In CS, IK is usually treated initially with topical ocular corticosteroid drops, such as prednisolone acetate 1%, and mydriatics. The IK usually responds within three to seven days. If IK fails to improve with topical corticosteroid therapy, then chlamydial infection should be considered as a possible alternative diagnosis. Only rarely does IK require systemic corticosteroid therapy. Conjunctivitis, anterior uveitis, and episcleritis/scleritis may also be treated initially with topical corticosteroids. Oral nonsteroidal anti-inflammatory drugs may benefit some patients with episcleritis/scleritis and may be added to the topical regimen. Refractory cases may be treated with systemic corticosteroids or other immunosuppressive agents.

Posterior segment inflammation should be treated with systemic corticosteroids beginning with prednisone 1 mg/kg/day. Other immunosuppressive drugs, such as methotrexate, cyclophosphamide, or cyclosporine, may be required for those patients who fail to respond adequately to two to three weeks of high-dose corticosteroid therapy (Table 2). Failure to taper the prednisone dose below 10mg/day or the development of corticosteroid toxicity may also be an indication for using another immunosuppressive drug.

Vestibular dysfunction or a decrease in auditory acuity in a newly diagnosed patient with CS warrants a trial of systemic corticosteroid therapy. No clinical trials of any therapy have been done in CS, due to the rarity of this condition. Patients with hearing loss are usually treated initially with prednisone 1 to 2 mg/kg/day, often in divided doses for three to seven days before consolidating to a single daily dose. With improvement in hearing after two to three weeks, the dose of prednisone is tapered gradually over the next six to eight weeks to an every-other-day regimen. Further tapering of the prednisone dose depends on stability of auditory and vestibular function. Some patients may require long-term corticosteroid therapy to maintain hearing function. Decline in auditory function warrants an increase in prednisone dose to 0.5 to 1 mg/kg/day, depending on the severity of the hearing loss.

Treatment with a corticosteroid-sparing agent is often considered for patients who develop corticosteroid toxicity or require excessively high prednisone doses to control hearing loss. In these cases, consideration may be given to using cyclophosphamide, methotrexate, azathioprine, cyclosporine, or tacrolimus (Table 2). The evidence for the efficacy of these agents is only anecdotal.

The treatment of systemic vasculitis and other organ system manifestations is beyond the scope of this chapter. However, patients with systemic vasculitis, including aortitis, are usually treated with high doses of prednisone, 1 to 2 mg/kg/day, in combination with methotrexate, azathioprine, or cyclophosphamide. In addition, cyclosporine may be considered in patients with a Takayasu's-like arteritis.

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