Modern Rheumatology最新文献

Objectives: To develop and validate enzyme-linked immunosorbent assay (ELISA) systems for the detection of autoantibodies against signal recognition particle (SRP), nuclear matrix protein 2 (NXP2), and small ubiquitin-like modifier activating enzyme (SAE).

Methods: Serum samples from 288 individuals were analysed, including 183 patients with idiopathic inflammatory myopathies (IIM), 20 with other neuromuscular diseases, 35 with non-IIM systemic autoimmune rheumatic diseases, and 50 healthy controls. ELISA systems were established using recombinant SRP, NXP2, and SAE proteins expressed in insect cells or Escherichia coli. The diagnostic performance of the ELISAs was assessed in comparison with the 'gold-standard' immunoprecipitation (IP) assays.

Results: The ELISAs demonstrated high concordance with IP assays, with positive and negative percent agreements of 97.4% and 100% for anti-SRP, 100% and 99.6% for anti-NXP2, and 100% and 99.6% for anti-SAE antibodies, respectively.

Conclusions: The newly developed ELISA systems showed excellent agreement with IP assays, supporting their applicability for routine clinical use in detecting anti-SRP, anti-NXP2, and anti-SAE autoantibodies.

Objectives: To assess treatment patterns for systemic lupus erythematosus (SLE) from 2012 to 2024 and evaluate corresponding changes in serological activity and relapse rates.

Methods: We retrospectively reviewed medical records of 1,705 patients with SLE treated at a single centre between 2012 and 2024. Temporal trends in therapeutic approaches, glucocorticoid (GC) use, and clinical outcomes were analysed.

Results: Use of GC monotherapy declined from 58.3% in 2012 to 22.4% in 2024. Combination therapies (GC and hydroxychloroquine [HCQ], with or without immunosuppressants) increased from 1.0% in 2015 to 41.6% in 2024, while biologics use rose from 0.9% in 2018 to 12.5% in 2024. Quadruple therapy (GC, HCQ, immunosuppressants, and biologics) also expanded from 0.4% in 2018 to 6.2% in 2024. The mean GC dose decreased from 7.7 mg/day in 2012 to 4.6 mg/day in 2024, and the median dose, stable at 5 mg/day for many years, declined to 4.6 mg in 2023 and 4.2 mg in 2024. The proportion of patients with elevated serological activity steadily decreased. Flare rates peaked at 8.1% in 2016 but stabilised at approximately 4% after 2020.

Conclusions: These findings suggest that improved disease control can increasingly be achieved in real-world practice while reducing long-term GC dependence.

Objectives: To clarify long-term prognosis and its relevant factors in patients with pure class V lupus nephritis (LN).

Methods: We reviewed consecutive patients with biopsy-proven pure class V LN from KEIO-SLE cohort. The treatment target and deep remission (DR) were defined as urine protein-to-creatinine ratio < 0.7 g/gCr and <0.15 g/gCr, respectively. Patients were divided into two groups based on the occurrence of renal flare, and baseline clinical and pathological characteristics were compared.

Results: Thirty patients with pure class V LN were included. All patients achieved the treatment target. Among them, seven patients (23.3%) experienced renal flare. In the Firth's penalized Cox regression analysis, univariate analysis showed that smoking history, glucocorticoid (GC) monotherapy throughout follow-up, anti-RNP antibody positivity, Chronicity index ≥3, and non-achievement of DR were associated with an increased risk of renal flare. Multivariable analysis revealed that only non-achievement of DR remained an independent risk factor. Kaplan-Meier analyses showed significantly lower flare-free survival in patients with these risk factors. No patients developed end-stage kidney disease during a median observation period of 85.6 months.Conclusion: Overall long-term prognosis of pure class V LN was favorable. However, approximately one-quarter of patients experienced renal flares. Failure to achieve DR, smoking history, throughout GC monotherapy, and anti-RNP antibody positivity were associated with an increased risk of renal flare, with failure to achieve DR being the only independent risk factor.

