Prognosis and Disease Activity

Abstract

Behçet’s disease (BD) is globally characterized by a variable spectrum of disease profile: while prevalent mucocutaneous 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. Beside the organ involvement, demographic factors could considerably influence the long-term and short-term outcomes of BD: age at disease onset, duration of disease, gender, and sex. Younger men patients are more suitable to have a more severe disease, due to an increased frequency both of morbidity and mortality, related to ocular, vascular, and neurological involvement. Eye involvement represents one of the most serious manifestation of BD and occurs in half of the patients. It seems more frequent and more severe among young males and, unluckily, it still remains one of the most significant causes of morbidity. Usually, ocular disease develops within the first years of disease onset and it seems to be more severe in this period; moreover, growing data suggest that the prognosis of BD patients with ocular involvement is mainly dependent upon the severity of visual acuity at presentation. Although not included in the ISG criteria for BD, neurological involvement represents the second main cause of mortality, preceded by large vessel disease. Parenchymal CNS involvement represents a serious morbidity of disease, often leading to disability and, if not treated early, to mortality, while dural sinus thrombosis is associated with a more favorable outcome than parenchymal involvement. The prevalence of vascular involvement varies from 20 to 35 % of BD patients and it may involve all types of vessels within the arterial and venous system. It is characterized by a clear male preponderance. Vascular involvement in BD represents a serious risk for multiple vessel-related complications, including thromboses, stenoses, occlusions, and aneurysms. Since there are no established laboratory findings to define BD and the diagnosis remains mainly dependent on the identification of the typical clinical pictures, to a certain extent, there are no optimal measures that would simplify the evaluation of the disease. Unluckily, there are no validated biomarkers that could reflect disease activity over time.

None of the authors had potential conflicts of interest.