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Multicenter Study
. 2022 Nov 17;24(1):253.
doi: 10.1186/s13075-022-02947-y.

Accrual of organ damage in Behçet's syndrome: trajectory, associated factors, and impact on patients' quality of life over a 2-year prospective follow-up study

Alberto Floris #  1   2 , Matteo Piga #  3   4 , Riccardo Laconi  1   2 , Gerard Espinosa  5 , Giuseppe Lopalco  6 , Luisa Serpa Pinto  7 , Nikolaos Kougkas  8   9 , Jurgen Sota  10 , Andrea Lo Monaco  11 , Marcello Govoni  11 , Luca Cantarini  10 , George Bertsias  8 , João Correia  7 , Florenzo Iannone  6 , Ricard Cervera  5 , Carlos Vasconcelos  12 , Alessandro Mathieu  1 , Alberto Cauli  1   2
Affiliations
Multicenter Study

Accrual of organ damage in Behçet's syndrome: trajectory, associated factors, and impact on patients' quality of life over a 2-year prospective follow-up study

Alberto Floris et al. Arthritis Res Ther. .

Abstract

Background: This study aimed to investigate the trajectory of damage accrual, associated factors, and impact on health-related quality of life (HR-QoL) in a multicenter cohort of patients with Behçet's syndrome (BS) over 2 years of follow-up.

Methods: Patients recruited in the BS Overall Damage Index (BODI) validation study were prospectively monitored for 2 years and assessed for damage accrual, defined as an increase ≥1 in the BODI score, and HR-QoL was evaluated by the SF-36 questionnaire. Logistic and multiple linear regression models were built to determine factors associated with damage accrual and impairment in the different SF-36 domains.

Results: During follow-up, 36 out of 189 (19.0%) patients had an increase ≥1 in the BODI score with a mean (SD) difference of 1.7 (0.8) (p <0.001). The incidence rate of damage accrual was stable over time, regardless of the disease duration. Out of 61 new BODI items, 25 (41.0%) were considered related to glucocorticoid (GC) use. In multivariate analysis, duration of GC therapy (OR per 1-year 1.15, 95% CI 1.07-1.23; p <0.001) and occurrence of ≥1 disease relapse (OR 3.15, 95% CI 1.09-9.12; p 0.038) were identified as predictors of damage accrual, whereas the use of immunosuppressants showed a protective effect (OR 0.20, 95% CI 0.08-0.54, p<0.001). Damage accrual was independently associated with the impairment of different physical domains and, to a greater extent, in emotional domains of the SF-36 questionnaire. Female sex, higher disease activity, and fibromyalgia were also significantly associated with impairment in HR-QoL.

Conclusion: In BS, organ damage accrues over time, also in long-standing disease, resulting in an impairment of the perceived physical and mental health. Adequate immunosuppressive treatment, preventing disease flares and minimizing exposure to GCs have a crucial role in lowering the risk of damage accrual.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Rate of patients experiencing accrual of organ damage over the 2 years of follow-up according to the disease duration at enrolment. Pts, patients. BODI, Behçet’s syndrome Overall Damage Index; GC, glucocorticoids
Fig. 2
Fig. 2
Type of damage accrual classified according to A the potential relationship with glucocorticoid treatment and B the involved organs/systems (BODI domains). GC, glucocorticoid; BODI, Behçet’s Syndrome Overall Damage index. § 13/14 Diabetes. *Cardiac and vascular BODI domains were merged
Fig. 3
Fig. 3
Radar plot representing the mean values of each SF-36 domain in patients having (∆-BODI ≥1) or not (∆-BODI=0) accrual of organ damage over the 2 years of follow-up. PF, physical function; RP, role-physical; BP, body pain; GH, general health; VT, vitality; SP, social function; RE, role emotional; MH, mental health. * p <0.05, § p <0.01, # p <0.001 in multiple regression
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