JCR: Journal of Clinical Rheumatology最新文献

Objectives: Our study aimed to identify potential predictors for additional systemic involvement in patients with noninfectious uveitis, specifically focusing on their demographic, etiological, clinical, and laboratory data features from the pediatric rheumatology perspective.

Methods: Patients with noninfectious uveitis before the age of 18 years and followed up for at least 3 months in 2 tertiary centers of pediatric rheumatology and ophthalmology departments were included in the study. Demographics, etiology, clinical features, laboratory data, and treatments administered were evaluated and compared based on the etiology (idiopathic and systemic disease-related uveitis [SD-U]) and the use of biologic disease-modifying antirheumatic drugs.

Results: Of 244 patients (131 with idiopathic uveitis and 113 with SD-U), 141 (57.8%) were female. The median (min-max) age at uveitis diagnosis was 8 (1-17) years, with a median (min-max) follow-up period of 36 (3-216) months. We observed that uveitis was mostly anterior (n = 140, 57.4%), chronic (n = 122, 67.4%), and bilateral (n = 146, 59.8%). Patients with SD-U showed a higher prevalence of female predominance, younger age at diagnosis, bilateral involvement, chronic course, increased erythrocyte sedimentation rate value, and antinuclear antibody positivity compared with patients with idiopathic uveitis ( p < 0.05). Uveitis-related complications occurred in 105 (43%) patients, with the most common being posterior synechiae (n = 60, 24.6%). Ocular surgery was required for 7 patients (5.3%) in idiopathic uveitis and for 14 patients (12.4%) in SD-U group.

Conclusion: Our study demonstrated that the antinuclear antibody positivity and the high erythrocyte sedimentation rate values were identified as significant, independent predictors for SD-U in patients referred with noninfectious uveitis.

Background/objective: This study assessed the outcomes of patients with antineutrophil cytoplasm antibody-associated vasculitis glomerulonephritis (AAV-GN).

Methods: This historical cohort study included patients with AAV-GN evaluated from 2000 to 2022. The outcomes included recovery of kidney function from kidney replacement therapy, incidence of kidney relapses, and early or late progression to kidney failure. All outcomes were assessed by time-to-event analyses, and predictors were evaluated through Cox proportional hazards regression.

Results: Among 154 patients, 104 (68%) were female with a median age of 52 years (interquartile range [IQR], 38-61 years). The median creatinine and estimated glomerular filtration rate at presentation were 2.5 mg/dL (IQR, 1.8-4.5 mg/dL) and 23 mL/min per 1.73 m 2 (IQR, 12-36 mL/min per 1.73 m 2 ), respectively. Fifty patients (32%) initially required kidney replacement therapy, with 22 (44%) of them subsequently recovering kidney function. Higher serum creatinine and a lower percentage of normal glomeruli were associated with lower rates of kidney function recovery. The kidney relapse rate was 24.9% by 5 years and 31.4% by 7 years. Proteinase 3-antineutrophil cytoplasm antibody positivity, kidney function, and persistent hematuria were associated with relapses. Kidney failure rates were 19.6% by 1 year and 30.5% by 5 years. Higher serum creatinine and proteinuria and a lower percentage of normal glomeruli were associated with higher rates of early kidney failure. Kidney relapses, persistent proteinuria, and kidney function posttreatment were associated with higher rates of late kidney failure.

Conclusions: The parameters at presentation of an episode of AAV-GN (creatinine, proteinuria, percentage of normal glomeruli) associate with progression to kidney failure within the first year. However, progression to kidney failure after the first year depends on posttreatment parameters and kidney relapses.

Background: Janus kinase (JAK) inhibitors have been approved for treating psoriatic arthritis (PsA); however, the comparative efficacy of different JAK inhibitors remains unclear. This study aimed to investigate the comparative efficacy and safety of different JAK inhibitors in treating PsA.

Methods: This network meta-analysis was conducted in strict accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Network Meta-Analyses and Cochrane methods.

Results: Five studies involving 2757 patients were included. Pairwise meta-analysis revealed that JAK inhibitors significantly increased the American College of Rheumatology 20 score and Psoriasis Area and Severity Index 75 responses, which were confirmed by the network meta-analysis. The network meta-analysis further suggested that filgotinib 200 mg once daily (OD) (odds ratio [OR] = 3.17, 95% credible interval [CrI] = 1.07-9.88) and upadacitinib 30 mg OD (OR = 2.34, 95% CrI = 1.13-4.78) had higher American College of Rheumatology 20 score responses compared with tofacitinib 5 mg twice a day. However, upadacitinib 30 mg OD was associated with a higher risk of adverse events (placebo: OR = 1.80, 95% CrI = 1.14-2.87) and serious adverse events compared with filgotinib 200 mg OD (OR = 0.05, 95% CrI = 0.00-0.82). Upadacitinib 15 mg OD, the currently recommended therapy, is comparable in both efficacy and safety to other treatment regimens.

