JCR: Journal of Clinical Rheumatology最新文献

Background: Musculoskeletal ultrasound (MSUS) is increasingly used to evaluate pediatric inflammatory arthritis. This study aimed to explore the relationship between MSUS findings with medication modifications in patients with juvenile idiopathic arthritis (JIA) and clinical disease activity measurements (clinical Juvenile Arthritis Disease Activity Score [cJADAS-10], active joint count [AJC], patient/parent global assessment [PPGA], and physician global assessment [PGA]).

Methods: Data from patients with JIA who underwent a 12-joint (bilateral second and third metacarpophalangeal, wrist, elbow, knee, and ankle) MSUS examination during a 57-month period were collected. Patients were categorized into 2 groups: a medication change group and a control group (patients without medication change). A pediatric-specific MSUS scoring system was used to assess MSUS findings. The association between clinical and MSUS findings was examined for the study groups.

Results: A total of 38 patients, 23 in the medication change group and 15 in the control group were included. The medication change group had higher AJC, PGA, and cJADAS-10. These patients also had a statistically significant presence of abnormal knee MSUS findings. For other joints, the frequency of abnormal MSUS findings was slightly higher in patients with a medication change, but the difference was not statistically significant. No strong correlation was observed between MSUS findings and clinical disease activity measurements.

Conclusions: Abnormal MSUS findings were not observed to be higher in patients with a change in medication except for the involvement of the knee joint. Further longitudinal studies are needed to understand the role of MSUS in the medical decision-making process in JIA.

Background: Sarcoidosis is a multisystem autoimmune disease that can result in significant morbidity and mortality. This study aims to identify factors associated with in-hospital death for sarcoid patients on a national level.

Methods: We performed a medical records review study of all adult sarcoid hospitalizations from 2016 to 2020 National Inpatient Sample database. A univariable screen followed by multivariable analysis was completed to identify predictors of in-hospital death among sarcoid patients.

Results: There were 405,650 admissions with a diagnosis of sarcoidosis, 10,210 of whom died. Multivariable analysis showed the following factors were independently associated with a higher odds of in-hospital death: age (odds ratio [OR], 1.03; 95% confidence interval [CI], 1.026-1.034), Charlson Comorbidity Index (OR, 1.09; 95% CI, 1.066-1.116), male sex (OR, 1.21; 95% CI, 1.101-1.331), other race (OR, 1.45; 95% CI, 1.073-1.954), arrhythmia/heart blocks (OR, 1.80; 95% CI, 1.617-1.995), cirrhosis/hepatic failure (OR, 8.26; 95% CI, 6.928-9.844), hemophagocytic lymphohistiocytosis (OR, 11.15; 95% CI, 4.172-29.802), infection (OR, 3.31; 95% CI, 3.007-3.633), interstitial lung disease (OR, 1.31; 95% CI, 1.193-1.438), heart failure/myocarditis (OR, 1.29; 95% CI, 1.157-1.436), neurologic diagnoses (OR, 1.37; 95% CI, 1.241-1.502), and pulmonary hypertension (OR, 1.47; 95% CI, 1.305-1.652).

Conclusions: Our multiyear national analysis showed that 2.5% of hospital admissions with a sarcoid diagnosis ended in death. The following factors were associated with death: age, Charlson Comorbidity Index, male sex, other race, arrhythmia/heart blocks, cirrhosis/hepatic failure, hemophagocytic lymphohistiocytosis, infection, interstitial lung disease, heart failure/myocarditis, neurologic diseases, and pulmonary hypertension. This information can help clinicians by improving awareness of these life-threatening complications because early recognition and intervention may improve inpatient sarcoid outcomes.

Background: Clinical features and prognosis of autoimmune inflammatory myopathies (AIMs) can vary depending on the age of disease onset. The aim of this study was to compare the demographic characteristics, clinical features, laboratory findings, and long-term prognosis of juvenile and adult AIMs.

Methods: Patients diagnosed with AIM between 2009 and 2023 in the pediatric rheumatology and rheumatology departments of our hospital were included in this medical records review study. Demographic characteristics, clinical features, laboratory findings, treatments, and prognosis of juvenile and adult AIM patients were compared with statistical methods.

Results: Of the 94 patients diagnosed with AIM, 34 (36.2%) patients were juvenile and 60 (63.8%) patients were adult. At the time of diagnosis, while Gottron papules, dysphonia, and subcutaneous edema were more common in juvenile patients, fever was more common in adult patients (p = 0.003, p = 0.05, p = 0.005 p = 0.05, respectively). During follow-up, while calcinosis was more common in juvenile patients, lung involvement and malignancy were more common in adult patients (p = 0.022, p = 0.009, p = 0.006, respectively). The methylprednisolone pulse therapy requirement was significantly higher in juvenile patients (p = 0.0001). Clinically inactive disease was more common in juvenile patients (p = 0.01).

