Modern Rheumatology最新文献

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.

Objectives: This systematic review evaluated the efficacy and safety of vaccination in patients with paediatric, adolescent, and transitional-age rheumatic diseases as per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement.

Methods: An independent investigator systematically searched PubMed to identify relevant studies published by September 2022. The search results were divided into vaccines or toxoids for diphtheria, pertussis, tetanus, pneumococcus, influenza virus, hepatitis A virus, hepatitis B virus, human papillomavirus, poliovirus, measles virus, mumps virus, rubella virus, varicella zoster virus, and tuberculosis.

Results: A meta-analysis was not feasible due to the lack of randomized controlled trials with standardized patient backgrounds and conditions. Non-live vaccines are generally immunogenic and safe for patients with rheumatic diseases. In contrast, live attenuated vaccines should usually be withheld in patients on immunosuppressants, corticosteroids, biologics, or Janus kinase inhibitors. However, for necessary immunizations against measles, rubella, mumps, or varicella, live attenuated vaccines may be considered for patients receiving low-dose corticosteroids, methotrexate, or tumour necrosis factor inhibitors.

Conclusions: This review highlights the significant gap in evidence for paediatric populations compared with adults, particularly concerning new biological therapies and Janus kinase inhibitors. Further evidence is needed regarding vaccination in paediatric patients with rheumatic diseases.

Objectives: Eosinophilic granulomatosis with polyangiitis (EGPA) causes complications in various organs. Cardiac involvement affects the prognosis but is difficult to detect during subclinical stages. We compared the results of cardiac magnetic resonance imaging with those of other imaging studies and biomarkers for patients with EGPA and suspected cardiac involvement.

Methods: This retrospective observational study included 29 patients diagnosed with and treated for EGPA at our hospital between 2015 and 2022. Of these patients, 16 with suspected cardiac involvement and cardiac magnetic resonance images were selected. Symptoms, autoantibodies, biomarkers, echocardiography, and other test results were compared with imaging findings.

Results: Of the 16 patients who underwent cardiac magnetic resonance imaging, eight had findings of cardiac involvement. A univariate analysis indicated significant differences in troponin I, creatine kinase-MB, and N-terminal pro-brain natriuretic peptide between patients with and without findings of cardiac involvement. A multivariate analysis identified creatine kinase-MB as an independent predictive factor for cardiac involvement.

Conclusions: Cardiac magnetic resonance imaging used to diagnose subclinical cardiac lesions possibly improved the prognosis of patients with EGPA.

Objectives: Osteonecrosis of the femoral head (ONFH) is a progressive hip disorder often misdiagnosed owing to overlapping symptoms and radiographic findings. MicroRNAs (miRNAs) have emerged as potential diagnostic biomarkers; however, their role in ONFH remains unclear. This study analysed miRNA expression in synovial fluid and hip joint tissues of patients with ONFH to identify diagnostic markers.

Methods: Synovial fluid and hip joint tissues were collected from 24 patients with ONFH and 12 patients with osteoarthritis (OA) undergoing total hip arthroplasty. MiRNA expression was assessed using microarray and validated using real-time polymerase chain reaction (PCR). Statistical analyses compared ONFH and OA groups and examined clinical factor correlations. Tissue-specific expression was evaluated in subchondral and cancellous bone, synovia, and labrum.

Results: Real-time PCR revealed significant upregulation of miR-210 (P < .01), miR-296, miR-659, and let-7c (P < .05) in ONFH synovial fluid compared to that of OA. Expression patterns were independent of clinical factors. MiR-210 was also significantly upregulated in cancellous bone of the reparative zone in patients with ONFH (P < .05).

Conclusions: MiR-210, miR-296, miR-659, and let-7c upregulation in ONFH synovial fluid independent of clinical factors indicates their potential as diagnostic markers. Localized increase in miR-210 in cancellous bone may indicate its role in ONFH pathophysiology.

Objectives: To validate the MCK model's prognostic utility in patients with idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD).

Methods: This retrospective study included 242 patients with IIM-associated ILD from the multicentre MYKO cohort. Patients were classified as anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive or antibody-negative. The MCK score was defined by the number of criteria met: C-reactive protein (CRP) ≥ 0.8 mg/dL and Krebs von den Lungen 6 (KL-6) ≥ 1000 U/ml for MDA5-positive patients; CRP ≥ 1.1 mg/dL and KL-6 ≥ 1000 U/ml for MDA5-negative patients. Outcomes included all-cause mortality, disease flares, and infections requiring hospitalisation, which were assessed via Kaplan-Meier and Cox regression analyses.

Results: Amongst MDA5-positive patients, an MCK score of 2 did not significantly predict mortality. However, in MDA5-negative patients, an MCK score of 2 was significantly associated with an increased risk of all-cause mortality, disease flares, and infections requiring hospitalisation. Subgroup analyses from 2018 onward showed similar patterns, although statistical significance was not maintained.

Conclusions: The MCK model demonstrates strong predictive performance for all-cause mortality and disease complications, particularly in patients with MDA5-negative IIM. However, its predictive utility may have declined in recent years, possibly due to advancements in treatment approaches.