Journal of Rheumatic Diseases最新文献

Posterior reversible encephalopathy syndrome (PRES) is a neuroradiological disorder primarily affecting the posterior circulation of the brain. It manifests with a spectrum of symptoms, typically accompanied by hypertension, headache, seizures, and blurred vision. The etiology of PRES remains uncertain, but it is believed to be associated with vasogenic edema. The underlying conditions can range from sepsis to drug-induced states, autoimmune diseases, systemic lupus erythematosus (SLE), which is the most prevalent connective tissue disease associated with PRES. SLE is a multisystemic disorder that impacts various organs in the body, with musculoskeletal and skin systems being the most affected. The morbidity and mortality associated with SLE stem from its potential involvement of life-threatening organs, including the central nervous system, kidneys, and respiratory system. This article will focus on the neurological impact of PRES, including its manifestations. The association between PRES and SLE will be discussed in detail, accompanied by a literature review that explores the variability of presentation, reversibility, and prognosis.

Objective: To examine the risk of cardiovascular disease (CVD) associated with use of tumor necrosis factor inhibitors (TNFi) in patients with ankylosing spondylitis (AS).

Methods: This study used data from the Korean National Health Insurance database. Patients aged ≥18 years who were newly diagnosed with AS between 2010 and 2018 and did not suffer prior CVD were included. The primary outcome was a composite endpoint of myocardial infarction (MI) or stroke. The secondary outcomes were specific endpoints of MI, stroke, or congestive heart failure. TNFi use was evaluated as a time-dependent variable. Cox proportional hazard regression was used to examine the association between TNFi use and risk of incident CVD.

Results: Of 19,775 patients (mean age, 36.1 years; 75% male), 5,978 (30.2%) were exposed to TNFi treatment during the study period. During a follow-up of 4.8 (interquartile range, 2.9~7.2) years, 522 events of MI or stroke occurred. TNFi use was associated with a lower risk of MI or stroke than no TNFi use after adjusting for traditional CV risk factors and medications (adjusted hazard ratio [aHR], 0.72; 95% confidence interval [CI], 0.55~0.94). The lower risk of MI or stroke associated with TNFi use was consistently observed across subgroups. Risk of congestive heart failure did not differ between users and non-users of TNFi (aHR, 0.94; 95% CI, 0.75~1.18).

Conclusion: In a real-world AS cohort, TNFi treatment was associated with a reduced risk of atherosclerotic CVD. However, TNFi use was not associated with risk of congestive heart failure.

Objective: The aim of this meta-analysis was to evaluate the association between circulating interleukin-17 (IL-17) levels and systemic sclerosis (SSc).

Methods: We conducted a comprehensive systematic search in MEDLINE, Embase, and Web of Science for relevant studies. The meta-analysis compared serum/plasma IL-17, IL-17A, IL-17B, IL-17E, and IL-17F levels in SSc patients with those in control subjects, incorporating subgroup analyses based on ethnicity and disease subtype.

Results: This analysis included 16 studies, comprising 1,229 patients diagnosed with SSc and 839 control participants. The meta-analysis did not identify a significant difference in circulating IL-17 levels between SSc patients and controls in overall group (standardized mean difference [SMD]=0.260; 95% confidence interval [CI]=-1.805 to 2.336; p=0.825). However, recent studies supported this finding, demonstrating a stronger association (SMD=2.040; 95% CI=1.049 to 3.030; p<0.001). IL-17A levels were significantly higher in SSc patients (SMD=2.046; 95% CI=0.724 to 3.369; p=0.002), along with notable increases in IL-17B (SMD=0.764; 95% CI=0.230 to 1.298; p=0.005), IL-17E (SMD=0.672; 95% CI=0.282 to 1.062; p=0.001), and IL-17F (SMD=0.402; 95% CI=0.019 to 0.784; p=0.039). However, no significant difference in IL-17A levels was observed between diffuse and limited SSc groups.

