In a series of 281 patients with histologically confirmed sarcoidosis, 79 initially had ophthalmic sarcoid manifestations. Of 22 patients with uveitis, 21 were seen regularly as long as the inflammation was active, and 71 of the 79 patients (90%) underwent a follow-up study five to 16 years (mean, nine years) later and using the same protocol. In the 21 patients with uveitis, the disease exhibited either a monophasic course (eight patients) with favorable visual outcome or a relapsing course (13 patients) with severe visual loss in five eyes. Thirty-three patients showed chronic ophthalmic changes at the follow-up examination, including conjunctival granulomas (13 patients), lacrimal gland involvement (22 patients), uveitis (four patients), and involvement of lacrimal passages (three patients). In 15 of these 33 patients, the general physical examination and the chest x-ray showed no evidence of systemic sarcoidosis. However, the serum angiotensin converting enzyme level was increased in a significantly greater proportion of these 15 patients than in the patients assessed as totally recovered from sarcoidosis.