Cogan syndrome

Cogan syndrome
Classification and external resources
DiseasesDB 32705
MeSH D055952

Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye (the cornea) and often fever, fatigue, and weight loss, episodes of dizziness, and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D.G. Cogan in 1945.

Contents

History

In 1945 ophthalmologist David Glendenning Cogan (1908–1993) first described the "nonsyphilitic interstitial keratitis and vestibuloauditory symptoms" that would later bear his name.[1] In 1963, the atypical form of Cogan syndrome, also known as "Logan Syndrome" was first described.[2]

Diagnosis

While the ESR, WBC, C-reactive protein test may be abnormal and there may be thrombocytosis or anemia, none of these findings are reliable indicators of the disease. A slit-lamp examination is essential. Recent work has suggested that high-resolution MRI and antibodies to inner ear antigens may be helpful. Cogan syndrome can occur in children, and is particularly difficult to recognize in that situation.[3]

Causes

It is currently thought that Cogan's syndrome is an autoimmune disease. The inflammation in the eye and ear are due to the patient's own immune system producing antibodies that attack the inner ear and eye tissue. Autoantibodies can be demonstrated in the blood of some patients, and these antibodies have been shown to attack inner ear tissue in laboratory studies. Infection with the bacteria Chlamydia pneumoniae has been demonstrated in some patients prior to the development of Cogan's syndrome, leading some researchers to hypothesis that the autoimmune disease may be initiated by the infection. Chlamydia pneumoniae is a common cause of mild pneumonia, and the vast majority of patients who are infected with the bacteria do not develop Cogan's syndrome.[4]

Symptoms

Cogan's syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes. Cogan's syndrome can lead to vision difficulty, hearing loss and dizziness. The condition may also be associated with blood-vessel inflammation (called vasculitis) in other areas of the body that can cause major organ damage in 15% of those afflicted or, in a small number of cases, even death. It most commonly occurs in a person's 20s or 30s. The cause is not known. However, one theory is that it is an autoimmune disorder in which the body's immune system mistakenly attacks tissue in the eye and ear.[5]

Treatment

For more severe disease, oral corticosteroids may be necessary to reduce the inflammatory response. When large amounts of steroids are required or if the disease is severe and is not responding to steroid therapy, other immunosuppressive medications often are recommended. These immunosuppressive drugs include methotrexate (Rheumatrex), cyclophosphamide (Cytoxan, Neosar), cyclosporine (Neoral, Sandimmune) or azathioprine (Imuran). In some cases, combinations of these medicines are prescribed. Occasionally, if the disease has damaged blood vessels, cochlear implantation may need to be done to correct the problem.[6] Studies have shown it to produce significant improvement in hearing loss in some patients.[7]

Related disorders

  • McCabe disease

References

  1. ^ Cogan DG (1945). "Syndrome of nonsyphilitic interstitial keratitis and vestiboloauditory symptoms". Archives of Ophthalmology (Arch Ophthalmol) 33 (2): 144–149. doi:10.1001/archopht.33.2.144. http://archopht.ama-assn.org/cgi/content/summary/33/2/144. 
  2. ^ Bennett FM (1963). "Bilateral recurrent episcleritis associated with posterior corneal changes, vestibulo-auditory symptoms and rheumatoid arthritis". Am J Ophthalmol 55: 8158. PMID 13967466. 
  3. ^ Timothy C. Hain. "Cogan's syndrome". http://www.dizziness-and-balance.com/disorders/autoimmune/cogans_syndrome.htm. Retrieved 2008-11-06. 
  4. ^ Timothy C. Hain. "Cogan's Syndrome". http://www.american-hearing.org/disorders/autoimmune/cogans_syndrome.html. Retrieved 2008-11-06. [dead link]
  5. ^ "Cogan's Syndrome". http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/35151.html. Retrieved 2008-11-06. 
  6. ^ "Cogan's Syndrome - Treatment". http://www.everydayhealth.com/publicsite/index.aspx?puid=855F5078-E74F-489C-A1BC-F04933DA6B77&ContentID=AZ_d0109&contentPage=6. Retrieved 2008-11-06. 
  7. ^ Timothy C. Hain: Cogan's Syndrome at the American Hearing Research Foundation, Chicago, Illinois 2008.

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Look at other dictionaries:

  • Cogan syndrome — Arteritis (also referred to as vasculitis) that involves the ear. This condition is called Cogan syndrome after the American ophthalmologist David Glendenning Cogan (1908 93) who first described it. Cogan syndrome features not only problems of… …   Medical dictionary

  • Cogan — may refer to: People Alma Cogan, English singer Andrew Cogan Barry Cogan (footballer) Barry Cogan (politician) Brian Cogan Dean Cogan, Irish priest and writer Fanny Cogan Frank Cogan Kevin Cogan, Formula 1 driver Maggie Cogan Patrick Cogan Robert …   Wikipedia

  • Cogan-I-Syndrom — Das Cogan I Syndrom, oft auch Oculovestibuloauditorisches Syndrom oder einfach nur Cogan Syndrom genannt, ist ein seltenes, audio vestibuläres Krankheitsbild des Innenohres mit Beteiligung der Augen, dessen Ursache in einer Autoimmunreaktion… …   Deutsch Wikipedia

  • Syndrome, Cogan — A form of arteritis (also referred to as vasculitis) that involves the ear. This condition is called Cogan syndrome after the American ophthalmologist David Glendenning Cogan (1908 93) who first described it. Cogan syndrome features not only… …   Medical dictionary

  • Cogan oculomotor apraxia — congenital oculomotor apraxia an absence or defect of horizontal eye movements, so that when the patient tries to look at an object off to one side, the head must turn to bring the eyes into line with the object and the eyes exhibit nystagmus;… …   Medical dictionary

  • Syndrome de cogan — CIM 10: M30.8 {{{CIM10}}}.0 {{{X.0}}} …   Wikipédia en Français

  • Syndrome de Cogan — CIM 10 : M30.8 Le syndrome de Cogan se définit par l association d une kératite interstitielle à une atteinte audiovestibulaire. Il a été décrit en 1934 (ou 1945[1]). Dans la plupart des cas, s y associent d autres manifestations de… …   Wikipédia en Français

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

  • Cogan oculomotor apraxia syndrome — Co·gan oc·u·lo·mo·tor aprax·ia, syndrome (koґgən) [David Glendenning Cogan, American ophthalmologist, 1908–1993] see under apraxia and syndrome …   Medical dictionary

  • Cogan's syndrome — a disorder in which keratitis and iridocyclitis (see uveitis) are associated with tinnitus, vertigo, and bilateral sensorineural deafness. D. G. Cogan (1908 93), US ophthalmologist …   Medical dictionary


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