Development of de novo major involvement during follow-up in XXXX .

@entity1 @entity16 @entity244
UNASSIGNED: The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of @entity1 with @entity244 ( @entity244 ); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those @entity1 who developed new major involvement. Among our cohort of 120 @entity244 @entity1 , 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 @entity1 characterized by a mild onset of disease, we observed that after at least 3  years from the diagnosis, 21 @entity244 @entity1 (34  %) still developed serious morbidities. Specifically, three @entity1 developed ocular involvement, nine @entity1 developed @entity16 and nine @entity1 presented vascular involvement. Comparing main epidemiological and clinical findings of the two groups, we observed that @entity1 who developed de novo major involvement were more frequently males and younger; furthermore, 95  % of these @entity1 were characterized by a young onset of disease (p   <   0.001). Being free of major organ complication in the first years of @entity244 is not necessary a sign of a favourable outcome. Globally, the development of de novo major involvement during the coursfce of @entity244 suggests that a tight control is strongly recommended during the course of the disease.
