Non-infectious Orbital Vasculitides
Non-infectious Orbital Vasculitides
Cogan syndrome (CS) is characterized by a bilateral nonsyphilitic interstitial keratitis and audiovestibular involvement. One of the systemic manifestations of the disease is a necrotizing vasculitis, which can affect vessels of all sizes. Some of the more common vessels involved are the aorta, renal arteries, and coronary arteries. Atypical CS (ACS) was defined in 1980 by Haynes et al; it encompasses CS along with other ocular manifestations including orbital inflammation, scleritis, retinitis, glaucoma, artery occlusions, and optic neuritis. CS and ACS classically occurs in young adults and equally in males and females.
The patients will typically develop vertigo, imbalance, and hearing loss in addition to blurred vision, conjunctival injection, and eye pain. Oftentimes there is a 2-year delay between the presentation of ocular and audiovestibular symptoms. Orbital involvement is uncommon; there have been four cases reported in the literature. These patients presented with hearing loss and vertigo along with exophthalmos and orbital inflammation. CT demonstrated evidence for myositis as well. None of these patients underwent an orbital biopsy and therefore it is difficult to determine if the inflammation was truly secondary to a vasculitis. Treatment of ACS consists of a glucocorticoid and occasionally an immunodulatory agent. Patients with orbital inflammation described in the literature were successfully treated with a combination of prednisone and cyclophosphamide, although given the scarcity of cases it is uncertain if cyclophosphamide is a necessary addition to the treatment regimen.
Atypical Cogan Syndrome
Cogan syndrome (CS) is characterized by a bilateral nonsyphilitic interstitial keratitis and audiovestibular involvement. One of the systemic manifestations of the disease is a necrotizing vasculitis, which can affect vessels of all sizes. Some of the more common vessels involved are the aorta, renal arteries, and coronary arteries. Atypical CS (ACS) was defined in 1980 by Haynes et al; it encompasses CS along with other ocular manifestations including orbital inflammation, scleritis, retinitis, glaucoma, artery occlusions, and optic neuritis. CS and ACS classically occurs in young adults and equally in males and females.
The patients will typically develop vertigo, imbalance, and hearing loss in addition to blurred vision, conjunctival injection, and eye pain. Oftentimes there is a 2-year delay between the presentation of ocular and audiovestibular symptoms. Orbital involvement is uncommon; there have been four cases reported in the literature. These patients presented with hearing loss and vertigo along with exophthalmos and orbital inflammation. CT demonstrated evidence for myositis as well. None of these patients underwent an orbital biopsy and therefore it is difficult to determine if the inflammation was truly secondary to a vasculitis. Treatment of ACS consists of a glucocorticoid and occasionally an immunodulatory agent. Patients with orbital inflammation described in the literature were successfully treated with a combination of prednisone and cyclophosphamide, although given the scarcity of cases it is uncertain if cyclophosphamide is a necessary addition to the treatment regimen.
