Development and preliminary validation of the Behçet’s syndrome Overall Damage Index (BODI)

Abstract

ObjectiveTo develop and validate the evidence-based and consensus-based Behçet’s Syndrome Overall Damage Index (BODI).MethodsStarting from 120 literature-retrieved preliminary items, the BODI underwent multiple Delphi rounds with an international multidisciplinary panel consisting of rheumatologists, internists, ophthalmologists, neurologists, and patient delegates until consensus was reached on the final content. The BODI was validated in a cross-sectional multicentre cohort of 228 patients with Behçet’s syndrome (BS) through the study of (a) correlation between BODI and Vasculitis Damage Index (VDI) and (b) correlation between BODI and disease activity measures (ie, Behçet’s Disease Current Activity Form (BDCAF), Physician Global Assessment (PGA), Patient Global Assessment (PtGA)), c) content and face validity and (d) feasibility.ResultsThe final BODI consists of 4 overarching principles and 46 unweighted-items grouped into 9 organ domains. It showed good to excellent reliability, with a mean Cohen’s k of 0.84 (95% CI 0.78 to 0.90) and a mean intra-class correlation coefficient of 0.88 (95% CI 0.80 to 0.95). Overall, 128 (56.1%) patients had a BODI score ≥1, with a median score of 1.0 (range 0–14). The BODI significantly correlated with the VDI (r=0.693, p<0.001), demonstrating to effectively measure damage (construct validity), but had greater sensitivity in identifying major organ damage and did not correlate with disease activity measures (ie, BDCAF: p=0.807, PGA: p=0.820, PtGA: p=0.794) discriminating damage from the major confounding factor. The instrument was deemed credible (face validity), complete (content validity) and feasible by an independent group of clinicians.ConclusionsPending further validation, the BODI may be used to assess organ damage in patients with BS in the context of observational and controlled trials.

Development and validation of a Behçet's Disease Damage Index for adults with BD: An Explicit, Composite and Rated (ECR) tool

“…There was an emerging demand to propose a damage index specific for BD. A recent study published the first tool for describing and measuring organ damage in BD patients, namely the BD Overall Damage Index (BODI) originating from 10 centers in 4 European countries 10 . The BODI showed good to excellent reliability, and significantly correlated with the VDI, and is pending for further validation.…”

Vasculitis damage index in Behçet’s disease

“…In contrast, some items mentioned in VDI may not be of value in patients with BD, such as alopecia and proteinuria. Thus, according to the aforementioned reasons, in our opinion, BD modified specific VDI version as that studied by Piga et al 2020 and including many modified items of VDI [14], may be needed for better assessment of damage in patients with BD, which must be assessed in further prospective, large scale, longitudinal studies.…”

“…The Outcome Measures in Rheumatology (OMER-ACT) has defined the core set domain of outcome measures for BD, and concluded that both measures of disease activity and damage should be included, as separate and complementary entities, into the core outcome set for BD [13]. However, no BD damage index had been developed until 2020, when Piga et al published the first BD damage index (BODI) and validated it through several steps, one of which was the correlation with VDI, and they concluded that pending further validation still required [14]. Thus, this study aimed to evaluate the relationship of the VDI to clinical manifestations and comorbidities in patients with BD.…”

Advances in the Treatment of Behcet’s Disease

“…Although widely used as low dose (5-10 mg/day prednisolone) for MC manifestations and high-dose (0.5-1 mg/kg/day prednisolone)/pulse (0.5-1 g methylprednisolone) regimens for remission-induction of major organ disease, studies on the optimal dose and duration of GCs, as well as the extent of GC-associated damage are limited with controversial results in BD [5,6]. Interestingly, in a validation cohort of a recently developed Behcet's syndrome overall damage index (BODI), "ever use of GCs" are not found to be associated with overall damage score [7]. This surprising observation either reflects the young age and overall lack of comorbidities in BD population or the limitation of the tool to adequately assess GC-related damage.…”