Conclusion: IL-17A, IL-17B, IL-17E, and IL-17F were significantly elevated in SSc, with IL-17A demonstrating the most pronounced association. These findings suggest that specific IL-17 subtypes, particularly IL-17A, play a role in SSc pathogenesis.

Objective: Interferon (IFN) signaling, and excessive apoptosis have a well-established role in systemic lupus erythematosus (SLE) pathogenesis. Long non-coding RNA (lncRNA) growth arrest-specific transcript 5 (GAS5) has been linked to excessive apoptosis and upregulation of IFN genes. We aimed to investigate the expression of IFN-stimulated genes in SLE patients compared to healthy controls, and to assess their association with lncRNA GAS5 and clinical characteristics of SLE.

Methods: The study included 30 SLE patients and 20 controls. IFN-stimulatory genes (interferon-induced protein 44-like [IFI44L] gene, MX dynamin like GTPase 1 [MX1], IFN-induced protein with tetratricopeptide repeats 1 [IFIT1]) and GAS5 expression levels in plasma were estimated by quantitative real-time polymerase chain reaction. IFN signature score was calculated.

Results: IFIT1 gene expression and IFN signature score were significantly higher in SLE patients compared to controls (p<0.001, p<0.001, respectively). LncRNA GAS5 positively correlated with IFIT1 expression (p<0.001) and IFN signature score (p=0.005). No significant associations were found between IFN gene expression or IFN signature score and disease activity (Systemic Lupus Erythematosus Disease Activity Index 2000, SLEDAI-2K) or organ damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, SDI). IFI44L expression was significantly higher in SLE patients with secondary antiphospholipid syndrome (p=0.040).

Conclusion: IFN signature is a potential biomarker for SLE diagnosis, but it can't be relied on for the assessment of activity or damage. The correlation between lncRNA GAS5 and IFN-related gene expression suggests a possible role for GAS5 in the IFN pathway in SLE.

Rheumatoid arthritis (RA) is a chronic, systemic autoimmune disease primarily targeting the joints, leading to progressive inflammation, joint damage, and functional impairment. The pathogenesis of RA involves a complex interplay of genetic predisposition and environmental factors, such as smoking, which together drive the dysregulated activation of both the innate and adaptive immune systems. The introduction of disease-modifying antirheumatic drugs marked a pivotal advancement in RA management, transforming the disease from a debilitating chronic condition into a treatable disorder. Ongoing progress in basic and clinical research has identified novel therapeutic targets, paving the way for precision medicine tailored to individual patient needs, with the ultimate goal of achieving long-term remission. This review outlines current treatment options and summarizes key treatment guidelines established by major rheumatology societies. Additionally, it highlights emerging therapies, including selective glucocorticoid receptor modulators, and Bruton's tyrosine kinase inhibitors, and epigenetic modulators. Finally, novel cell-based therapies, such as chimeric antigen receptor T cells and T cell engagers, which hold the potential to enable durable, drug-free remission. These current and emerging treatments will help further optimize RA management and improve patient outcomes.

Janus kinase inhibitors (JAKis) are associated with increased risk of Varicella-Zoster Virus reactivation, particularly in Asian populations. While reactivation of cytomegalovirus (CMV) is very rare in rheumatoid arthritis (RA) patients on JAKis, CMV retinitis (CMVR) is a sight-threatening infection primarily observed in immunocompromised individuals. We report the first Korean case of CMVR in a 75-year-old male with RA when taking upadacitinib. Upadacitinib was initiated due to refractory disease despite anti-tumor necrosis factor therapy, and methotrexate was tapered out over 3 months. During upadacitinib monotherapy, he developed subacute blurred vision in his right eye. Ophthalmologic examination showed mixed features suspicious of acute retinal necrosis and CMVR. CMVR was ultimately diagnosed through polymerase chain reaction testing of aqueous humor for CMV. Treatment with intravitreal ganciclovir alone led to the resolution of symptoms without major complications. A literature review identified seven reported cases of CMVR in RA patients, mostly in those treated with biologics or JAKis, with advanced age emerging as a key risk factor. Given the increasing use of JAKis, clinicians should maintain a high index of suspicion for CMVR in elderly RA patients taking JAKis when presenting with acute or subacute ocular symptoms.

