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AB0630 (2022)
Assessment of organ damage accrual in Behçet's Syndrome over 2-year follow-up: results from the BODI Project longitudinal extension.
A. Floris1,2, R. Laconi1,2, G. Espinosa3, G. Lopalco4, L. Serpa Pinto5, N. Kougkas6,7, J. Sota8, A. Lo Monaco9, M. Govoni9, L. Cantarini8, G. Bertsias6, J. Correia5, F. Iannone4, R. Cervera3, C. Vasconcelos10, A. Mathieu1, A. Cauli1,2, M. Piga1,2, on behalf of BODI project group
1University of Cagliari, Medical Sciences and Public Health, Monserrato, Italy
2AOU University Clinic and University of Cagliari, Rheumatology Unit, Cagliari, Italy
3University of Barcelona, Department of Autoimmune Diseases, Hospital Clinic, Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
4University of Bari , Rheumatology Unit, Bari, Italy
5Hospital Santo Antonio Centro Hospitalar do Porto, Unidade de Imunologia Clinica, Porto, Portugal
6University of Crete, Rheumatology, Clinical Immunology and Allergy Unit, Heraklion, Greece
7Aristotle University of Thessaloniki, Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Thessaloniki, Greece
8University of Siena , Rheumatology Unit, Siena, Italy
9University of Ferrara, Rheumatology Unit - AOU S. Anna, Ferrara, Italy
10University of Porto, UMIB Abel Salazar Biomedical Sciences Institute, Porto, Portugal

Background: Preventing accrual of organ damage is a major goal in the treatment of Behçet’s Syndrome (BS). The BS Overall Damage Index (BODI) is the first damage assessment tool developed and preliminarily validated for BS.


Objectives: To assess the prevalence, extent, and determinants of organ damage accrual in the BODI validation cohort over 24 months of follow-up.


Methods: Overall, 189 patients from the multicenter BODI cohort underwent a 24 ±3 months follow-up (FU) visit. Demographics, ongoing medication, Behçet’s Disease Current Activity Form (BDCAF) score, Physician (PGA) and Patient Global Assessment (PtGA) of disease activity, disease relapsing (defined by any treatment change due to increased disease activity), and the BODI score were recorded. Damage accrual was defined as any increase ≥1 in the BODI score between baseline and follow-up visit (Δ-BODI). Logistic regression models were built to identify factors associated with BODI damage accrual.


Results: The mean age (standard deviation) at enrolment and the disease duration was 46.2 (12.1) and 10.8 (8.3) years, respectively, and 92/189 (48.7%) patients were males. During 24 months, 36 (19.0%) patients had an increase in the BODI score of at least 1 point (mean increase of 1.7 points). The BODI score increased from 1.6 (2.1) to 1.9 (2.1), with a mean Δ-BODI of 0.3 (0.8). Overall, 61 new BODI items of damage were recorded ( Figure 1 ); 22 (34%) were steroid-related (diabetes, osteoporotic fractures, cataract). Factors independently associated with increased BODI score were longer glucocorticoids exposure (OR 1.01 per month, 95%CI 1.01-1.02, p<0.001), and occurrence of flares (OR 3.1, 95%CI 1.1-8.9, p = 0.035), whereas stable treatment with conventional and/or biologic immunosuppressants was negatively associated with an increase in the BODI score (OR 0.19, 95% 0.07-0.97, p <0.001) ( Table 1 ).

Determinants of organ damage accrual over 2 years of follow-up.

Univariate analysis Multivariate analysis
Candidate determinants Δ-BODI ≥1 (n 36) Δ-BODI = 0 (n 153) p OR (95%CI) p
Males 16 (44.4%) 76 (49.7%) 0.572
Age at enrolment 56.2 (42.9-62.0) 46.6 (35.4-53.1) 0.001 -- --
Disease duration 12.9 (7.1-22.0) 11.1 (5.4-21.2) 0.483
Major organ involv. 22 (61.1%) 72 (47.1%) 0,129
BDCAF at BL 3 (0-5) 2. (0-5) 0.365
BDCAF at FU visit 3.0 (3-5) 3 (0-7) 0.188
GC duration 112 (26.0-147.0) 24.0 (8.0-72.0) <0.001 1.012 (1.006-1.018 <0.001
cIS or TNFì ever 24 (66.7%) 133 (86.9%) 0.004 0.194 (0.073-0.972 ) <0.001
Relapse 9 (25.0%) 20 (13.1%) 0.070 3.093 (1.066-8.972 ) 0.038
BODI score at BL 1.0 (0-2.0) 1 (0-2) 0.579

Continuous variables are presented as median (IQR). Dichotomic variable are presented as n (%). BODI, Behçet’s Syndrome Overall Damage Index. FU, follow-up. cIS, conventional immunosuppressant. Δ-BODI increase of BODI score from baseline to the FU visit.


Conclusion: Despite the relatively high disease duration in the studied cohort, organ damage accrual was recorded in a relevant proportion of patients. BODI proved to capture the damage associated with major determinants such as inadequate control of disease activity and prolonged exposure to glucocorticoids.


Disclosure of Interests: None declared


Citation: , volume 81, supplement 1, year 2022, page 1442
Session: Vasculitis - small vessel vasculitis (Publication Only)