Background/Purpose: Behçet’s disease (BD) is globally characterized by a variable spectrum of disease profile: while prevalent muco-cutaneous lesions and arthritis represent the only clinical features in patients with a benign disease subset, there are other patients who develop potentially sight or life-threatening manifestations, due to ocular, neurological or major vascular involvement. The primary aim of the study was to evaluate the incidence of the novo major involvement during the follow-up in a cohort of  patients with BD; the secondary aim was to analyze the epidemiological profile and the long-term outcome of those patients who developed de novo major involvement.

Methods:  One hundred and twenty patients were evaluated. The male/female ratio was 1.6:1, with a mean disease duration of 11±5 years. Their mean age was 42±9 years (min:18, max:77), while the mean age at disease onset was 25±4 years (min:10, max:58). The mean ± SD duration of follow-up at our centre was 8±2 (min:2, max:12) years. We have defined the development of de novo major involvement during the follow-up as the occurrence of severe ocular (anterior uveitis, posterior uveitis, retinal vasculitis), vascular (deep vein thrombosis, superficial vein thrombosis, arterial thrombosis, arterial aneurysm) or CNS (ischaemic pons-mesencephalon lesions and meningoencephalitis) involvement after a latency period from the diagnosis of at least 3 years

Results: At the time of diagnosis, the 52% of the cohort (n=62) presented a prevalent muco-cutaneous involvement. Among this sub group of patients, we observed that after at least 3 years from the diagnosis, 21 patients (34%)  was characterized by the occurrence of de novo major involvement (i.e. ocular: 3; CNS: 9; vascular: 9). The demographic profile of this subgroup (male: 19, female: 2) was characterized by a mean age of 33±4 years (min 24-max 40) and a young age at disease onset. The long term outcome after a mean follow-up of 8 years has shown that the majority of these subjects presented during the disease course also relapsing attacks. Moreover, they were characterized by a bigger number of DMARDs (both traditional and anti TNF-alpha)  compared to the other part of the cohort

Conclusion: As awaited, younger age and male sex represent predictive factors of poor long-term clinical outcome. We have found that ocular disease seems the organ involvement that more frequently has an onset before the first years of disease. Globally, he development of the novo major involvement during the course of BD represents an entity that may confirm that a tight control is strongly recommended during the course of BD

ACR Meeting Abstracts - https://acrabstracts.org/abstract/development-of-de-novo-major-involvement-during-the-follow-up-in-behcets-disease/