Publications by authors named "Giulia Camilla Varnier"
Objective: The objective of this study was to evaluate the efficacy and safety of MMF in juvenile idiopathic inflammatory myopathies (JIIMs).
Methods: Patients diagnosed with JIIM and treated with MMF enrolled in the Juvenile Dermatomyositis Research Group (JDRG) in the UK or followed at the Giannina Gaslini Institute in Genoa, Italy, were included. The following information was collected retrospectively at MMF initiation, at 3, 6 and 12 months after treatment start, and at last follow-up visit: clinical manifestations, laboratory data, physicians' subjective assessment of disease activity, standardized outcome measures of muscle strength/endurance, cutaneous disease activity, physical function, global disease activity, cumulative damage, and ongoing treatment.
Objectives: To search for predictors of polyarticular extension in children with oligoarticular-onset juvenile idiopathic arthritis (JIA) and to develop a prediction model for an extended course.
Methods: The clinical charts of consecutive patients with oligoarticular-onset JIA and ≥2 years of disease duration were reviewed. Predictor variables included demographic data, number and type of affected joints, presence of iridocyclitis, laboratory tests including antinuclear antibodies, and therapeutic interventions in the first 6 months.
Objectives: To compare the treatment approaches and disease outcomes of children with JDM followed in two European tertiary care peadiatric rheumatology centres.
Methods: The medical notes of patients with JDM seen at Istituto Giannina Gaslini (IGG) of Genoa, Italy or Great Ormond Street Hospital (GOSH) of London, UK between January 2000 and December 2015 within 6 months after disease onset and followed for at least 6 months were reviewed. Demographic, clinical and therapeutic data were collected.
Objectives: To investigate the frequency of arthritis flare and factors affecting occurrence of flare in children with juvenile idiopathic arthritis (JIA) who achieved inactive disease (ID) with methotrexate (MTX) monotherapy.
Methods: A total of 217 patients were included. The modality of treatment discontinuation, time of MTX withdrawal, and disease course were examined retrospectively.
Article Synopsis
- - The study aimed to create and validate a composite Disease Assessment Scale (DAS) specifically for Juvenile DermatoMyositis (JDM) through a new measurement tool called the Juvenile DermatoMyositis Activity Index (JDMAI).
- - JDMAI consists of four evaluation components, including assessments from both physicians and parents, muscle strength, and skin disease activity, validating its effectiveness across 627 patients from multinational samples.
- - Results showed that JDMAI has solid validity and reliability, making it a suitable tool for measuring disease activity in clinical and research settings; the final version will be confirmed after further prospective validation.
Purpose Of Review: This paper aims to provide a summary of the recent therapeutic advances and the latest research on outcome measures for clinical trials in juvenile dermatomyositis (JDM).
Recent Findings: Recent randomized controlled trials (RCTs) have demonstrated the superiority of the combination of prednisone with methotrexate over other conventional therapies and the potential effectiveness of rituximab in refractory cases. A multinational project has led to develop new criteria for the definition of minimal, moderate, and major improvement in future JDM clinical trials.
Article Synopsis
- A new hybrid muscle strength measure for juvenile dermatomyositis (JDM) combines Manual Muscle Testing (MMT-8) and key elements from the Childhood Myositis Assessment Scale (CMAS) to improve comprehensiveness and feasibility.
- The hybrid measure (hMC) was validated with 810 JDM patients, demonstrating strong validity, reliability, and responsiveness to treatment changes.
- The hMC is useful for routine clinical care, but further testing is needed in other patient populations to confirm its effectiveness.
Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA.
View Article and Find Full Text PDFObjective: To evaluate the measurement properties of 21-numbered circle visual analog scales (VAS) and traditional 10-cm horizontal line VAS for physician and parent subjective ratings in children with juvenile idiopathic arthritis (JIA).
Methods: We studied 2 patient samples in whom physician global rating of overall disease activity, parent global rating of the child's overall well-being, and parent rating of intensity of child's pain were performed using traditional 10-cm horizontal line VAS (n = 397) or 21-numbered circle VAS (n = 471). The measurement performances of the 2 VAS formats were examined by assessing construct validity, score distribution, responsiveness to change over time, and minimal clinically important difference (MCID).