Background/Purpose: Behçet’s disease (BD) is a multisystemic, chronic relapsing inflammatory disease classified among the vasculitis. Neurological and eye involvement are two of the most serious manifestation of BD and both still represent significant causes of morbidity. The primary aim of the study was to evaluate the long-term outcome in a cohort of patents with BD; the secondary aim was to explore any potential correlation between long-term outcome and demographic and clinical profile.

Methods:  The study enrolled 122 patients, all fulfilling the International Study Group (ISG) criteria for BD. The male/female ratio was 1.6:1, with a mean disease duration of 11±4 years. Their mean age was 42±9 years (min:18, max:77), while the mean age at disease onset was 25±4 years. The mean ± SD duration of follow-up at our centre was 10±2 years. Long-term outcome was evaluated by means of disease flares according the BD Current Activity Form (BDCAF), persistent low-level of disease (defined as a level minimal activity of disease requiring only low-medium doses of corticosteroids), and disease damage according the Vasculitis Damage Index (VDI). The statistical analysis was performed using Student t-test, Mann-Whitney-U test, ANOVA and Pearson correlation

Results: The main clinical features presented during the follow-up by the cohort were: mucocutaneous involvement (100%), joint involvement (49%), ocular involvement (40%), neurological involvement (38%), vascular thrombotic events (22%), gastro-enteric involvement (16%). Globally, we observed 108 episodes of disease flare, of which: 25% of patients with neurological involvement, 60% of ocular involvement, 50% of muco-cutaneous involvement, 12% of joint involvement, 50% of vascular involvement and 28% of gastro-enteric involvement. Thirty-four patients presented a persistent low-level disease, that required a medium corticosteroids dose of 4 mg of 6-methylprednisolone. Moreover, 39 patients presented a VDI > 1. A significant correlation was found between disease flares and younger age and male sex; persistent low-level of disease and disease damage resulted significantly associated with early onset of disease and high number of disease flare in the first 2 years of disease. Notably, persistent low-level of disease and disease damage resulted inversely correlated with the use of anti TNF alpha agents, independently of the type of organ involvement.

Conclusion: As literature data suggest, a specific demographic profile exists of poor outcome in BD, represented by young males. The use of anti TNF alpha agents is associated with a positive effect on maintaining remission of disease, most likely due to their steroid sparing effect.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/disease-flares-and-persistent-low-level-of-disease-long-term-outcome-in-a-cohort-of-patients-with-behcets-disease/