Objectives: Familial Mediterranean Fever (FMF) is characterized by recurrent fever and serositis. Although not classical in FMF, vasculitis is increasingly reported, suggesting shared pathogenic mechanisms.This study aimed to evaluate clinical features and treatment responses in pediatric FMF patients with concomitant vasculitis.

Methods: The records of 1,598 pediatric FMF patients followed at a tertiary pediatric rheumatology center were reviewed retrospectively. After exclusions, 1,481 patients were included: 1,434 without vasculitis (Group 1) and 47 with vasculitis (Group 2). Demographic, clinical, genetic, and treatment-related data were compared between the groups, and vasculitis subtypes were characterized in detail.

Results: Vasculitis was found in 3.2% of patients, most commonly IgA vasculitis and Behçet's disease Group 2 had higher attack rates, more chest pain/myalgia, and more non-exon 10 heterozygous MEFV mutations. Colchicine response and biologic therapy requirement did not differ significantly between groups.There was no statistically significant difference between groups in colchicine response (97.3% vs. 74.5%) or biologic therapy requirement (2.7% vs. 6.4%).

Conclusions: The coexistence of vasculitis in FMF likely reflects shared inflammatory mechanisms rather than coincidence. Vasculitis occurred even in patients with milder MEFV genotypes, suggesting an enhanced proinflammatory milieu. Early recognition of vasculitis in FMF may influence therapeutic strategies and improve outcomes.

Objective: To assess whether extended HLA-B typing, together with simple clinical indices, distinguishes axial spondyloarthritis (axSpA) from radiographic mimics in Japanese population.

Methods: We retrospectively reviewed 127 patients who underwent HLA-B typing at a single centre between 2008 and 2024. Ankylosing spondylitis met the 1987 modified New York criteria, and non-radiographic axSpA met the 2009 ASAS criteria. Diffuse idiopathic skeletal hyperostosis (DISH), palmoplantar pustulosis arthritis (PAO), and osteitis condensans ilii (OCI) were identified by characteristic imaging and clinical findings. Two rheumatologists reassigned final diagnoses. HLA-B phenotypes, age at onset, sex, and initial C-reactive protein (CRP) were compared across groups.

Results: A total of 25 patients satisfied axSpA criteria; 80% were HLA-B27-positive. DISH, PAO and OCI showed apparent enrichment of common Japanese alleles (B52, B61/B62, B39). DISH and PAO presented later than axSpA (54.0 y and 42.5 y versus 25.2 y) and DISH had lower CRP (0.31 versus 1.51 mg/dL, P = 0.003). OCI occurred exclusively in women and was CRP-negative.

Conclusion: In Japanese practice, no single non-B27 allele reliably separates axSpA from its mimics; instead, early age at onset, male sex, and raised CRP provide the most practical safeguards against diagnostic error and unnecessary therapy.

Objectives: Proinflammatory substances from neutrophils play an essential role in gout flare. This study aimed to compare the levels of heparin-binding protein (HBP), released by neutrophils, among patients with gout, patients with osteoarthritis (OA), patients with asymptomatic hyperuricemia (HUA), and healthy controls (HCs).

Methods: We enrolled 102 patients diagnosed with gout, 26 patients with OA, 25 patients with asymptomatic HUA, and 23 HCs. Serum HBP was measured by enzyme-linked immunosorbent assay.

Results: We found that the level of HBP was significantly elevated in patients experiencing gout flare compared with those in patients with relieving gout after taking nonsteroidal anti-inflammatory drugs (NSAIDs), patients with intercritical gout, and other control groups. Serum HBP was significantly different between patients who experienced gout relieving after NSAIDs and patients with intercritical gout. Receiver operating characteristic curve showed HBP could distinguish gout flare from relieving gout after NSAIDs. The area under the curve (AUC) was 0.8130, sensitivity was 0.9412, and specificity was 0.6286 at the cutoff HBP value of 42.09 ng/ml. The HBP cutoff for differentiating relieving gout after NSAIDs from intercritical gout was 19.75 ng/ml, which had an AUC of 0.8971, a sensitivity of 0.8485, and a specificity of 0.9118. Moreover, serum HBP levels were positive correlated with erythrocyte sedimentation rate, C-reactive protein, white blood cell, absolute neutrophil count, and neutrophil ratio.

