Atypical Cogan Syndrome Featuring Orbital Myositis and Dacryoadenitis

Abstract

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.

From precise diagnosis to effective treatment: a case report of atypical Cogan’s syndrome

“…Due to the variable onset symptoms of CS and the lack of specific laboratory or radiological examination, the diagnosis of CS is more challenging and diagnostic criteria consensus is lacking . At present, CS is diagnosed mainly based on the clinical diagnosis of auditory vestibular symptoms, ocular inflammation with negative syphilis serological test and vasculitis confirmed by histology [8] , as well as the positive response to corticosteroid treatment [9] . At present, the treatment of reported cases mainly depends on the involved organs, systems, and the severity of the disease [10][11][12] .…”

Systemic Vasculitis and Its Association with the Eye

“…Other common manifestations are episcleritis and scleritis and occasionally uveitis [111,115]. Less common manifestations are choroidal effusion, dacryoadenitis, orbital inflammation, and orbital myositis [116][117][118].…”