Cogan’s Syndrome

Part of the Medical Radiology book series (MEDRAD)


Cogan’s syndrome is thought to be an autoimmune disease of uncertain etiology which causes inflammation of the eye, most typically interstitial kcratitis, and hearing loss. It typically occurs in the third and fourth decades of life. About half of patients have systemic disease manifestations, including constitutional symptoms of fever and weight loss, arthritis, neuropathy, rash, pericarditis, pleuritis, and vasculitis. The vasculitis typically affects large vessels, particularly the aorta and other large vessels, but may also affect medium and small vessels as well. Vascular complications may lead to limb claudication and aortic aneurysm aortic heart disease and failure of other involved organs. Vasular imaging, which can include angiography using conventional imaging computed tomography, ultrasound, magnetic resonance scanning, and use of positron emission tomography, is required to assess the extent and activity vascular involvement. Treatment is directed to the involved organs, and can include glucocorticosteroids, cytotoxic agents, and vascular repair.


Hearing Loss Giant Cell Arteritis Relapse Polychondritis Endolymphatic Hydrops Central Nervous System Vasculitis 


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Copyright information

© Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  1. 1.Division of RheumatologyMayo Clinic College of MedicineRochesterUSA
  2. 2.Department of RadiologyMayo Clinic College of MedicineRochesterUSA

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