Publications by authors named "Ilaria Maccora"
Objective: Prognostic factors associated with medication discontinuation in children with juvenile dermatomyositis (JDM) remain largely elusive. We aim to identify the predictors of medication-free remission (MFR) in children with JDM.
Methods: In this retrospective study, patients diagnosed with JDM according to Peter & Bohan criteria and followed for ≥18 months at a tertiary care center from 2006 through 2022 were included.
Uveitis in paediatric Behçet disease: a large multicentric Italian cohort.
Ther Adv Musculoskelet Dis
December 2024
Background: Behçet disease (BD) is a rare disease in childhood and its uveitis may lead to blindness if not properly treated.
Objectives: We aim to describe a cohort of paediatric BD patients with uveitis.
Design: This is a multicentric retrospective study.
Background: Uveitis is an inflammatory ocular disease secondary to disruption of the retinal pigmented epithelium (RPE) and blood retinal barrier (BRB). Known clinical factors do not accurately predict uveitis risk in Juvenile Idiopathic Arthritis (JIA). Tear fluid is easily obtained for biomarker study.
View Article and Find Full Text PDFBrain MRI White Matter Abnormalities in Pediatric Non-Infectious Uveitis.
Ocul Immunol Inflamm
October 2024
Article Synopsis
- Childhood chronic non-infectious uveitis (cNIU) is a complex condition that can mimic other diseases, like demyelinating disorders, and this study focuses on the brain MRI findings in affected children.
- A total of 123 children with cNIU were analyzed, revealing that 26.8% exhibited white matter abnormalities (WMA), particularly among those with non-anterior uveitis and more frequently in males.
- The study suggests that while brain MRIs might be beneficial for monitoring cNIU, the clinical implications of the observed WMAs remain unclear, highlighting the need for interdisciplinary assessment and further long-term studies.
Article Synopsis
- Autosomal dominant neovascular inflammatory vitreoretinopathy (NIV) is a rare adult condition caused by CAPN5 gene mutations, which leads to eye inflammation; this study focuses on its treatment in children.* -
- Eight children were studied, receiving systemic immunosuppression after being diagnosed with NIV; although they initially responded to glucocorticoids, the effects were not sustained, and other treatments like methotrexate and infliximab were ineffective.* -
- The findings suggest that systemic therapies did not control the disease in these pediatric patients, highlighting the need for more research on effective long-term treatments for NIV.*
Article Synopsis
- Juvenile Idiopathic Arthritis (JIA) is a common chronic childhood condition that can lead to uveitis in 20-25% of cases, requiring regular screening for early detection.
- Uveitis is typically chronic, asymptomatic, and involves inflammation in the front part of the eye, making collaboration between paediatric rheumatologists and ophthalmologists essential for effective management.
- Treatment usually begins with methotrexate, and if ineffective, may progress to adalimumab, with alternative options like anti-IL6 and anti-TNF agents available for persistent cases, though evidence for these is limited.
Article Synopsis
- The study investigates treatment options for children with chronic non-infectious uveitis (cNIU) who don't respond to the initial anti-TNF therapy, focusing on whether to switch to another anti-TNF or another biologic.
- A systematic review and meta-analysis was done, analyzing data from January 2000 to August 2023, with 23 articles covering 150 children, to evaluate treatment efficacy based on intraocular inflammation improvement.
- Results indicate that both tocilizumab and infliximab show a higher response rate compared to other treatments, suggesting these might be better options for managing cNIU after the first anti-TNF fails.
Article Synopsis
- The study investigates the effects of stopping TNF inhibitors (TNFi) in children with juvenile idiopathic arthritis (JIA) who show persistent disease inactivity and identifies factors related to relapse.
- A multicentric analysis of medical records from 136 JIA patients who discontinued TNFi treatment revealed that 79.4% experienced a relapse within a median of 5 months after stopping therapy.
- Key predictors for relapse include younger age at onset, presence of uveitis, duration of treatment tapering, and not having persistent oligoarticular JIA.
Purpose: Autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) is a rare genetic (CAPN5) autoimmune condition typically diagnosed in adulthood and characterized by a triad of inflammation, retinal degeneration, and neovascularization. We report novel multimodal imaging findings in children and young adults with ADNIV, and early treatment response to short-duration local and systemic corticosteroids.
Design: Retrospective consecutive case series.
Objectives: To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children.
Methods: Prospective collection of demographics, clinical and treatment data. Assessment of type 1 interferon (IFN) score, CXCL9, CXCL10, Interleukin (IL)18, IFNγ, IL6, IL1b at disease onset and at recovery.
Article Synopsis
- Childhood Mixed Connective Tissue Disease (cMCTD) is a rare pediatric condition displaying symptoms of various connective tissue diseases, making it difficult to diagnose.
- A systematic literature review examined 39 articles involving 215 patients to identify early clinical features of cMCTD and to evaluate symptoms not included in existing diagnostic criteria.
- Findings revealed a diverse range of symptoms at onset, with common manifestations like Raynaud's phenomenon, arthritis, and muscular involvement, suggesting the need for new diagnostic approaches for earlier detection of cMCTD.
This narrative review aims to report the main clinical manifestations, therapeutic strategies, outcomes, and complications of acute SARS-CoV-2 infection in childhood and to summarize the data relating the SARS-CoV-2 vaccination efficacy and safety in pediatric age. SARS-CoV-2 infection mostly occurs asymptomatically in the pediatric population, while multisystem inflammatory syndrome in children (MIS-C) represents the most severe coronavirus disease 2019 (COVID-19)-related illness, a life-threatening event with a high morbidity rate. After the development of SARS-CoV-2 vaccines and their subsequent approval in children, the rate of infection as well as the number of its related complications have shown a drastic decrease.
View Article and Find Full Text PDF: Childhood chronic non-infectious uveitis (cNIU) is a challenging disease that needs close monitoring. Slit lamp evaluation (SLE) is the cornerstone of ophthalmological evaluation for uveitis, but it is affected by interobserver variability and may be problematic in children. Laser flare photometry (LFP), a novel and objective technique, might be used in children with uveitis.
View Article and Find Full Text PDFObjective: Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement, including interstitial lung disease (ILD). Available data on JIIM-associated ILD are very limited. We performed a systematic review of the available clinical, laboratory, and radiological features of JIIM-associated ILD.
View Article and Find Full Text PDFIntroduction: Interleukin-17 (IL-17) is a family of cytokines that plays a key role in several rheumatic diseases in both adults and children. In the last few years, several drugs targeting IL-17 have been developed.
Areas Covered: We present a review on the current state of the art regarding the use of anti-IL17 in childhood chronic rheumatic diseases.
Multisystem Inflammatory Syndrome in Children (MIS-C) is a systemic hyperinflammatory disorder that is associated with a hypercoagulable state and a higher risk of thrombotic events (TEs). We report the case of a 9-year-old MIS-C patient with a severe course who developed a massive pulmonary embolism that was successfully treated with heparin. A literature review of previous TEs in MIS-C patients was conducted (60 MIS-C cases from 37 studies).
View Article and Find Full Text PDFIntroduction: Deficiency of adenosine deaminase 2 (DADA2) is a rare monogenic autoinflammatory disease, whose clinical phenotype was expanded since the first cases, originally described as mimicker of polyarteritis nodosa, with immunodeficiency and early-onset stroke.
Methods: A systematic review according to PRISMA approach, including all articles published before the 31st of August 2021 in Pubmed and EMBASE database was performed.
Results: The search identified 90 publications describing 378 unique patients (55.