Conclusions: Taken together, our results showed elevated HBP levels in patients with gout flare.

Systemic lupus erythematosus (SLE) is an autoimmune disease that causes organ damage and negatively affects a patient's quality of life (QoL). Despite the recent remarkable progress in treatment, patients continue to experience a substantial disease burden. In SLE, which is more common in young people, this casts a sizable shadow over patients' social activities. Treatment goals are to control disease activity, minimise treatment-related adverse events, avoid organ damage, and optimise health-related QoL. However, optimising QoL remains challenging, as many unresolved issues remain, including subjective symptoms, which are difficult for physicians to perceive. As such, physicians must work to understand this burden from the patient's perspective and pursue improved patient QoL. In this review, we discuss issues associated with disease burden and health-related QoL faced by patients with SLE in their daily lives, along with available treatments and management practices that can be implemented to optimise them. To enable SLE patients to live a normal life, we must transform current SLE care to move beyond symptom control to drive clinical remission, assessing and addressing the disease burden patients face in daily life.

Objectives: Glucocorticoid use poses perioperative concerns in orthopaedic surgery, but its impact on postoperative outcomes following total knee arthroplasty (TKA) remains unclear.

Methods: This retrospective cohort study used the Japanese Diagnosis Procedure Combination database to evaluate postoperative complications in patients undergoing TKA between April 2016 and March 2023. Patients on continuous glucocorticoid therapy (≥5 mg/day prednisolone equivalent) were matched 1:1 with non-users using propensity score matching based on age, sex, body mass index, anaesthesia type, bilateral surgery, and Charlson Comorbidity Index.

Results: After matching, 12,212 patients (6106 per group) were analyzed. The glucocorticoid group had significantly higher rates of pneumonia [odds ratio (OR): 3.70, 95% confidence interval (CI): 1.89-7.26, P = .0001] and in-hospital mortality (OR: 3.59, 95% CI: 1.47-8.73, P = .005). No significant differences were observed in venous thromboembolism or surgical site infection. Male sex was also an independent risk factor for mortality.

Conclusions: Ongoing glucocorticoid use is associated with an increased risk of postoperative pneumonia and in-hospital mortality following TKA. These findings highlight the need for careful perioperative management in this high-risk population.

Objectives: With the introduction of anifrolumab to clinical practice, there is a growing interest in identifying factors associated with interferon (IFN) activity in patients with systemic lupus erythematosus (SLE). An association between IFN-stimulated gene (ISG) expression and anti-U1-ribonucleoprotein (RNP) antibody positivity has been reported. However, data from Asians are scarce. In this study, we investigated clinical parameters associated with ISG expression in patients with SLE in Japan.

Methods: We utilized transcriptome data from 130 Japanese patients diagnosed with SLE from the ImmuNexUT database. We defined the ISG score as the average normalized expression levels of IFI27, IFI44, IFI44L, and RSAD2. The associations between clinical parameters and the ISG score were assessed.

Results: Clinical characteristics were compared between the low- and high-ISG-score groups. Clinical SLE disease activity index, anti-U1-RNP antibodies, and anti-Sjogren's syndrome antigen A (SS-A) antibodies were associated with the high-ISG-score group in multiple logistic regression analysis. Classification and regression tree analysis indicated that anti-U1-RNP antibody positivity was the best serological factor predicting the ISG score. Even among patients with clinically inactive disease, the ISG score was higher in those positive for than in those negative for anti-U1-RNP antibodies.

Conclusions: Autoantibody profiles, especially anti-U1-RNP antibodies, are useful for predicting ISG scores in Asians.