Publications by authors named "James Lilleker"
Background: Autoantibodies are found in up to 80 % of patients with idiopathic inflammatory myopathies (IIM) and are associated with distinct clinical phenotypes. Autoantibodies targeting cytosolic 5'-nucleotidase 1A (anti-NT5C1A) are currently the only known serum biomarker for the subgroup inclusion body myositis (IBM), although detected even in other autoimmune diseases. The aim of the study was to identify new autoimmune targets in IIM.
View Article and Find Full Text PDFReproducible and standardised neurological assessment scales are important in quantifying research outcomes. These scales are often performed by non-neurologists and/or non-clinicians and must be robust, quantifiable, reproducible and comparable to a neurologist's assessment. COVID-CNS is a multi-centre study which utilised the Neurological Impairment Scale (NIS) as a core assessment tool in studying neurological outcomes following COVID-19 infection.
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- The study aimed to analyze how damage from idiopathic inflammatory myopathies (IIM) changes over time and its relationship with different autoantibody subgroups using data from a large patient registry.
- Researchers examined data from 757 patients classified by their autoantibody profiles and found that damage increased over the years, with varying rates depending on the type of autoantibody present.
- Results indicated that patients with dermatomyositis-specific autoantibodies exhibited less damage per year, while those with anti-PM/Scl autoantibodies experienced greater damage, highlighting significant differences among the groups over a five-year follow-up.
Breakthrough SARS-CoV-2 infection in fully vaccinated patients with systemic lupus erythematosus: results from the COVID-19 Vaccination in Autoimmune Disease (COVAD) study.
Rheumatol Int
October 2024
Article Synopsis
- The study aimed to compare the occurrence of breakthrough COVID-19 infections (BIs) in fully vaccinated patients with systemic lupus erythematosus (SLE) against those with other rheumatic autoimmune diseases (rAIDs), non-rheumatic autoimmune diseases (nrAIDs), and healthy controls (HCs).
- Results showed that 10.7% of SLE patients reported at least one BI, with frequencies similar to HCs and nrAID patients but higher than those with other rAIDs.
- Overall, vaccinated SLE patients experienced comparable levels of COVID-19 infection frequency and severity as healthy individuals, with no significant association found between demographic factors or treatments and BIs in SLE patients.
Article Synopsis
- Late-onset Pompe Disease (LOPD) is a rare genetic disorder caused by a deficiency in an enzyme, leading to muscle damage and glycogen accumulation in cells.
- This study used advanced techniques like single nuclei RNA sequencing to explore gene expression changes in muscle biopsies from LOPD patients compared to healthy controls, revealing significant metabolic shifts and inflammatory responses in affected fibers.
- Findings suggest potential benefits of enzyme replacement therapy in restoring metabolic function, especially in healthy muscle fibers, emphasizing the importance of using modern methods to understand muscle disease at a cellular level.
A protocol for scoping reviews on the role of whole-body and dedicated body-part magnetic resonance imaging for assessment of adult and juvenile idiopathic inflammatory myopathies.
Rheumatol Int
November 2024
Article Synopsis
- * The review aims to gather and assess existing MRI scoring systems to create an evidence-based foundation for a universal standardized system that can be used in both research and clinical settings.
- * A systematic search of electronic databases will be conducted to consolidate information on MRI scanning protocols for evaluating muscle involvement in IIMs, with the goal of producing guidelines for consistent clinical and research practices.
Article Synopsis
- Minority ethnic groups are often underrepresented in research, which affects the validity of findings; the study aimed to improve recruitment strategies for a study on neurological issues related to COVID-19.
- Involving 807 participants, the research found a good representation of various ethnicities, with specific data showing centers in London attracted more non-White participants.
- The study concluded that it's possible to overcome recruitment barriers for underrepresented ethnic groups using targeted strategies identified in their research.
Objectives: To investigate health-related quality of life in patients with idiopathic inflammatory myopathies (IIMs) compared with those with non-IIM autoimmune rheumatic diseases (AIRDs), non-rheumatic autoimmune diseases (nrAIDs) and without autoimmune diseases (controls) using Patient-Reported Outcome Measurement Information System (PROMIS) instrument data obtained from the second COVID-19 vaccination in autoimmune disease (COVAD-2) e-survey database.
Methods: Demographics, diagnosis, comorbidities, disease activity, treatments and PROMIS instrument data were analysed. Primary outcomes were PROMIS Global Physical Health (GPH) and Global Mental Health (GMH) scores.
Since the publication of the 2013 European Neuromuscular Center (ENMC) diagnostic criteria for Inclusion Body Myositis (IBM), several advances have been made regarding IBM epidemiology, pathogenesis, diagnostic tools, and clinical trial readiness. Novel diagnostic tools include muscle imaging techniques such as MRI and ultrasound, and serological testing for cytosolic 5'-nucleotidase-1A antibodies. The 272nd ENMC workshop aimed to develop new diagnostic criteria, discuss clinical outcome measures and clinical trial readiness.
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- COVID-19 could trigger disease flares in rheumatoid arthritis (RA) patients, but the specific associated factors were unknown prior to this study.
