DE BENEDETTI , Fabrizio

Most juvenile idiopathic arthritis (JIA) patients need to attend adult rheumatology centres to continue the clinical management of their disease and to receive adequate long-term treatment. Transition from the paediatric to the adult health care team is a critical moment in the clinical history of these patients, but unfortunately, about 50% of...

Objective: To evaluate the expression of type I interferon (IFNα/β)– and type II IFN (IFNγ)–inducible genes in muscle biopsy specimens from patients with juvenile dermatomyositis (DM) and to correlate their expression levels with histologic and clinical features. Methods: Expression levels of IFN-inducible genes and proinflammatory cytokines...

Objectives Interferon-gamma (IFN gamma) is the pivotal mediator in murine models of primary haemophagocytic lymphohistiocytosis (pHLH). Given the similarities between primary and secondary HLH (sec-HLH), including macrophage activation syndrome (MAS), we investigate the involvement of the IFN gamma pathway in MAS by evaluating levels of IFN...

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Objectives To predict the occurrence of inactive disease in JIA in the first 2 years of disease. Methods An inception cohort of 152 treatment-naïve JIA patients with disease duration <6 months was analysed. Potential predictors were baseline clinical variables, joint US, gut microbiota composition and a panel of inflammation-related compounds...

To present the results of a Delphi consensus survey among Italian paediatric and adult rheumatologists on transitional care (TC) of young people (YP) with juvenile idiopathic arthritis (JIA).

Adenosine deaminase 2 deficiency (DADA2) is an autoinflammatory disease characterized by inflammatory vasculopathy, early strokes associated often with hypogammaglobulinemia. Pure red cell aplasia, thrombocytopenia, and neutropenia have been reported. The defect is due to biallelic loss of function of ADA2 gene, coding for a protein known to...