Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis causing multi-organ damage and long-term disability. Various treatments including IL-5-targeting agents and other biologics have recently emerged, prompting regular updates of clinical practice guidelines. This scoping review and systematic review (SR) aimed to evaluate the efficacy and safety of rituximab and benralizumab in EGPA. A scoping review was conducted to identify relevant clinical questions, followed by an SR of trials published between December 2018 and July 2023. Studies comparing rituximab or benralizumab with standard treatments were included. Risk of bias (RoB) was assessed using RoB 2 and quality of evidence was rated using GRADE. One randomised controlled trial (RCT; n = 105) comparing rituximab with conventional therapy, showed a 180-day remission rate of 63.5% versus 60.4% (risk ratio: 1.05, 95% CI: 0.78-1.42), with very low certainty due to serious RoB and imprecision. Another RCT compared benralizumab with mepolizumab, showing similar rates of remission (58.6% versus 55.7%), relapse (30%), and glucocorticoid tapering, with low certainty owing to imprecision. Rituximab showed no clear benefit over conventional therapy, whereas benralizumab demonstrated comparable efficacy and safety to mepolizumab. However, evidence remains limited, and further EGPA-specific trials are needed.

This real-world study evaluated the effectiveness of baricitinib on activities of daily living (ADL) and health-related quality of life (HRQoL) in patients with rheumatoid arthritis (RA) in Japan and explored the association between early pain and fatigue improvements and 12-month remission. The study involved patients with RA aged ≥20 who initiated baricitinib and were followed for 12 months. Patient-reported outcomes (PROs) were assessed using visual analogue scale, Locomo 5, EQ-5D-5L, and SF-36 subscales. The study included 353 patients (mean age 63.4 years). Baricitinib substantially reduced pain levels from a mean (SD) of 48.4 (26.5) mm, fatigue levels from 42.3 (28.3) mm, and morning stiffness from 51.8 (126.9) min at baseline, to 25.2 (21.4) mm, 24.3 (22.7) mm, and 15.8 (38.7) min, respectively, at 12 months. Clinically meaningful improvements were observed in ADL (Locomo 5 scores: 6.9 [5.7] to 4.7 [5.3]) and HRQoL (EQ-5D-5L index scores: 0.700 [0.217] to 0.829 [0.166]) over 12 months. Logistic regression analysis revealed that early improvements in pain and fatigue were predictive of disease remission at 12 months. Baricitinib improved PROs in patients with RA in Japan, with early symptom relief predicting mid-term disease remission.

This study aimed to determine whether interferon regulatory factor 5 (IRF5) activation and its temporal dynamics in immune cells correlate with disease activity and flares in patients with systemic lupus erythematosus (SLE). Patients with SLE were prospectively enrolled and followed up for 52 weeks. The IRF5 nuclear translocation rate (NTR) was assessed in monocytes, dendritic cells, and B cells during baseline and follow-ups. Associations among IRF5 NTR, disease activity, and flares were statistically analyzed. Of 40 patients enrolled, 27 were included in the analysis after meeting all inclusion criteria. Baseline IRF5 NTR in B cells was correlated with SLE disease activity index scores (r = 0.529, p = 0.005). Nineteen patients developed flares, and high baseline IRF5 NTR showed good predictive accuracy (AUC = 0.763) and an increased flare risk (OR = 25.5, p = 0.003). In survival analysis, patients with high IRF5 NTR had significantly shorter flare-free survival (log-rank p = 0.030) and higher hazard of flare (HR = 7.43, p = 0.010). IRF5 NTR further increased during the 12 weeks preceding flares (p = 0.040). The IRF5 NTR in B cells is a potential biomarker for disease activity and flare prediction in SLE.