Conclusions: Filgotinib 200 mg OD is the safest and most effective JAK inhibitor for PsA, followed by upadacitinib 30 mg OD. However, upadacitinib 30 mg OD carries the highest risk of adverse events. Upadacitinib 15 mg OD, the currently recommended therapy, is not superior in efficacy and safety compared with other treatment options. More high-quality studies are needed to confirm these findings due to the limited number of included studies.

Objective: The prevalence of juvenile-onset systemic lupus erythematosus (JSLE) differs by race/ethnicity with environmental, genetic, and social factors implicated in disease severity and outcomes. Yet, the role of social determinants of health (SDoH) in disease presentation is not well understood. We hypothesized that in an urban center with a large, diverse catchment area, SDoH influence the severity of JSLE at diagnosis.

Methods: We completed an institutional review board-approved medical record review of children newly diagnosed with JSLE between January 1, 2018, and May 31, 2022, at Texas Children's Hospital in Houston, TX. We collected demographic data, clinical severity measures, and SDoH variables such as Area Deprivation Index (ADI), insurance status, pollution burden, and food accessibility. Statistical analysis to compare SDoH with JSLE severity included Kruskal-Wallis test, Fisher exact test, and univariable and multivariable regression.

Results: Mean diagnosis age for 136 patients was 13.4 years, with 82.4% female, 52.9% Hispanic, and 25.7% non-Hispanic (NH) Black. One-third of patients did not have a documented primary care provider, and one-third preferred non-English language. We found NH Black patients had worse clinical severity measures, with highest Systemic Lupus Erythematosus Disease Activity Index and more central nervous system involvement and cyclophosphamide therapy. Uninsured and publicly insured patients were more likely to use inpatient resources at diagnosis and live in neighborhoods with higher pollution levels and higher ADI. Hispanic patients reside in communities with higher ADI scores and limited access to supermarkets.

Conclusion: In children with JSLE from a large urban catchment area, we observed significant association of nonmodifiable (race/ethnicity) and modifiable (insurance status, access to care, food accessibility) factors on disease severity at presentation.

Background: Axial spondyloarthritis (axSpA) is a chronic inflammatory disease primarily affecting the spine and sacroiliac joints, with anterior uveitis (AU) as a common extra-articular manifestation. Predicting AU onset in axSpA patients is challenging, as traditional statistical methods often fail to capture the disease's complexity.

Methods: This study aimed to develop an interpretable machine learning (ML) model to predict AU onset in axSpA patients through a historical cohort analysis of 1508 patients from a tertiary medical center. Clinical data involving 54 variables were preprocessed through imputation, factorization, oversampling, outlier capping, and standardization. Recursive feature elimination identified 12 key predictors. Subsequently, 10 ML algorithms were assessed using performance metrics and visualization techniques.

Results: The gradient boosting machine model incorporating 12 key factors showed high accuracy in predicting AU risk. Shapley additive explanations analysis revealed that hip involvement, nonsteroidal anti-inflammatory drug use, and smoking were the most influential predictors. The model's interpretability provided clear insights into the contribution of each feature to AU risk, supporting early diagnosis and personalized treatment.

Conclusion: The gradient boosting machine model predicts AU risk in axSpA patients, helping identify high-risk cases for early intervention and personalized treatment to prevent complications such as vision loss.

Objective: In the previous Spondyloarthritis EYE study, we confirmed the potential of a screening strategy for early axial spondyloarthritis (axSpA) detection using acute anterior uveitis (AAU) and chronic back pain (CBP) as referral criteria. This follow-up study assessed changes in diagnostic categories (definite, suspected, and no axSpA) over 2 years and identified baseline factors predicting axSpA diagnosis at 24 months.

Methods: Patients with AAU and CBP were categorized into 3 groups: definite axSpA, suspected of axSpA, and no axSpA, based on clinical and radiographic data within 6 months after baseline. Suspected cases were monitored for 24 months, with the possibility of reclassification. A competing risk analysis was used to estimate the probability of transitioning from "suspected of axSpA" to "definite axSpA" or "no axSpA," and logistic regression analysis was employed to determine if baseline factors could predict definite axSpA at 24 months.

Results: Among 81 patients, 26 were classified as no axSpA, 36 as suspected of axSpA, and 19 as definite axSpA. At 24 months, suspected patients had an 18% probability to transition to definite axSpA (4 cases) and a 60% to no axSpA (15 cases). Significant predictors of axSpA diagnosis included the following: HLA-B27 positivity, good response to nonsteroidal anti-inflammatory drugs, inflammatory back pain, increasing C-reactive protein levels, buttock pain, and higher Bath Ankylosing Spondylitis Metrology Index scores.

