Methods

We performed a retrospective consecutive observational case series of all 69 patients with peripheral multifocal chorioretinitis who were assessed at our hospital from 1992 through 2007 and who completed a follow-up course of at least 5 years; 39 of these patients were followed for at least 10 years. The Institutional Review Board of the University Medical Center Utrecht waived the need for approval of this retrospective chart review. The study adhered to the tenets of the Declaration of Helsinki.

We extracted each patient’s demographics and each patient’s clinical, laboratory, and imaging findings from the hospital records. We noted the development of complications, including macular edema, macular pucker, cataract, optic atrophy, ocular hypertension, and glaucoma, as well as all ocular surgical interventions. Ocular comorbidity was also noted, as well as any medications used during the entire follow-up period. Throughout the follow-up period, none of the 69 patients took any biological pharmaceutical agent. The data were collected at disease onset and again at 1 and 5 years after onset; where available, data were also recorded at the 10-year follow-up visit. Data regarding VA were available at all follow-up visits for 31 patients.

Peripheral multifocal chorioretinitis was defined as the combined presence of multiple (>10) small round “punched-out” lesions in the peripheral retina, an absence of central chorioretinal lesions, and the presence of associated intraocular inflammatory disease. The diagnostic criteria for ocular sarcoidosis—as defined by the Scientific Committee of the First International Workshop on Ocular Sarcoidosis—were applied as well as was possible. In brief, a diagnosis of definite sarcoidosis was given to biopsy- or bronchoalveolar lavage–confirmed cases, and a diagnosis of presumed sarcoidosis was given to patients who had radiographic signs characteristic of sarcoidosis but no available biopsy data. In this study, a diagnosis of probable sarcoidosis was given to patients who did not have a positive chest radiograph or, if available, a positive chest computed tomography (CT) scan, but had classic signs of peripheral multifocal chorioretinitis and serum angiotensin-converting enzyme (ACE) levels >30 U/L; in our own laboratory, the diagnostic threshold for serum ACE is 20 U/L.

Macular edema was defined either as leakage in the macular area on fluorescein angiography or in accordance with the following optical coherence tomography (OCT) criteria. For Stratus Time-Domain OCT (Carl Zeiss Meditec AG, Jena, Germany), macular edema was defined as retinal thickness >249 μm (the mean thickness of nonaffected retinas plus 2 standard deviations) and/or the presence of retinal cysts. After 2008, Cirrus Spectral-Domain OCT (Carl Zeiss Meditec AG, Jena, Germany) was used; using this method, macular edema was defined as retinal thickness >304 μm and/or the presence of retinal cysts. Chronic macular edema was defined as macular edema that lasted longer than 1 year. In the data analysis, recurrent macular edema was also classified as chronic macular edema. Macular epiretinal membrane was diagnosed based on funduscopy and confirmed by OCT when possible.

Glaucoma was diagnosed based on the presence of an excavated optic disc, glaucomatous visual field loss, and concurrent elevated intraocular pressure. Papillitis was defined as optic disc edema and/or a leaking, hyperfluorescent optic disc on fluorescein angiography.

If available, the VA of each eye was obtained from the medical records at the onset of uveitis; 1, 5, and 10 years after onset; and at the end of the follow-up period (for patients who were followed for longer than 10 years). For analysis, VA was transformed to logMAR values. Amblyopic eyes were not included in the VA measurements.

The data were analyzed using SPSS 20.0 (IBM, Armonk, New York, USA). The Mann-Whitney U test was used to analyze continuous variables and the Fisher exact test was used to analyze categorical variables. Prognostic factors for low VA at the 5-year and 10-year follow-up visit were studied using generalized estimating equations with statistical correction to test for correlations between the 2 eyes of 1 patient. Characteristics with a relevant effect on outcome in the univariate analyses were evaluated further using a multivariate analysis. All statistical tests were 2-sided, and differences were considered significant if P < .05.

Results

The 69 patients in this retrospective study had a median age at onset of peripheral multifocal chorioretinitis of 64 years (range: 4–87 years); 46 of the 69 patients (67%) were 60 years of age or older at the time of onset. The general clinical characteristics of our study cohort are summarized in Table 1 . The majority of patients were female (58/69, 84%) and/or white (66/69, 96%). Twenty-six of 67 patients (39%) were diagnosed with definite or presumed sarcoidosis; of these, 17 patients (68%) were diagnosed while screening for uveitis. Thirteen of 63 patients had an abnormal chest radiograph and 14 of 18 patients had an abnormal CT scan. Associated thyroid disorders were noted in 10 of 69 patients (14%). The ophthalmic features that developed during the course of the disease ( Figure ) are summarized in Table 2 . We found no significant difference between patients with systemic sarcoidosis and patients without systemic sarcoidosis with respect to the occurrence of clinical manifestations (for all clinical manifestations, P > .05). During the course of the disease, papillitis was observed in 61 of 64 patients (95%).