Conclusions: AIM with different onset ages is associated with distinct clinical manifestations and outcomes. The present study reported that in AIM patients, lung involvement and malignancy increase with age while clinically inactive disease decreases.

Background: Klotho, which is known to negatively regulate metabolic disorders and kidney disease, has a role in gout that remains unclear. This research explored how klotho levels correlate with the prevalence of gout.

Methods: Participants aged 40 to 79 from the National Health and Nutrition Examination Survey (2007-2016) were examined in both lines. The connection between klotho levels and gout was analyzed through weighted multivariate logistic regression. Restricted cubic splines were used to assess linearity and investigate the dose-response relationship. To ensure the stability of the results, subgroup and sensitivity analyses were conducted.

Results: In total, 9660 individuals participated, with the weighted sample size calculated at 88,892,738.77. The group included 47.79% males (4793), with the median age being 57.00 years. Upon adjusting for all covariates, the multivariate analysis indicated an odds ratio of 0.51 (95% confidence interval [CI]: 0.33~0.78, p = 0.003) for the likelihood of occurrence of gout. When compared with the lowest klotho quartile Q1 (≥151.3, <655.3 pg/mL), the adjusted odds ratios for the subsequent quartiles Q2 (≥655.5, <800.9 pg/mL), Q3 (≥801.0, <991.6 pg/mL), and Q4 (≥991.7, ≤3998.5 pg/mL) were 0.97 (95% CI: 0.68~1.38), 0.78 (95% CI: 0.50~1.21), and 0.48 (95% CI: 0.32~0.73), respectively. Analyses focusing on subgroups and sensitivity confirmed these results.

Conclusions: This research found a negative correlation between serum α-klotho concentrations and the occurrence of gout. Those with the highest levels of klotho exhibited the lowest likelihood of gout, indicating potential importance for future studies and clinical uses.

Objectives: The aim of this study was to report the spectrum of Familial Mediterranean Fever (FMF) in children living in Southeast Michigan.

Methods: We reviewed prerecorded data in medical records of FMF patients. Statistical analysis of the data included Fisher exact test, Pearson χ 2 procedure, parametric independent samples t test, and parametric analysis of variance using SPSS Version 29.0, IBM Inc.

Results: The study included 29 males and 21 females. The mean age at presentation was 4.63 ± 3.66 years, and the mean time to diagnosis was 2.1 ± 2.18 years. A slight majority presented in the first 3 years of age (54%). Family history of FMF was reported in only 58% of patients. Clinical manifestations included fever (84%), gastrointestinal (84%), musculoskeletal (64%; including chronic arthritis, sacroiliitis, and nonbacterial osteomyelitis), chest (28%), cutaneous (14%), and other manifestations (16%). Fever without other manifestations was reported only in patients presenting at ≤3 years of age ( p = 0.016), whereas older patients reported more gastrointestinal manifestations ( p = 0.04). Reported MEFV variants included p.M694V (n = 26), p.V726A (n = 23), p.M694I (n = 13), and others (n = 10). Homozygote and compound heterozygote patients had more gastrointestinal manifestations ( p < 0.001), whereas fever was more common in the heterozygote patients ( p = 0.04). The mean follow-up period was 5.34 ± 4.13 years with no renal disease.

Conclusions: We report the largest childhood FMF cohort in the United States. A negative family history should not preclude consideration of FMF as a cause of periodic fever. Recurrent fever can be the only manifestation, particularly in young patients with FMF. The absence of fever and chronic progressive musculoskeletal manifestations can uncommonly occur.

Background/objective: Despite advances in clinical care and treatment options, adolescents with lupus continue to experience adverse health outcomes. Poor adherence to medication regimens is a major contributor to these negative outcomes. The utility of short message service (SMS) in tracking barriers to adherence prospectively remains untested for adolescents with lupus. Our objectives were (1) feasibility of incorporating a Health Insurance Portability and Accountability Act (HIPAA)-compliant SMS text messaging application into REDCap and (2) acceptability of using SMS text messaging to track barriers to medication adherence in adolescents with lupus.

Methods: This study is a 12-week pilot cohort study of adolescents with SLE per the 1997 revised American College of Rheumatology. A REDCap-embedded HIPAA-compliant text messaging application was used to send biweekly messages with survey link to track medication adherence. Measures were completed. Descriptive statistics were used to summarize demographics, medical, and acceptability data. Response to text messages and survey completion rates were reported as a measure of feasibility.

Results: Most eligible adolescents approached agreed to participate (n = 17, 71% enrollment rate). The cellphone ownership rate among adolescents eligible for participation was 92%. Nine subjects responded to all text messages sent (53% response and completion rate). Eleven subjects (65%) responded to two thirds of the text messages. Overall, 77% of enrolled subjects completed at least half of the surveys sent. Reminders to complete surveys were sent to 30% of enrolled adolescents.

Conclusions: This study shows that embedding a HIPAA-compliant SMS text message application in REDCap is feasible and can be used to engage adolescents with chronic conditions in monitoring between clinic visits.