Prevention of Organ Damage Accrual Key to Preserving Quality of Life in Behçet Syndrome

Accrual of organ damage significantly affects the quality of life (QOL) of patients with Behçet syndrome; however, effective immunosuppressive treatment, prevention of relapses, and minimal use of steroids reduces the risk for organ damage accrual, according to study findings published in Arthritis Research & Therapy.

Behçet syndrome is a rare, chronic, multisystem disease that causes widespread vasculitis and tissue inflammation.

Researchers conducted a 2-year, multicenter, prospective, cohort study based on the Behçet syndrome Overall Damage Index (BODI) validation study. They aimed to assess the trajectory of organ damage accrual, associated risk factors, and impact of Behçet syndrome on health-related quality of life (HR-QOL).

A total of 189 of the 228 patients in the BODI study were enrolled in the follow-up study to analyze organ damage and associated factors, and data from 147 patients were assessed to analyze the association between organ damage accrual and HR-QOL.

During the 2-year follow-up, 36 patients (19.0%) demonstrated an increase in 1 or more points on their BODI scores, with an average change of 0.3 points (average at baseline vs 2 years, 1.6 vs 1.9; P <.001).

The number of patients reporting at least 1 area of damage increased from 114 (60.3%) to 131 (69.3%). During the follow-up period, patients experienced an onset of 61 new BODI items, the most frequent of which included diabetes (21.3%), mucocutaneous scars (11.5%), visual impairment (11.5%), and osteoporotic fracture or vertebral collapse (8.2%). Of the new BODI items reported, 25 (41.0%) definitely or probably correlated to prolonged glucocorticoid treatment for Behçet syndrome.

Risk factors predicting accrual of organ damage included:

• older age at study enrollment (median with vs without organ damage, 56.2 vs 46.6 years; P =.001),
• prolonged treatment with glucocorticoid therapy (median with vs without organ damage, 9.3 vs 2.0 years; P <.001),
• and, occurrence of at least 1 relapse (with vs without organ damage, 25% vs 13.1%; P =.07 on univariate analysis and P =.038 on multivariate analysis).

Treatment with conventional synthetic and biologic immunosuppressants decreased likelihood of developing organ damage (with vs without organ damage, 66.7% vs 86.9%; P =.004). Multivariate analysis confirmed the protective effect of conventional or biologic immunosuppressants (odds ratio [OR], 0.20; 95% CI, 0.08-0.54; P <.001).

The researchers used the Short Form (SF)-36 item questionnaire to assess HR-QOL. Based on SF-36 scores, they noted that organ damage accrual significantly affected physical roles (P =.018), physical functioning (P =.005), vitality (P =.006), general health (P =.004), social functioning (P =.035), emotional roles (P =.001), and mental health (P <.001) among patients with Behçet syndrome.

Independent risk factors associating organ damage accrual with decreased HR-QOL included the female sex, comorbid fibromyalgia, and higher disease activity levels based on the Behçet’s Disease Current Activity Form.

Study limitations included the relatively low percentage of patients with major organ involvement, the need for a longer follow-up duration, lack of validation of the definition of relapse for Behçet syndrome, and the need for further validation of BODI among more ethnically diverse cohorts and those with earlier forms of the disease.

“This study highlights how measuring and targeting organ damage is crucial in the management of BS to prevent its progressive accrual and the related impairment of perceived physical and mental health,” the study authors said. “From this perspective, the use of an adequate immunosuppressive therapy, minimizing steroid exposure, and lowering the risk of relapse seem to have a key role in preventing damage accrual,” they added.