Abstract Objectives To evaluate the association between disease-specific autoantibody profiles and disease progression in systemic sclerosis-associated interstitial lung disease (SSc-ILD), with a focus on a quadruple-negative phenotype. Methods This retrospective cohort study included 80 patients with SSc-ILD who were followed for at least 12 months at a single tertiary centre. ILD progression was defined according to the OMERACT criteria and/or worsening on high-resolution computed tomography. Autoantibody status was categorized based on four antibodies: anti-topoisomerase I, anti-centromere, anti-RNA polymerase III, and anti-U1-RNP. Quadruple-negative (QN) was defined as negativity for all four antibodies. Factors associated with ILD progression were evaluated using multivariable logistic regression. Exploratory comprehensive autoantibody profiling was performed in patients with available stored serum samples. Results During a median follow-up of 44.5 months, disease progression occurred in 32 of 80 patients with SSc-ILD. Anti-centromere antibody (ACA) positivity was associated with a lower risk of progression, whereas anti-topoisomerase I antibody positivity was not associated with disease progression (Model 2: OR 3.19, 95% CI 0.70–14.60). Compared with ACA positivity, QN status was associated with ILD progression (Model 2: OR 10.40, 95% confidence interval [CI] 1.77–60.70). However, CIs were wide. Exploratory analyses showed a high prevalence of anti-Ro-52 antibody positivity among patients with QN status. Conclusion QN autoantibody status may be associated with an increased risk of ILD progression in SSc. The QN status may represent a heterogeneous serological category that could help identify patients who require closer clinical monitoring. However, these results are hypothesis-generating and require validation in larger, independent cohorts.
Abstract Objectives To report primary care prescribing rates of analgesics in people with PMR treated with glucocorticoids, overall and stratified by age, sex and deprivation. Methods A sample of > 43,000 individuals aged ≥50 years, with a coded PMR diagnosis January 2010-March 2022, prescribed glucocorticoids within 21 days of first PMR diagnosis code, was constructed in CPRD Aurum. Prescriptions for ‘all analgesics’, opioids, non-steroidal anti-inflammatory drugs (NSAIDs), gabapentinoids and amitriptyline were defined as prevalent (pre-PMR diagnosis), incident (at diagnosis), or late (post-diagnosis, still glucocorticoid-treated), reported overall and stratified by age/sex/deprivation. Results Whilst they were receiving glucocorticoids for PMR, 56.1% of participants were prescribed an analgesic. Opioids were the most frequently co-prescribed analgesics, followed by NSAIDs. Opioid prescribing was commoner in females than males, at older ages, and in more deprived areas. Amitriptyline and gabapentinoids were less commonly prescribed but followed the same sex and deprivation trends as opioids. NSAID prescribing was lower with older age, higher for males than females and did not vary with deprivation. Conclusion Analgesics are frequently co-prescribed with glucocorticoids in people with PMR despite lack of evidence of effectiveness and risks of adverse events. This evidences an unmet need for better pain management in PMR. The identified variation in prescribing of opioids and gabapentinoids by gender/deprivation is consistent with prescribing data for the general population and people with other painful musculoskeletal conditions and suggests that psychosocial-cultural factors affect pain and pain management in patients being treated for PMR. This needs further exploration to address inequalities and reduce iatrogenic harm.
Abstract Objectives The 2025 Paediatric Rheumatology Getting It Right First Time (GIRFT) National Report highlighted variation in service provision across England. Understanding current care pathways is essential to support equitable access and optimise outcomes for children and young people (CYP) with rheumatic conditions. We describe outpatient paediatric rheumatology activity across all commissioned and non-commissioned care providers in England. Methods Hospital Episode Statistics outpatient data for from April 2023-April 2025 were extracted for encounters coded to rheumatology or paediatric rheumatology for patients aged ≤17 years. Descriptive analyses examined patient-level characteristics, referral sources, and appointment types alongside diagnostic and procedural coding. Results A total of 174,093 encounters involving 41,115 patients were identified. Most encounters (131,355, 75.5%) was delivered by commissioned providers, with the remainder by 113 non-commissioned providers. Median age was 13 years (IQR 9–16). Fewer than half of new referrals originated from general practice. Diagnostic and procedural coding was missing in approximately 85% of encounters. Conclusion Paediatric rheumatology care is predominantly delivered to older CYP and occurs both within and outside commissioned providers, highlighting the need for strong regional collaboration to ensure equitable access to specialist multi-disciplinary care. The high proportion of referrals from outside general practice suggests persistently complex referral pathways. Education to raise awareness of JIA and referral guidance should therefore target clinicians most involved in early pathways. Improving outpatient diagnostic coding is essential to enable case mix analysis, service planning and evaluation of inequalities across providers.
Abstract Objectives Lung involvement in interstitial lung disease (ILD) with myositis-associated antibodies (myositis-ILD) predominantly has two radiologic patterns—nonspecific interstitial pneumonia (NSIP) and organizing pneumonia (OP)—that often coexist, yet clinical outcomes of these patterns remain unclear. We aimed to describe functional and radiologic trajectories during immunomodulatory therapy by predominant radiologic pattern in myositis-ILD. Methods In this retrospective, single-center cohort study, we studied participants from the Northwestern University ILD Registry with a circulating myositis-associated antibody, ILD, and at least 6 months of immunomodulatory therapy during a 24-month observation period. Two thoracic radiologists assigned the predominant radiologic pattern (NSIP or OP). The primary outcome was change in forced vital capacity (FVC); secondary outcomes were change in diffusing capacity (DLCO) and qualitative and quantitative (Kazerooni score) radiologic change. Groups were compared with Fisher exact and Wilcoxon rank-sum tests. Results Forty-one participants were characterized (29 [71%] OP-predominant, 12 [29%] NSIP-predominant). Both subgroups had improved FVC on immunomodulatory therapy (OP + 0.36 L [p = 0.006]; NSIP +0.25 L [p = 0.09]). DLCO did not change significantly in either subgroup (OP + 1.22 [p = 0.11]; NSIP -0.75 [p = 0.37]). The OP cohort improved on qualitative imaging analysis (p < 0.05), driven by reduced groundglass/consolidative opacity (p = 0.006). Participants with NSIP/OP overlap had greater baseline radiologic severity. Conclusions In myositis-ILD, both radiologic subgroups showed measurable functional and radiologic change during immunomodulatory therapy, with larger improvements in the OP-predominant subgroup; the magnitude of change did not differ significantly between subgroups. These descriptive, hypothesis-generating findings require multicenter validation.
