Juvenile psoriatic arthritis (JPsA) accounts for 1–7% of all cases of juvenile idiopathic arthritis (JIA) and its definition has been a matter of controversy among pediatric rheumatologists for many years. The traditional attribution of JPsA to the spondyloarthropathy group was challenged in the early 1990s, whereas the recent demonstrations of its heterogenous nature have led to questions about its identification as a distinct category in JIA classification. It has been shown that children with the phenotype of JPsA can be divided in two subgroups, one presenting with the features of early-onset ANA-positive JIA, and another that belongs to the spectrum of spondyloarthropathies. The few studies that have compared the clinical characteristics and genetic determinants of JPsA with those of the other JIA categories have obtained contrasting findings. The debate on the categorization of JPsA as a distinct entity within JIA classification is still ongoing and has prompted the revision of its current classification.

TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly) syndrome is a rare, life‐threatening inflammatory condition linked to infections, neoplasms, and idiopathic multicentric Castleman disease. Interleukin (IL)‐6 inhibitors are the primary treatment, but refractory cases require alternatives. This study reports the first two pediatric TAFRO cases successfully treated with anakinra, an IL‐1 receptor antagonist. Both patients had severe, rapidly progressing disease with multiorgan involvement. Anakinra, combined with corticosteroids, led to significant improvement and remission. We provide a literature review of pediatric TAFRO, confirming its rarity and the partial efficacy of IL‐6 inhibitors in many cases.

Objective: to seek for predictors of inactive disease (ID) in juvenile idiopathic arthritis (JIA) with artificial intelligence. Methods: The clinical charts of patients seen within 6 months after disease onset between 2007 and 2019 and with follow-up visits at 6, 12, 18, and 24 months were reviewed retrospectively. Sixty-eight potential predictors were recorded at each visit. The primary endpoint was ID at 24 months by 2004 Wallace criteria. Data obtained from diverse combinations of visits were examined to identify the best forecasting model. After pre-processing, the cohort was divided into training (50%) and testing (50%) cohorts. Multivariate time series forecasting, coupled with the Random Forest method, was used to train the machine learning (ML) forecasting model. Predictive performance was assessed through the Matthews correlation coefficient (MCC). Results: A total of 414 patients were included. The best performance in predicting ID at 24 months in the training cohort was provided by the 0–12 months interval (MCC = 0.68). In the testing cohort, the same ML model confirmed a high forecasting performance (MCC = 0.65). Assessment of feature importance and impact analysis showed that the most relevant predictor of ID was the physician’s global assessment (PhGA), followed by the count of active joints (AJC). Conclusions: PhGA and AJC values over the first 12 months were the strongest predictors of ID at 24 months. This finding highlights the importance of regular quantitative assessment of disease activity by the caring physician in monitoring the course of the patient toward achievement of complete disease quiescence.

The “biologic era” in the management of juvenile idiopathic arthritis (JIA) has begun in the year 2000, with the publication of the randomized controlled trial on etanercept. In the subsequent years, there has been continued progress, marked by the availability of new therapeutic agents and the shift towards early aggressive interventions. In addition, a more rational therapeutic approach has been fostered by the promulgation of therapeutic recommendations and guidelines. In parallel with the growing use of the novel biologic disease-modifying antirheumatic drugs (bDMARDs) in the real world of clinical practice, additional information has been gained about their effectiveness and safety. Furthermore, the role of the various bDMARDs in the management of the different JIA categories and of the main disease complications and comorbidities has been scrutinized. Innovative management strategies, such as the step-down and the treat-to-target, have been proposed to maximize the therapeutic benefits through the optimal combination of the newer and conventional medications. However, despite this progress several unmet needs remain, including the lack of well-established criteria for medication discontinuation after the attainment of sustained disease remission and of effective alternatives for patients who respond inadequately to the contemporary therapeutic modalities. The research agenda also calls for the search for reliable early predictors of therapeutic response that foster personalization of treatment and increase its precision. The aim of this Review is to summarize the evidence obtained in the past 5 years in the field of biologic therapy for JIA and to discuss the remaining gaps and the future perspectives of the use of these medications.