Fluorescein angiography of a patient with active peripheral multifocal chorioretinitis. The patient is a 67-year-old white woman with bilateral disease, 1 year after onset of the uveitis and prior to systemic treatment. (Left) The nasal-inferior periphery of the left eye contains several lesions leading to “punched-out” scars. (Right) Fluorescein leakage from the posterior pole of this eye reveals the presence of cystoid macular edema and papillitis.

The development of ocular comorbidity and complications is summarized in Table 3 . Macular edema was the most prevalent complication of peripheral multifocal chorioretinitis, developing in at least 1 eye in 63 of 69 patients (91%) within the follow-up period. Further analysis per eye revealed that macular edema was present in 42 of 69 eyes (61%) at the onset of peripheral multifocal chorioretinitis (data were available in only 69 of 134 eyes), in 67 of 86 eyes (78%) at 1 year, in 34 of 74 eyes (46%) at 5 years, and in 19 of 43 eyes (44%) at 10 years. During the entire follow-up period, the cumulative incidence of macular edema was 81% (ie, 108 of 134 eyes had developed macular edema). The presence of systemic sarcoidosis had no influence on the risk of developing macular edema ( P = .49).

Within the follow-up period, optic disc atrophy was observed in 17 of 64 patients (25%), glaucoma was observed in 24 of 69 patients (35%), and an epiretinal membrane was observed in 32 of 68 patients (47%). No optic atrophy was found in the 3 patients without papillitis; and in 16 of 60 patients with papillitis (27%) optic disc atrophy occurred. In the group of patients with papillitis, the age at onset of uveitis and the duration of follow-up did not differ between the patients with or without optic disc atrophy ( P = .11 and P = .89, respectively). Within the papillitis group, the prevalence of sarcoidosis did not differ between the patients with or without optic disc atrophy ( P = .77); however, the prevalence of AMD differed between these 2 groups (4/16 vs 1/44, respectively; P = .02).

The therapeutic interventions are listed in Table 4 . One patient received eye drops as the sole treatment. Periocular corticosteroid injections were used to treat 56 of 69 patients (81%). Immunosuppressive drugs were given to 13 of 69 patients (19%), and 24 of 69 patients (35%) received a systemic course of prednisone; in total, 30 of 69 patients (44%) received systemic corticosteroids and/or immunosuppressive drugs. Fifty-eight of the 69 patients (84%) underwent a surgical intervention in at least 1 eye; the majority of surgical interventions were cataract surgeries. Although 24 patients developed glaucoma, only 3 of these patients required surgical intervention. Glaucoma was not significantly associated with the use of periocular steroid injections ( P = .52). In addition, the patients with peripheral multifocal chorioretinitis and systemic sarcoidosis did not differ significantly from the patients with peripheral multifocal chorioretinitis without systemic sarcoidosis with respect to their medical and/or surgical treatment modalities ( P > .1). Sixteen patients underwent pars plana vitrectomy (PPV); however, PPV did not affect the need for local or systemic medical therapy.

The time course of VA in the patient cohort is summarized in Table 5 . At onset, the median VA of all affected eyes was 0.50. Median VA at the 1-year follow-up was 0.50; at the 5-year follow-up, median VA was 0.61 ( P = .08 compared to the VA at onset), and median VA at 10 years was 0.68 ( P = .23 compared to the VA at onset). At the 5-year follow-up, 25 of 66 patients (38%) had visual acuity of 20/63 or worse in at least 1 eye. At onset, 5 years, and 10 years, 7 of 112 affected eyes (6%), 11 of 126 affected eyes (13%), and 7 of 72 affected eyes (10%), respectively, had VA worse than 20/200. VA decreased to worse than 20/200 in 19 of 131 eyes (14%) at the end of follow-up, with a median follow-up period of 10 years (range: 5–21 years). Approximately one third of affected eyes had VA worse than 20/40 at both 5 and 10 years of follow-up, which was significantly lower than at onset, in which 43% of affected eyes had VA worse than 20/40 ( P < .005 for both the 5-year and 10-year follow-up times). Bilateral loss of VA to worse than 20/200 had occurred in 4 of 69 patients (6%) at the end of follow-up.

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