Development of de novo major involvement during follow-up in Behçet’s syndrome

Abstract

The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of patients with Behçet's syndrome (BS); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those patients who developed new major involvement. Among our cohort of 120 BS patients, we evaluated all subjects who had no major organ involvement during the early years of their disease; specifically, at disease onset, the 52% of the cohort presented a prevalent mucocutaneous involvement. The primary outcomes were represented by the following: Hatemi et al. (Rheum Dis Clin North Am 39(2):245-61, 2013) the incidence of de novo major involvement during the follow-up and Hatemi et al. (Clin Exp Rheumatol 32(4 Suppl 84):S112-22, 2014) the use of immunosuppressive drugs during the follow-up. We have defined the development of de novo major involvement during the follow-up as the occurrence of severe ocular, vascular or CNS involvement after a latency period from the diagnosis of at least 3 years. Among 62 patients characterized by a mild onset of disease, we observed that after at least 3 years from the diagnosis, 21 BS patients (34%) still developed serious morbidities. Specifically, three patients developed ocular involvement, nine patients developed neurological involvement and nine patients presented vascular involvement. Comparing main epidemiological and clinical findings of the two groups, we observed that patients who developed de novo major involvement were more frequently males and younger; furthermore, 95% of these patients were characterized by a young onset of disease (p < 0.001). Being free of major organ complication in the first years of BS is not necessary a sign of a favourable outcome. Globally, the development of de novo major involvement during the coursfce of BS suggests that a tight control is strongly recommended during the course of the disease.

Relationship between age at onset and clinical characteristics of Behçet’s disease

“…It must be kept in mind that, in the afore mentioned study, patients 24 years old or younger at onset were assigned to the early-onset group (9). Furthermore, Talarico et al (11) showed that 95% of patients that developed major organ involvement during follow-up had experienced disease onset before they were 25 years old with major organ involvement defined as eye disease, vascular involvement, and neurological involvement; this is only partially in agreement with the findings reported here.…”

Being a caregiver of a Behçet’s syndrome patient: challenges and perspectives during a complex journey

“…2 Psychotherapist, Translational Analyst, Rome, Italy. 3 Institute of Management, Scuola Superiore Sant' Anna, Pisa, Italy. 4 • support for research data, including large and complex data types • gold Open Access which fosters wider collaboration and increased citations maximum visibility for your research: over 100M website views per year…”

“…BS is characterized by a variable spectrum of disease profile; while prevalent muco-cutaneous involvement and arthritis represent the main clinical features in patients with a benign disease subset, there are other patients who potentially develop sight or life-threatening manifestations, due to ocular, neurological or major vascular involvement [2]. The relapsing nature of the disease can determine exacerbations and remission of symptoms over time; moreover, being free of major organ complications in the first years is not necessarily a sign of a favourable outcome, since about one third of patients may experience the development of de novo major involvement during the course of BS [3]. Various demographic factors are considered predictable of poor outcome in the short and long-term, such as age at disease onset, duration of disease or gender.…”

Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

“…BD is clinically characterised by multiple organ involvement, in particular by the “triple symptom complex,” consisting of recurrent oral aphthosis, genital ulcers, and recurrent bilateral uveitis. Besides this classical clinical trial, BD recognizes also other organ involvements, as summarised in Table 1 [ 24 – 28 ].…”