Conclusions: Our screening strategy identified approximately one third of previously undiagnosed axSpA cases among patients with AAU and CBP, mostly at baseline, with few additional cases at follow-up. The predictors revealed in this study could aid physicians in estimating axSpA disease probability.

Background: In this case series, we present longitudinal imaging surveillance of 6 cases of osseous sarcoidosis, each of which was effectively treated with tumor necrosis factor (TNF) inhibition.

Methods: We identified 6 patients from Brooke Army Medical Center with osseous sarcoidosis, who were treated with TNF inhibition and followed with longitudinal imaging studies. Cases of osseous sarcoidosis were defined as having pathologic evidence of noncaseating granulomas on bone biopsy and evidence of osseous lesions on imaging attributable to sarcoidosis by the radiologist, treating clinician, and reviewer. Clinical data were obtained through review of the military electronic medical record.

Results: Longitudinal imaging with positron emission tomography/computed tomography, magnetic resonance imaging, and bone scintigraphy assisted in the identification of active disease and clinical remission. Imaging progression of asymptomatic lesions was associated with the eventual development of bone pain 1 to 3 years later. Clinical remission was achieved in all six cases of osseous sarcoidosis and effective doses for TNF inhibition were adalimumab 40 mg subcutaneously every 1 to 2 weeks and infliximab 5 mg/kg every 6 to 8 weeks. Time to complete imaging response ranged from 3 to 8 months.

Conclusions: Longitudinal imaging with bone scintigraphy, positron emission tomography/computed tomography, and magnetic resonance imaging demonstrated several benefits including evaluation for occult disease, surveillance of asymptomatic lesions, and evaluation of treatment response. TNF inhibition with adalimumab or infliximab was successful in all cases, and complete resolution of osseous lesions was demonstrated in 5 of 6 patients. Discontinuation of TNF inhibition led to disease recurrence in 2 cases, which prompted the use of long-term immunosuppressive therapy in all treated patients.

Background: Project ECHO (Extension for Community Healthcare Outcomes) links experts with community providers through video teleconferences that include both didactics and case discussions. We piloted the first ECHO with a specific focus on reproductive rheumatology intended to increase rheumatologists' knowledge and self-efficacy in providing reproductive health care.

Methods: The Project ECHO guides informed ReproRheum ECHO curriculum development, provider recruitment, logistics, and assessment. Assessments included interviews and pre/post surveys to assess knowledge, self-efficacy, and identify program strengths and weaknesses.

Results: Eight rheumatology providers (5 physicians, 2 nurse practitioners, 1 rheumatology fellow) and 4 experts (2 reproductive rheumatologists, 2 maternal-fetal medicine physicians) participated in six 1-hour ReproRheum ECHO sessions from January to March 2023. All but one provider attended all sessions, demonstrating feasibility. Knowledge of the rate of birth defects after exposure to both azathioprine and mycophenolate significantly increased in participating physicians. Provider self-efficacy also increased significantly (6.8 ± 1.2 pre-ECHO to 8.1 ± 0.5 post-ECHO, p = 0.03). All participants "agreed" or "strongly agreed" that they had increased confidence in their ability to answer colleagues' questions and guide patients' choices in contraception and medication in pregnancy. In interviews, providers reported satisfaction with and appreciation of both didactic and case discussions, the multidisciplinary approach, and connecting with other providers. They reported improved comfort and increased frequency of discussing reproductive health in practice.

Conclusions: The pilot ReproRheum ECHO was feasible and improved knowledge and self-efficacy among rheumatologists in reproductive health. This model is a promising approach to improving reproductive health care for women with rheumatic disease.

Background: Idiopathic inflammatory myopathies (IIMs) are rare autoimmune diseases with limited epidemiological data from Latin America.

Objective: To characterize IIMs through incident rate patterns and clinical features in a major Chilean referral center over a 10-year period.

Methods: Historical cohort study (2012-2021) reviewing clinical records from rheumatology outpatient clinic of patients with IIM diagnosis. Incident rates were calculated as IIM cases per specialty consultations. Clinical characteristics, antibody profiles, and treatment outcomes were analyzed. Both consultation-based and population-based estimates for incidence and prevalence were determined.

Results: Among 3,594,047 specialty consultations, 100 IIM cases were identified (2.78 cases per 100,000 consultations; 95% confidence interval, 2.27-3.39). Mean annual incidence was 0.58 cases per 100,000 adults (95% confidence interval, 0.47-0.69), with 2021 prevalence ranging from 5.07 to 8.57 per 100,000 adults, depending on the denominator population. Dermatomyositis was the most frequent subtype (71%).

Conclusions: This first consultation-based analysis of IIMs in Chile provides baseline data for health care resource utilization. The methodology offers a practical approach for rare disease epidemiology in similar health care settings, whereas the findings align with international reports.