JCR: Journal of Clinical Rheumatology最新文献

Background/objective: Inflammatory arthritis frequently affects patients with systemic sclerosis (SSc) but musculoskeletal corticosteroid (MSKC) injections are often avoided due to concerns of scleroderma renal crisis (SRC). This study investigated the incidence of SRC following MSKC injections.

Methods: In a 136-SSc cohort, 46 subjects underwent a total of 330 MSKC injections each receiving a significant dosage of triamcinolone acetonide (mean, 95.2 ± 44.2 mg per injection session). Data on blood pressure (BP), serum creatinine and glucose, urine protein, and complications were obtained before and after injection from the patients' medical records.

Results: MSKC and control subjects were similar in age (MSKC: 58.9 ± 12.1 vs. 55.5 ± 14.9 years), female (MSKC: 97.8% [45/46] vs. 89.9% [81/90]), antinuclear antibody (MSKC: 71.7% [33/46] vs. 81.1% [73/90]), anti-centromere antibody (MSKC: 47.8% [22/46] vs. 37.8% [34/90]), anti-topoisomerase antibody (MSKC: 26.1% [12/46] vs. 26.7% [24/90]), and anti-RNA polymerase III antibody (MSKC: 17.4.1% [8/46] vs. 24.4% [22/90]) (all p > 0.05). Pre- and post-MSKC demonstrated nonsignificant changes in systolic BP (pre: 127 ± 22 vs. post: 127 ± 21 mm Hg, p = 1.0), diastolic BP (pre: 71 ± 13 vs. post: 71 ± 11 mm Hg, p = 1.0), creatinine (pre: 0.78 ± 0.56 vs. post: 0.76 ± 0.20 mg/dL, p = 0.64), glucose (pre: 100 ± 21 vs. post: 99 ± 24 mg/dL, p = 0.67), and urine protein-creatinine ratio (pre: 0.14 ± 0.12 vs. post: 0.12 ± 0.11 mg/mg, p = 0.41). One case of SRC with mortality occurred in the controls and none in the MSKC group. No infections, hematologic abnormalities, or tendon rupture were noted.

Conclusion: MSKC injections in established SSc are generally safe with low incidences of SRC and complications. However, it is still prudent to monitor high-risk individuals and recent-onset SSc post-MSKC injection.

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.

Background/historical perspective: The advent of genome-wide sequencing and large-scale genetic epidemiological studies has led to numerous opportunities for the application of genetics in clinical medicine. Leveraging this information toward the formation of clinically useful tools has been an ongoing research goal in this area. A genetic risk score (GRS) is a measure that attempts to estimate the cumulative contribution of established genetic risk factors toward an outcome of interest, taking into account the cumulative risk that each of these individual genetic risk factors conveys. The purpose of this perspective is to provide a systematic framework to evaluate a GRS for clinical application.

Summary of current literature: Since the initial polygenic risk score methodology in 2007, there has been increasing GRS application across the medical literature. In rheumatology, this has included application to rheumatoid arthritis, gout, spondyloarthritis, lupus, and inflammatory arthritis.

Major conclusions: GRSs are particularly relevant to rheumatology, where common diseases have many complex genetic factors contributing to risk. Despite this, there is no widely accepted method for the critical application of a GRS, which can be a particular challenge for the clinical rheumatologist seeking to clinically apply GRSs. This review provides a framework by which the clinician may systematically evaluate a GRS.

Future research directions: As genotyping becomes more accessible and cost-effective, it will become increasingly important to recognize the clinical applicability of GRSs and identify those of the highest utility for patient care. This framework for the evaluation of a GRS will also help ensure reliability among GRS research in rheumatology, thereby helping to advance the field.

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.