Purpose
Behcet’s disease (BD) is uncommon in the United States (US). Prior studies have indicated different characteristics among different ethnic groups. The aim of this study is to describe demographic and clinical features, ocular and systemic manifestations in a US population with BD.
Methods
Electronic medical records of BD patients seen for ocular screening as part of an interdisciplinary clinical study (systemic cohort) and those seen as part of a natural history study for ocular disease (ocular cohort) at the National Eye Institute between 1999 and 2011 were reviewed. Data collected included demographics, clinical features, ocular and systemic manifestations. Patients were also categorized based on ethnicity into two groups: Caucasians of European descent (CEu) or Non-Caucasian or non-European descent (NCEu). Results were also compared to previously published large epidemiologic studies from different geographic regions.
Results
A total of 70 patients were identified. The mean age at diagnosis was 27.8 years, 44 were female (63%), 52 were CEu (74%), 32 (46%) were in the ocular cohort and 38 (54%) in the systemic cohort. Female-to-male (F/M) ratio in the entire cohort was 1.7; 2.0 among CEu, 1.0 among NCEu patients. HLA B51 positivity was 23% and 33% in the CEu and NCEu groups, respectively. Uveitis was slightly more common among NCEu compared to CEu patients (78% vs 67%), and posterior/panuveitis was the most common form of uveitis in all groups (71%). F/M ratio was higher in the systemic cohort (3.8) compared to ocular cohort whereas, HLA B51 positivity was higher in the ocular cohort (31%). Anterior uveitis was the most common anatomical location in the systemic cohort (53%) whereas posterior/panuveitis was the most common in the ocular cohort (88%). In terms of extraocular manifestations there was no significant difference between the ethnic categories or systemic and ocular cohorts. Compared to previous reports, F/M ratio in this study was higher and HLAB51 prevalence was lower.
Conclusions
BD in the US appears to have different clinical characteristics with more females affected and lower prevalence of uveitis as well as a lower HLAB51 prevalence among Caucasians of European descent. Posterior/panuveitis was the most common anatomical location for uveitis in the entire cohort. Extraocular manifestations were similar among different ethnic groups in the US BD patients.
Keywords: 746 uveitis-clinical/animal model •
461 clinical (human) or epidemiologic studies: natural history •
557 inflammation