Clinical Characteristics of Ocular Sarcoidosis: A Population Based Study 1976-2013

Background/Purpose: To characterize the epidemiology and clinical characteristics of ocular involvement in patients with systemic sarcoidosis.

Methods: An inception cohort of patients with systemic sarcoidosis in 1976-2013 in a geographically well-defined population was identified based on comprehensive individual medical record review.  Inclusion required physician diagnosis of sarcoidosis supported by histopathology of non-necrotizing granulomata, characteristic radiologic features of intrathoracic sarcoidosis and exclusion of other granulomatous diseases.  Medical records of those patients were then reviewed for ocular involvement.

Results: A total of 345 incident cases of systemic sarcoidosis were identified.  Ocular involvement occurred in 23 patients (mean age 51.8 years, 65% female and 78% Caucasian).  The most common ocular disease was uveitis (61%) followed by conjunctival nodule (17%).  Other types of ocular involvement that were observed in this cohort included episcleritis, anterior scleritis, conjunctivitis, lacrimal gland involvement, eyelid lesion, dry eye disease and optic neuritis.  ACE level was elevated in 83% of patients (10 out of 12 tested patients) while hypercalcemia was found in 46% of patients (6 out of 13 tested patients) in whom these tests were obtained.  All patients had intrathoracic involvement of sarcoidosis (hilar adenopathy and/or interstitial infiltration).

Among 14 cases with uveitis, 86% were female; 86% were Caucasian, 7% were African-American and 7% were Native-American.  Mean age at diagnosis of uveitis was 56.5 years.  Anterior uveitis was the most common type of uveitis (71%) followed by intermediate uveitis (21%), posterior uveitis (7%) and panuveitis (7%).  Uveitis was the initial presentation prior to the diagnosis of systemic sarcoidosis in 8 (57%) of 14 uveitis cases.  Visual acuity (VA) of patients with uveitis was generally good at diagnosis with the majority of eyes having VA of 20/20 to 20/25 in each eye.  Visual outcome was also good; only 2 of 24 affected eyes with adequate follow-up lost more than or equal to 3 lines of VA (define as losing more than or equal to 0.3 logMAR) during follow-up due to uveitis, and only 1 eye had VA worse than 20/200 at last visit. 

The majority of patients with sarcoid uveitis in this cohort were treated with topical glucocorticoids for the eyes (78%) and/or oral glucocorticoids (64%).  Disease modifying anti-rheumatic agents (DMARDs) and biologic agents were infrequently required (only 1 case with methotrexate and 1 case with hydroxychloroquine). 

Conclusion:  Ocular involvement occurred in 7% of sarcoidosis patients in this first ever population-based study of ocular sarcoidosis.  Uveitis was the most common type of ocular disease.  Visual outcome was generally good.