This study explored the role of cellular senescence in the progression of rheumatoid arthritis (RA) and evaluated the targeting of Cyclin E2 (CCNE2) in synovial fibroblasts as a potential therapeutic approach. A risk prediction model for RA was developed using LASSO regression analysis, which involved analyzing differential gene expression and performing Gene Set Enrichment Analysis (GSEA). The model was validated using the Receiver Operating Characteristic (ROC) curve. CCNE2 expression was examined via Western blotting. Knockdown of CCNE2 in RA synovial fibroblasts (RASFs) using shRNA resulted in reduced cell viability, increased apoptosis, and elevated levels of senescence markers such as p16, p21, and p53. Additionally, senescence-associated β-galactosidase (SA-β-Gal) activity and H3K9me3 fluorescence intensity were significantly increased. In vivo, Adeno-Associated Virus (AAV)-mediated intra-articular injection of shCCNE2 in a collagen-induced arthritis (CIA) mouse model significantly reduced the arthritis index, alleviated joint inflammation, and suppressed CCNE2 expression. Furthermore, the secretion of SASP factors such as MMP-3 and IL-8 was significantly enhanced. These findings suggest that targeting CCNE2 induces senescence in RASFs and may offer a novel strategy to mitigate RA progression and inflammation.

Use of the etanercept biosimilar during pregnancy was associated with favourable outcomes, supporting its safety and utility in managing rheumatoid arthritis during pregnancy.

Rituximab (RTX) is a standard maintenance therapy for ANCA-associated vasculitis. Its efficacy in Japan remains unclear, where microscopic polyangiitis (MPA) predominates and clinical characteristics differ from Western-dominated RCT populations. Japanese patients with MPA or granulomatosis with polyangiitis (GPA) enrolled in a nationwide registry were included. Exposure was RTX use during maintenance therapy. The primary endpoint was major relapse-free survival at 104 weeks. The secondary endpoint was any relapse-free survival (major or minor) at 104 weeks. Baseline differences were adjusted using inverse probability of treatment weighting (IPTW) based on key demographic and disease-related covariates. A total of 389 patients were analyzed, with 85 in the RTX group. The RTX group included a higher proportion of GPA cases (37/85 vs. 74/304), resulting in baseline imbalance. After IPTW, no major relapses were observed in the RTX group, whereas the major relapse-free survival at 104 weeks was 94.8% in the non-RTX group. The RTX group showed significantly higher any relapse-free survival at 104 weeks (95.4% vs. 83.3%; HR for any relapse, 0.27; 95% CI, 0.09-0.74; p = 0.02). Our findings suggest that RTX may be an effective option for remission maintenance in Japanese patients with MPA or GPA.

To evaluate the long-term safety and exploratory efficacy of filgotinib, a JAK1-preferential inhibitor, in patients with idiopathic multicentric Castleman disease (iMCD) over 52 weeks in a small, single-arm extension study. This Phase Ib single-arm extension study enrolled five patients with iMCD who completed an initial 8 weeks' treatment with filgotinib. Patients received filgotinib 200 mg once daily for an additional 44 weeks (total 52 weeks). The primary endpoints assessed the comprehensive safety parameters. The secondary endpoints were efficacy. All five patients completed the full 52-week treatment period without permanent discontinuation. No serious adverse events or deaths were observed. All adverse events were mild-to-moderate (Grade 1-2), with upper respiratory infections in 4 patients and headache in 3 of 5 patients. Herpes zoster occurred in one patient. At week 52, three of five patients had ≥1-point CHAP score reduction (one additional patient compared with week 8). Median changes from baseline included: CRP -1.85 mg/dL, hemoglobin +0.50 g/dL, albumin +0.30 g/dL, and ECOG-PS 0 points. Filgotinib was well-tolerated over 52 weeks with no serious adverse events reported. Numerical changes in disease activity markers were observed over time, providing preliminary support for the therapeutic concept of JAK inhibition in iMCD.

