JCR: Journal of Clinical Rheumatology最新文献

Abstract: Systemic lupus erythematosus is considered a prototype of human autoimmune disease based on the appearance of multiple autoantibodies, some of which can have a direct pathogenic effect on tissues. Most therapeutic modalities aim to check the enhanced humoral responses by targeting T and B cells with conventional or biologic drugs. However, in some cases, the clinical response is limited and frequently takes a high toll of toxicity in patients. The last 2 decades have brought up novel discoveries showing profound disturbances of innate immune cell function in systemic lupus erythematosus, including dysregulated NETosis, increased apoptosis, type 1 interferon, and granulopoiesis signatures that are grounded in basic cell biology abnormalities, including response to excessive oxidative stress, mitochondrial dysfunction, and upregulation of the cGAS-STING pathway. Whether the prominent autoimmunity component of lupus patients is sufficient to drive this chronic disease or follows a breakdown of innate immune homeostasis in response to the environmental factors triggering disease is the subject of this revision.

Background/objective: Gout is the most common inflammatory arthritis, and its morbidity can be substantially reduced through urate-lowering therapy. However, adherence to allopurinol-the most common urate-lowering therapy-is notoriously poor. Prior studies have not fully elucidated factors associated with allopurinol adherence, particularly psychosocial factors.

Methods: We used 2018-2021 data from the Medical Expenditure Panel Survey, a national longitudinal survey on health care expenditures and utilization. We calculated the medication possession ratio (MPR) for allopurinol for participants with gout and categorized each as follows: no allopurinol fills, low adherence (MPR ≤0.8), or high adherence (MPR >0.8) to allopurinol. We used multivariable logistic regression to identify factors associated with high adherence, using person-year as the unit of measure and accounting for clustering for participants who contributed more than 1 person-year.

Results: The analyses included 919 respondents (1453 person-years), representing a weighted total of 15,084,439 person-years. Across all years, 27.4% had no allopurinol fills, 37.4% had low adherence, and 35.2% had high adherence. In multivariable models for high adherence, Black race (odds ratio, 0.49; 95% confidence interval, 0.33-0.73, compared with White) and residence in the South US region (odds ratio, 0.54; 95% confidence interval, 0.35-0.82, compared with Northeast) were negatively associated with high adherence.

Conclusions: Black race and residing in the Southern US were associated with lower allopurinol adherence among gout patients. Interventions to improve adherence, particularly among Black patients in the South, are needed to maximize the potential benefits of allopurinol.

Objective: To evaluate the impact of the different types of neoplasms and lineages on Sjögren syndrome (SjS) patient mortality.

Methods: Medical records review study based on the Spanish Hospital Discharge Database and the International Classification of Diseases, Tenth Revision, Clinical Modification coding list. The neoplasm-related deaths in SjS patients with the general population during the period 2016-2019 were compared. A binary logistic regression analysis considering age, sex, tobacco use, and alcohol use was performed to determine the impact of SjS on the risk of dying from each neoplasm group and lineage.

Results: In the period studied, 705,557 in-hospital deaths were certified in Spain, 139,531 (19.8%) from neoplasms. Neoplasms surpassed SjS activity as a cause of mortality in primary SjS patients (11.3% vs. 1.6%, p < 0.001). SjS patients presented higher mortality rates from small bowel carcinoma (0.3% vs. 1.8%; odds ratio [OR], 5.41; 95% confidence interval [CI], 1.33-22) and gynecological neoplasms (6.4% vs. 3%; OR, 2.13; 95% CI, 1.01-4.58), related to ovarian carcinomas (4.6% vs. 1.3%; OR, 3.65; 95% CI, 1.48-8.97), than the general population. Hematological neoplasm-related deaths were more prevalent in SjS patients than in the non-SjS population (18.3% vs. 8.9%; OR, 2.04; 95% CI, 1.25-3.31), mostly attributable to the higher proportion of deaths from B-cell non-Hodgkin lymphoma (8.3% vs. 2.5%; OR, 3.04; 95% CI, 1.54-6.03) and mucosa-associated lymphoid tissue lymphoma (1.8% vs. 0.1%; OR, 70.17; 95% CI, 16.61-296.36).

