White Dot Syndromes: A 20-year Study of Incidence, Clinical Features, and Outcomes

Purpose: To measure the incidence of white dot syndromes (WDS) in a community-based population and to report clinical features and outcomes. Methods: Multicenter retrospective study using the Rochester Epidemiology Project medical records linkage system of Olmsted County, Minnesota. Databases were searched to identify all patients with WDS from January 1, 1988 through December 31, 2008. Results: Mean ophthalmic follow-up was 4.5 years and mean general medical follow-up was 9.1 years. The incidence of WDS was 0.45 per 100,000 per year (95% CI 0.19-0.71). Incidence rates for specific disease entities were also calculated. The authors report some associated autoimmune diseases in this series. Multiple evanescent white dot syndrome (MEWDS) was more common in females, and acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was more common in males. Both MEWDS and APMPPE generally carried a good visual prognosis. Fifty percent of cases with APMPPE had a positive history of psoriasis. The only punctuate inner choroidopathy (PIC) case carried that diagnosis as well. Conclusions: WDS are rare diseases and may be associated with other autoimmune diseases. Further studies with more patients and longer follow-up periods are needed to draw conclusions about visual prognosis, development of other ocular conditions, and associated medical diseases.