To evaluate the relationship between serum biomarkers (VEGF, MMP-3, leptin) and disease activity and the changes in those biomarker levels during tumour necrosis factor inhibitor (TNFi) treatment in patients with axial spondyloarthritis (axSpA). Patients with axSpA initiating their first TNFi and had serum samples at baseline and the 6th month of therapy were included, and the relation between these biomarkers and disease activity, function parameters were analysed. A total of 74 axSpA patients (mean age [SD] 48.7 [12.8] years, 54.1% male) were included. Disease activity parameters (BASDAI, ASDAS, and BASFI) and CRP were significantly decreased at the 6th month of TNFi therapy (all p < 0.001). The correlation between biomarker levels and disease activity was evaluated, and only serum MMP-3 levels were found to be associated with baseline ASDAS (p = 0.02). Serum VEGF level decreased compared to the baseline at the 6th month (p < 0.001), however, serum leptin and MMP-3 levels were not affected by TNFi (p = 0.50). Serum VEGF levels were substantially correlated with gender, hip involvement, delay in diagnosis, and disease activity in the longitudinal analysis. In patients with axSpA, TNFi therapy significantly reduces serum VEGF levels and effectively reduces disease activity. VEGF has the potential to function as a dynamic biomarker that reflects the characteristics of the disease and the response to treatment.

This systematic review and meta-analysis aimed to assess the clinical outcomes of biologic therapies, which include platelet-rich plasma and cell-based therapies (e.g., adipose-derived mesenchymal stem cells), on pain, physical function, and disease progression in patients with knee osteoarthritis (OA), focusing on studies with a follow-up of at least 12 months. We searched for randomized controlled trials (RCTs) posted at some stage in January 2000-May 2025. Eligible research protected the ones in adults with Kellgren-Lawrence grades I-III OA who underwent at least 12 months of follow-up. The bias risk was assessed, and the evidence certainty was evaluated. Random-effects models were used for pooled analyses. Fourteen RCTs were included. Compared with control treatments, biologic therapies significantly reduced pain and improved physical function. Potential structural benefits, including cartilage thickness preservation and favourable biochemical changes, were noted. However, substantial heterogeneity in study design and intervention protocols, along with potential publication bias, reduced the certainty of evidence to a very low level. Biologic therapies may be associated with improvements in pain and physical function at ≥12 months of follow-up, with preliminary indications of structural benefit. Nevertheless, high-quality multicenter RCTs with extended follow-up are warranted.

To develop and validate data-driven algorithms for identifying patients with dermatomyositis (DM) and polymyositis (PM) using Japanese administrative claims data. This multicentre retrospective cross-sectional study included outpatients from six university hospitals between November and December 2023. Administrative claims data covering one year were linked with chart-confirmed diagnoses. Twenty-six candidate variables, including diagnosis codes, laboratory tests, prescriptions, and administrative billing items, were evaluated. Three feature-selection methods were applied to identify relevant predictors. Decision tree analysis was used to construct simplified rule-based algorithms, which were validated in independent internal and external cohorts. Among 8,199 training, 3,512 testing, and 1,827 external validation patients, 576 (7.0%), 244 (6.9%), and 136 (4.2%) carried diagnosis codes for DM/PM, of whom 352, 150, and 98 were confirmed as true cases, respectively. The performance of diagnosis codes alone yielded positive predictive values (PPVs) of 0.602 in the testing set and 0.713 in the external validation set. Anti-double-stranded DNA antibody testing, intractable disease management fees, and diagnosis codes for Sjögren's syndrome were identified as key discriminative variables. The optimal algorithm demonstrated sensitivity of 0.878, specificity of 0.984, PPV of 0.761, and F1-score of 0.815 in the external validation cohort, providing superior accuracy compared to the use of ICD-10 codes alone. ICD-10 codes alone were insufficient for accurate identification of DM/PM in Japanese claims data. Integrating laboratory tests and administrative information substantially improved PPV, providing a practical and generalizable framework for claims-based research in Japan.