Table 1.
Primary objectives | To gather as much data as possible from a large cohort of patients enrolled on an international basis | |
To obtain real-world experience applicable to all geographic contexts | ||
To identify prognostic factors capable of tapering patients' management and treatment in the light of a personalized medicine approach | ||
Additional objectives | Regarding diagnosis | To highlight disease differences in the severity and modalities of presentation of the disease according to the geographical context |
To assess the reproducibility (sensitivity/specificity) of the different classification/diagnostic criteria currently used for sJIA and AOSD | ||
To cluster disease features in order to identify subgroups of patients with different prognosis or requiring different treatment strategies | ||
Regarding prognosis | To look for any impact of diagnostic delay on disease prognosis | |
To identify any possible predisposing factor and trigger inducing disease exacerbations | ||
To better characterize the behavior of the disease during pregnancy and postpartum period | ||
To monitor the cardiovascular risk, adjusting for treatments employed | ||
To identify clinical and biological factors predisposing to MAS development, which is the most frequent life-threatening complication of Still's disease | ||
To assess whether and how disease course has changed due to the current diagnostic and therapeutic evolution | ||
Regarding therapy | To define a treat-to-target strategy regarding how to use corticosteroids, conventional immunosuppressants and biotechnologic agents in the earliest phase of the disease | |
To evaluate the best timing to start biotechnologic treatment | ||
To assess starting posologies and posologies adjustments | ||
To look for evidence on the tapering and withdrawal of treatment strategies | ||
To assess the socioeconomic impact of the disease before and after treatment | ||
To explore the therapeutic options and results about the pharmacological agents used in this severe condition | ||
To identify different diagnostic strategies fitting with regional areas and evaluating the treatment response according to the resources available worldwide | ||
To describe old and new therapeutic regimens, specifically focusing on their global efficacy and role on the different features of the disease | ||
Ancillary objectives | To quickly find patients to be potentially included in randomized controlled trials | |
To think about retrospective and prospective studies capable of answering future unmet needs |
Primary objectives have been distinguished from additional objectives: the former represents the general purposes at the basis of the Registry development, while the latter consist of the main lines of research the AIDA Network will follow in next years.