Characteristics of Behcet's Disease in the American Southwest

Abstract

Objective: The goal of this study was to determine the characteristics of Behçet's disease (BD) in the American Southwest.

Material and methods: This was a cross-sectional study of BD patients clinically encountered during a 2-year period. All subjects fulfilled the International Study Group criteria (ISG) or International Criteria for Behcet's Disease (ICBD). Age, gender, clinical characteristics, substance use, and HLA-B51 status were determined.

Results: 63 patients (female: male ratio: 4.7:1) fulfilled ISG criteria and 76 the ICBD criteria (estimated prevalence of 8.9-10.6 per 100,000). 84.1% (53/63) were initially diagnosed with non-BD primary diagnoses including inflammatory arthritis (15.9%), fibromyalgia (7.9%), vasculitis (7.9%), or systemic lupus erythematosus (7.9%). Common BD manifestations were oral aphthous ulcers (100%), acneiform lesions (69.8%), genital aphthous ulcers (61.9%), papulopustular lesions (52.4%), pseudofolliculitis (42.9%), inflammatory arthritis (41.3%), anterior uveitis (23.8%), posterior uveitis (15.9%), pathergy (15.9%), deep vein thrombosis (14.3%), non-ocular vasculitis (11.1%), erythema nodosum (7.9%), arterial thrombosis (6.3%), and retinal vasculitis (1.6%). BD ethnic proportions were 49.2% Hispanic American (HA), 31.7% European-American (EA), 14.3% Native American (NA), and 1.7% Silk Road. HLA-B51 was present more in NA (89.0%, p = 0.02) and HA (74.2%, p = 0.02) compared to EA (42.1%). Therapy of BD was conventional, except for the frequent use of hydroxychloroquine.

Conclusions: BD is common in the American Southwest with a prevalence of 8.9-10.6 cases per 100,000. BD patients are commonly initially diagnosed with alternative primary conditions. Hydroxychloroquine may be an effective alternative therapy for BD. This is one of the first reports of BD in HA and NA populations.

Keywords: Behçet's disease; Ethnicity; HLA-B51.