Conclusion: SjS patients face an elevated risk of mortality from small bowel neoplasms, ovarian carcinomas, and B-cell and mucosa-associated lymphoid tissue lymphoma compared with the general Spanish population. Apart from developing approaches to mitigate their occurrence, it is crucial to explore thoroughly and consider the implementation of targeted early-detection programs for these specific conditions.

Objective: The aim of this study was to evaluate and validate the accuracy and performance characteristics of administrative codes in diagnosing autoinflammatory syndromes (AISs).

Methods: We identified potential AIS patients from the electronic medical records at the University of Iowa Hospital and Clinics and the Stead Family Children's Hospital using a screening filter based on the 10th edition of the International Classification of Diseases ( ICD-10 ) codes and interleukin-1 antagonists. Diagnostic criteria for adult-onset Still disease, systemic juvenile idiopathic arthritis, Behçet disease (BD), familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and chronic nonbacterial osteomyelitis (SAPHO-CNO) were reviewed for each patient. Patients who did not meet the diagnostic criteria were categorized as non-AIS. In this cross-sectional study, we calculated the sensitivity, specificity, positive predictive value, negative predictive value, and area under the receiver operating characteristic curve for the ICD codes in diagnosing AIS.

Results: Out of the 502 patients with potential AIS, 338 patients (67%) had a true AIS diagnosis. Sensitivity ranged from 80% (SAPHO-CNO) to 100% (BD and FMF), and positive predictive value ranged from 15% (FMF) to 80% (SAPHO-CNO). Specificity ranged from 81% (FMF) to 99% (CAPS and SAPHO-CNO), whereas negative predictive value ranged from 98% (adult-onset Still disease) to 100% (systemic juvenile idiopathic arthritis, BD, FMF, and CAPS). All ICD codes or code combinations for the diagnosis of specific AIS subtypes showed high accuracy with areas under the receiver operating characteristic curve ≥0.89.

Conclusions: This study validated the accuracy of administrative codes for diagnosing AIS, supporting their use in constructing AIS cohorts for clinical outcomes research.

Objective: This systematic review and meta-analysis assess the efficacy and safety of rituximab (RTX) in treating idiopathic inflammatory myositis (IIM).

Methods: PubMed and Embase were systematically searched for trials and observational studies involving RTX use in IIM. Data were analyzed using a random-effects model to generate pooled estimates for overall response, complete remission, partial response, and adverse events, with subgroup analyses by myositis type and RTX dosage (PROSPERO registered number CRD42022353740). Risk of bias assessments were done using the Newcastle-Ottawa Scale for observational studies and risk of bias 1 tool for trials.

Results: Seventeen studies (1 randomized controlled trial and 16 observational studies), encompassing 362 patients, were included. The overall pooled response rate was 70% (95% confidence interval [CI]: 57%-82%; I2 = 74%, p < 0.001). Complete remission occurred in 13% (95% CI: 3%-25%; I2 = 79%, p < 0.001) and partial response in 48% (95% CI: 30%-67%; I2 = 87%, p < 0.001), both with significant heterogeneity. Subgroup analysis revealed high response rates across all myositis types: polymyositis 69%, dermatomyositis 67%, antisynthetase syndrome 70%, juvenile dermatomyositis 60%, and immune-mediated necrotizing myopathy 86%. Response rates were similar between RTX induction doses of 1 g IV on days 0 and 14 (68%) and 375 mg/m 2 weekly for 4 weeks (71%). Reported adverse events totaled 120, including infusion reactions (18.5%) and infections (12.4%).

Conclusions: RTX shows a favorable clinical response in IIM treatment, though response rates vary. There was a significant heterogeneity in treatment effect estimates that are based on a small number of patients. The incidence of infusion reactions and infections highlights the need for careful monitoring. Further controlled trials are essential to refine treatment protocols and evaluate long-term outcomes for RTX's role in IIM.