Objective: We aimed to identify predictors of short- and long-term sustained remission in early rheumatoid arthritis (eRA) and very early rheumatoid arthritis (veRA), and to assess their relevance within the joint accumulation model paradigm.

Methods: This retrospective real-world cohort study included adult Colombian RA patients. Based on symptom onset, patients were classified as eRA (≤12 months) or veRA (≤3 months). Sustained remission was defined using Clinical Disease Activity Index (CDAI) thresholds maintained for ≥6 months (short-term) and ≥24 months (long-term). Predictors were identified using robust Poisson regression.

Results: In eRA, short-term remission was more likely in antinuclear antibodies (ANA)-positive patients (risk ratio [RR] 3.29, 95% confidence intervals [CI] 1.38~7.83) and less likely in those with higher baseline Health Assessment Questionnaire (HAQ) (RR 0.48, 95% CI 0.29~0.78). Long-term remission was more frequent in males (RR 2.67, 95% CI 1.11~6.46) and in patients with lower baseline Simple Disease Activity Index (SDAI) (RR 0.92, 95% CI 0.85~0.99). In veRA, short-term remission was negatively associated with anti-citrullinated protein antibodies (ACPA) (RR 0.74, 95% CI 0.58~0.93), swollen joint count (RR 0.52, 95% CI 0.27~1.00), and patient global assessment (RR 0.61, 95% CI 0.40~0.93). Long-term remission was associated with higher rheumatoid factor levels (RR 1.76, 95% CI 1.20~2.56), lower tender joint counts (RR 0.71, 95% CI 0.55~0.92), and slower joint accumulation rates (RR 0.51, 95% CI 0.29~0.90). These findings were consistent among disease-modifying anti-rheumatic drug (DMARD)-naïve patients.

Conclusion: Distinct predictors of remission in eRA and veRA underscore the need for stage-specific treatment strategies. Our findings provide real-world evidence supporting the joint accumulation model and highlight the potential to optimize outcomes through tailored time-sensitive interventions.

Axial spondyloarthritis (axSpA) is a chronic inflammatory disease with variable clinical presentation and often delayed diagnosis. The Assessment of Spondyloarthritis International Society (ASAS) recently proposed a consensus definition of early axSpA to standardize research and improve comparability across studies. This review outlines the rationale and methodology of the ASAS-SPEAR (SPondyloarthritis EARly) initiative and discusses its implications for clinical research. Despite its promise, recent studies applying the definition have shown that treatment outcomes in early axSpA, defined as ≤2 years of symptom duration, do not consistently differ from established disease. Methodological limitations and heterogeneous criteria in earlier literature underscore the need for refined, evidence-based definitions. Future prospective trials using the ASAS criteria and stratified by symptom duration and clinical features are needed to clarify the value of early intervention and its potential impact on disease progression. The ASAS definition offers a valuable research framework, though its application in clinical practice awaits further validation.

Rheumatoid arthritis (RA) is a chronic inflammatory condition that significantly impacts multiple joints, including the elbow, crucial for daily activities. Despite advancements in medical treatments like disease-modifying antirheumatic drugs that delay disease progression, surgical interventions are often necessary to improve the quality of life in RA patients. This review provides a comprehensive evaluation of the elbow, focusing on history taking, physical examination, and radiographic assessments, particularly magnetic resonance imaging findings. We detail the selection and counseling of patients, as well as various surgical options tailored to specific symptoms of RA elbow, including synovectomy for persistent pain due to synovitis, capsulotomy and osteophytectomy for limited range of motion, cubital tunnel release for ulnar neuropathy, and joint replacement or interposition arthroplasty for advanced joint destruction. Additionally, we emphasize the differentiation between RA flare and septic elbow, highlighting the importance of timely and accurate diagnosis. The review underscores the necessity of a multidisciplinary approach, integrating surgical and medical strategies to manage RA elbow effectively.