Clinical Manifestations

For patients with IK alone, the most common ocular complaints are pain, redness, and photophobia. Other eye symptoms may include excessive tearing, foreign body sensations, and blurry vision. Conjunctivitis, anterior uveitis, episcleritis, scleritis, and retinal vasculitis may be accompanied by eye pain, redness, photophobia, or reduced visual acuity. Acute angle glaucoma and proptosis have also been described in a few cases. The ophthalmological findings of IK may be relatively mild and evanescent and may consist of faint, peripheral, subepithelial corneal infiltrates. These lesions are similar to those of keratitis caused by adenovirus and chlamydia. The corneal lesions can evolve to a patchy granular infiltrate, localized predominately in the posterior cornea. The cornea can later vascularize and opacify with persistent inflammation, although such opacities occur in less than 5% of adequately treated patients.

The most common presenting vestibuloauditory complaints include Meniere's-like attacks with vertigo, nausea and vomiting, sudden hearing loss, and tinnitus. The vestibular component may be severe enough to warrant hospitalization. Sometimes, patients may present with a gradual decrease in hearing, of variable severity. Ataxia, nystagmus, and oscillopsia may also be present. The severe Meniere's-like attacks usually resolve within a few days. In one study, the mean interval between the onset of ocular and vestibuloauditory disease was three months (20).

The majority of patients with CS exhibit systemic manifestations. Cardiovascular disease develops in 10% to 15% of patients, with aortic value regurgitation developing in about 10% of these cases (29). Aortic valve insufficiency results from aortitis, with or without associated valvulitis. Other cardiac features observed in patients with CS include ostial coronary disease, coronary arteritis, myocardial infarction, pericarditis, and arrhythmias.

In CS, the most common type of vasculitis affects the large vessels and resembles Takayasu's arteritis. A large-sized vessel vasculitis, depending on the nature and severity of disease, may present with an asymptomatic bruit, intermittent limb claudication, severe hypertension, or constitutional complaints. Less frequently, CS may be associated with a medium-sized vessel vasculitis similar to polyarteritis nodosa. In these cases, the signs and symptoms will originate from the site of vessel involvement and may include purpura, multiple mononeuropathies, mesenteric ischemia, and glomerulonephritis.

Other manifestations associated with CS are fever, fatigue, weight loss, myalgias, arthralgia/arthritis, hepatomegaly, splenomegaly, lymphadenopathy, various types of rashes (urticaria, nodules, purpura ulcers, and pyoderma gangrenosum), pleuritis, lymphocytic meningitis, encephalitis, cerebral vascular accident, cerebellar syndrome, myelopathy, seizures, peripheral neuropathy, cranial neuropathies, and chondritis.

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