TBA (17A121)

Moving towards a National Behçet’s disease Registry: Results From a Single Registry in the Midwest of Ireland

Author(s)

Fahd Adeeb1,2, Alwin Sebastian1, Maria Usman Khan1,2, Wan Lin Ng1, Mary Brady1, Siobhan Morrissey1, John Paul Doran1, Austin Stack2, 3, Joseph Devlin1, Alexander D. Fraser1, 2

Department(s)/Institutions

1. Department of Rheumatology, University Hospital Limerick, Limerick, Ireland 2. Graduate Entry Medical School, University of Limerick, Limerick, Ireland 3. Department of Nephrology, University Hospital Limerick, Limerick, Ireland

Introduction

The epidemiology of Behçet's disease (BD) has been poorly studied in many parts of the world, especially among the non-endemic countries. One of the main challenges faced by the scientific community today relates to the magnitude of racial, regional and geographical phenotypic and genotypic differences among BD patients. An established national registry will enable physicians to improve disease awareness, assist in making informed decisions and enhance patients' overall outcome.

Aims/Background

1) To conduct a pilot project to establish a local BD registry and provide a framework for a national registry 2) to clarify the true prevalence, incidence and treatment strategies among BD patients in Ireland and to compare with other international studies.

Method

BD patients attending our rheumatology service were identified and specific clinical data were collected: 1) Basic demographics including ethnicity 2) time and age of diagnosis 3) evolution of clinical characteristics 4) HLA-B*51 status 5) pathergy phenomenon 6) details of treatment strategy, safety and efficacy

Results

24 Caucasian Irish patients (16 female, 8 male) and 1 male patient from the Middle Eastern ancestry were identified satisfying the diagnostic criteria for BD. The most common clinical manifestation was recurrent oral aphthosis (100%) followed by genital ulcerations (92%) and skin lesions (92%), arthralgia/arthritis (40%), ocular involvement (32%), vascular thrombosis (12%) and pathergy phenomenon (8%). The most frequently encountered skin lesion was pseudofolliculitis and/or papulopustular eruptions (18 patients, 72%) followed by erythema nodosum-like lesions, (2 patients; 8%). The most common ocular manifestation was unilateral uveitis (5 patients; 20%), while two patients had bilateral involvement. Two male patients (8%) lost their vision totally in one eye. Only 1 patient was positive for HLA- B*51. 18 patients (72%) were on anti-TNF (5 of which had potentially life-threatening structural laryngeal destruction). The point prevalence of BD on the 30th of June 2017 was 6.5 per 100,000 population.

Conclusions

The prevalence of BD in the Midwest of Ireland is higher than previously reported in the Northern European region. The establishment of this first BD registry in Ireland would hopefully drive further new research and collaborations to improve patients' overall outcome. We advocate for future collaborations among all rheumatology centres throughout Ireland to gain more insights into the epidemiology and determine the extent of geographical influences on this rather complex disease.