Cogan and Behcet syndromes.
Cogan and Behcet syndromes are considered large vessel vasculitides. Both are rare diseases, with varied clinical manifestations affecting multiple organ systems. Although both have hallmark symptoms (ocular and vestibuloauditory inflammation in Cogan syndrome and aphthous ulcers in Behcet syndrome), neither has confirmatory diagnostic testing. Delayed diagnosis can result in poor outcomes. In both syndromes, large vessel arterial inflammation may result in severe morbidity and mortality. Treatment strategies in both syndromes vary based on organ system involvement and severity of manifestations. In this article, the epidemiology, proposed pathogenesis, manifestations, and the most current treatment paradigms for these syndromes are reviewed.
Division of Rheumatology, University of Michigan, 300 North Ingalls Street, Suite 7C27, Ann Arbor, MI 48109-5422, USA. Electronic address: singero@med.umich.edu.
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Singer
D001299:Audiometry; D001528:Behcet Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D055952:Cogan Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009887:Ophthalmoscopy; D012720:Severity of Illness Index; D066167:Slit Lamp
D005938:Glucocorticoids; D007166:Immunosuppressive Agents
Behcet syndrome; Cogan syndrome; Large vessel vasculitis