Vasculitis damage index in Behçet’s disease

Abstract

Background Vasculitis damage index (VDI) is a validated damage index for systemic vasculitis, and as Behçet’s disease is considered one of systemic vascular disease we aimed to study the relationship of the vasculitis damage index to clinical manifestations and comorbidity in patients with Behçet’s disease (BD) to determine if VDI could be used to assess damage in patients with BD. Methods A total of 109 patients with BD were recruited from the Rheumatology Department (outpatient and inpatient clinic), Cairo University Hospitals. All patients were subjected to full history taking, clinical examination, and routine laboratory investigations. Disease activity was assessed by the BD current activity form, and the VDI was calculated in all patients. The relationship of the VDI to the disease clinical manifestations was studied. Mann–Whitney and Kruskal Wallis tests were used to estimate differences in quantitative variables. Spearman correlation test was used to test for correlation between quantitative variables. Results In the current study, the VDI ranged from 1 to 10, with a mean of 3.5 ± 1.8. It was significantly associated with total thrombosis (P = 0.022); total neurological manifestations (P = 0.000), especially stroke and cranial nerve affection; uveitis (P = 0.005); avascular necrosis (AVN) (P = 0.015); osteoporosis (P = 0.01); impaired vision (P < 0.0001); cataract (P < 0.0001); and diabetes (P = 0.012). Generally, immunosuppressive treatment was significantly associated with VDI (P = 0.039), especially cyclophosphamide (P < 0.0001), biological agent (P = 0.008), chlorambucil (P = 0.003), and anticoagulant (P = 0.02). VDI was also significantly correlated with age (P = 0.033), disease duration (P = 0.029), and duration of eye involvement (P = 0.003). Conclusion VDI is significantly associated with most disease parameters of BD, except for parameters such as mucocutaneous manifestations and uncomplicated venous thrombosis; however, further studies may be needed to establish BD-specific damage index.

Damage accrual in Behçet disease: Behçet's Syndrome Overall Damage Index (BODI) versus Behçet's Disease Damage Index (BDI)

“…This can be explained by the diversity of the items scored under each organ system in each index. Regarding the vascular system, VDI includes complicated peripheral venous thrombosis (superficial varicosities, thrombophlebitis) but does not record other complicated venous occlusions such as Budd‐Chiari syndrome, cerebral venous sinus and vena cava thrombosis 8 . On the other hand, BODI includes vena cava thrombosis, pulmonary artery aneurysm and thrombosis despite excluding the pulmonary system.…”

“…18 This agreed with previous reports of Piga et al and Elgengehy et al who found that mucocutaneous damage and ocular damage were the most common. 8,9 On the other hand, Suppiah et al stated that ENT and neuropsychiatric systems had the most frequent organ damage as determined by VDI. 17 correlation.…”

“…Accordingly, The Outcome Measures in Rheumatology (OMERACT) has emphasized that assessment of both BD activity and damage are mandatory, separate and complementary items in the BD outcome measures 7 . Considering that BD is classified as a type of vasculitis, the most frequently used measure for the assessment of damage in BD is the Vasculitis Damage Index (VDI) 8 . There was no specific tool for the assessment of organ damage in BD until 2020 when Piga et al introduced and initially validated the Behçet's Syndrome Overall Damage Index (BODI) as the first specific damage index for BD 9 …”

“…7 Considering that BD is classified as a type of vasculitis, the most frequently used measure for the assessment of damage in BD is the Vasculitis Damage Index (VDI). 8 There was no specific tool for the assessment of organ damage in BD until 2020 when Piga et al introduced and initially validated the Behçet's Syndrome Overall Damage Index (BODI) as the first specific damage index for BD. 9 Moreover, Gheita et al in 2021, published and validated a new simple and specific Behçet's Disease Damage Index (BDI) and concluded that BDI is a rapid and comprehensive tool for the proper assessment of BD damage in clinical research and daily practice.…”

Osteonecrosis as a rare musculoskeletal complication in Behcet’s disease- the largest case series with literature review

“…Vascular involvement in BD may also play a role in progression of ON, however, there is not enough evidence to support this hypothesis [ 21 ]. In the study by Elgengehy et al Vasculitis damage index in BD patients is significantly associated with ON [ 22 ]. Vascular damage and impaired endothelial dysfunction play different role in development of ON [ 23 ].…”