- The study analyzed data from 1928 RA patients involved in the COVAD research, focusing on demographics, comorbidities, treatments, and disease flare details.
- Key findings indicate that younger age, non-Asian ethnicity, comorbidities, and certain treatments like methotrexate, along with aspects of physical and mental health, are important factors linked to disease flares in RA patients post-COVID-19.
Objectives: To explore prevalence, characteristics and risk factors of COVID-19 breakthrough infections (BIs) in idiopathic inflammatory myopathies (IIM) using data from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) study.
Methods: A validated patient self-reporting e-survey was circulated by the COVAD study group to collect data on COVID-19 infection and vaccination in 2022. BIs were defined as COVID-19 occurring ≥14 days after 2 vaccine doses.
Aim: To explore if patient global assessment (PGA) is associated with inflammation over time and if associations are explained by other measures of disease activity and function in patients with idiopathic inflammatory myopathies (IIM).
Methods: PGA and systemic inflammatory markers prospectively collected over five years were retrieved from the International MyoNet registry for 1200 patients with IIM. Associations between PGA, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and creatine kinase (CK) were analyzed using mixed models.
Article Synopsis
- The study investigates delayed adverse events (DAEs) from COVID-19 vaccinations in patients with systemic lupus erythematosus (SLE) compared to other autoimmune diseases and healthy individuals, with a focus on data scarcity impacting vaccine hesitancy among immunocompromised individuals.
- The research, conducted through a global online survey of over 7200 individuals, found that SLE patients experienced significantly more major DAEs and hospitalizations post-vaccination than healthy controls and had higher rates of severe rashes compared to those with other rheumatic diseases.
- The findings suggest a need for careful monitoring of SLE patients post-vaccination due to their elevated risk of adverse events, particularly after receiving the Moderna vaccine.
Patients with neuromuscular diseases (NMD) can present to the neurologist with symptoms and signs of respiratory failure, either acutely or as an insidious process in the outpatient setting. Since the advent of non-invasive ventilation, the outcomes of patients with ventilatory failure due to NMD have dramatically improved. However, the natural history of different NMDs requires a nuanced approach to respiratory investigation and management.
View Article and Find Full Text PDFThe idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of systemic autoimmune diseases that affect the skeletal muscles and can also involve the skin, joints, lungs and heart. The epidemiology of IIM is obscured by changing classification criteria and the inherent shortcomings of case identification using healthcare record diagnostic coding. The incidence of IIM is estimated to range from 0.
View Article and Find Full Text PDFObjectives: We aimed to investigate the gender-based differences in idiopathic inflammatory myopathies (IIMs), with a particular focus on patient-reported outcomes, utilizing the data obtained through the international COVID-19 vaccination in autoimmune disease e-survey.
Methods: Patient-reported outcomes including fatigue, pain, and physical function were extracted from the COVID-19 vaccination in autoimmune disease database and compared between genders, adjusting for demographics and IIM subgroups by multivariable analysis. Inclusion body myositis (IBM) was analysed separately because of the substantial differences in outcomes.
Objectives: To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM.
Methods: Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded.
This study aimed to assess the incidence, predictors, and outcomes of breakthrough infection (BI) following coronavirus disease (COVID-19) vaccination in patients with systemic sclerosis (SSc), a risk group associated with an immune-suppressed state and high cardiopulmonary disease burden. Cross-sectional data from fully vaccinated respondents with SSc, non-SSc autoimmune rheumatic diseases (AIRDs), and healthy controls (HCs) were extracted from the COVAD database, an international self-reported online survey. BI was defined according to the Centre for Disease Control definition.
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- * Rapid symptom progression was noted, with a median of just 3 days from symptom onset to hospital admission, and most patients received aggressive treatment like intravenous methylprednisolone and intravenous immunoglobulin.
- * While one patient died from complications, the remaining nine patients had positive recovery outcomes, suggesting that early multi-modal immunosuppression could be effective, indicating a need for standardized treatment protocols.
Objectives: We investigated coronavirus disease 2019 (COVID-19) vaccine safety in pregnant and breastfeeding women with autoimmune diseases (AID) in the COVID-19 Vaccination in Autoimmune Diseases (COVAD) study.
Methods: Delayed-onset (>7 days) vaccine-related adverse events (AE), disease flares and AID-related treatment modifications were analysed upon diagnosis of AID vs healthy controls (HC) and the pregnancy/breastfeeding status at the time of at least one dose of vaccine.
Results: Among the 9201 participants to the self-administered online survey, 6787 (73.
Limited evidence on long-term COVID-19 vaccine safety in patients with idiopathic inflammatory myopathies (IIMs) continues to contribute to vaccine hesitancy. We studied delayed-onset vaccine adverse events (AEs) in patients with IIMs, other systemic autoimmune and inflammatory disorders (SAIDs), and healthy controls (HCs), using data from the second COVID-19 Vaccination in Autoimmune Diseases (COVAD) study. A validated self-reporting e-survey was circulated by the COVAD study group (157 collaborators, 106 countries) from Feb-June 2022.
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