PIRES MARAFON , Denise

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...

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...

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Objective. To develop a composite DAS for JDM and provide preliminary evidence of its validity. Methods. The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity; parent's/child's global assessment of child's wellbeing; measurement of muscle strength; and assessment...

To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing 8 (MMT8) and the Childhood Myositis Assessment Scale (CMAS), but is more comprehensive than the former and more feasible than the latter.

To evaluate the demographic, disease activity, disability and health-related quality of life (HRQoL) differences between children with juvenile idiopathic arthritis (JIA) and their healthy peers, and between children with JIA with and without clinical temporomandibular joint (TMJ) involvement and its determinants.