Journal of Rheumatology最新文献

Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.

Results: In total, 73 patients with Still disease and cardiac involvement were assessed (mean age 36.3 [SD 19.9] years; male sex, 42.5%), out of which 21.9% were children. The most common cardiac manifestation was pericarditis, occurring in 90.4% of patients; patients also presented with myocarditis (26%), and less frequently endocarditis (2.7%) and tamponade (1.4%). In comparing clinical features of patients with myocarditis to those without, significantly increased frequencies of skin rash and pleuritis, as well as higher systemic scores, were seen. Further, a higher mortality rate was shown in patients with myocarditis. In regression models, skin rash and the systemic score independently predicted the myocarditis.

Conclusion: The characteristics of patients with Still disease and cardiac involvement were assessed in the AIDA Network. The most common feature was the pericarditis, but a more severe clinical picture was also reported in patients with myocarditis. The latter was associated with increased mortality rate and higher systemic score, identifying patients who should be carefully managed.

Objective: More than 130 susceptibility loci for rheumatoid arthritis (RA) have been identified with genome-wide association studies. To investigate the genetic predisposition of Chinese patients to anticitrullinated protein antibody (ACPA)-positive RA, we carried out an exome sequencing study.

Methods: Patients were recruited from 3 major public hospitals in Singapore: Tan Tock Seng Hospital (TTSH), Singapore General Hospital, and the National University Hospital. Controls came from an established exome collection and from the TTSH Health Control Biobank. All the participants were of Chinese descent. We performed whole-exome sequencing (WES) in 595 ACPA-positive patients with RA and 1281 controls and validated the candidate variants by genotyping 795 RA cases and 600 controls.

Results: The discovery cohort yielded 73 susceptibility single-nucleotide variants (SNVs) that reached statistical significance. In the validation study with an independent cohort, 2 SNVs remained significant: PCNXL4 (P = 1.50 × 10^-5) and DHRS7 (P = 6.02 × 10^-5). The majority of known susceptibility foci were not captured by exome sequencing.

Conclusion: In this WES study of ACPA-positive RA in Chinese patients, we discovered 2 new variants in PCNXL4 and DHRS7 associated with risk for RA.

Objective: Few data are available about the epidemiology of cutaneous lupus erythematosus (CLE) in the Southern hemisphere and in multiethnic populations. We describe the prevalence, incidence, and clinical characteristics of isolated CLE in the multiethnic population of Reunion Island, France, including patients with dark skin.

Methods: The study was performed in all public hospitals and private dermatology practices in Reunion Island. Cases were identified through informatics databases. Cases were defined as isolated CLE, meaning they did not fulfill the criteria for systemic lupus erythematosus (SLE). Incident cases were collected from 2008 to 2021. Prevalence was calculated on January 1, 2022. A capture-recapture analysis was performed to estimate both prevalence and incidence.

Results: A total of 268 cases of CLE were identified and 218 were incident cases. The standardized prevalence of CLE was 43 out of 100,000 persons and the average annual standardized incidence was 3.1 per 100,000 person-years (PY). With a capture-recapture analysis, prevalence and annual incidence were estimated to be 99 out of 100,000 persons (95% CI 77.10-136.45) and 5.7 per 100,000 PY (95% CI 4.40-7.95), respectively. The mean age at diagnosis was 41.7 years and the ratio of female to male individuals was 4:1. Patients with dark skin had a higher rate of discoid CLE and were more likely to receive immunosuppressants. Generalized discoid CLE, panniculitis, and overlapping subtypes of CLE appeared as predictive markers of progression toward SLE.

Conclusion: The prevalence and incidence of CLE in the multiethnic population of Reunion Island seem higher than in light-skinned populations. We highlight new risk factors of evolution toward SLE that should be known by practitioners to adjust follow-up.

Objective: Some concerns remain about the safety of nintedanib in patients with rheumatoid arthritis-related interstitial lung disease (RA-ILD), such as in the presence of comorbidities or in combination with biologic, targeted synthetic, and/or conventional synthetic disease-modifying antirheumatic drugs (DMARDs). In this multicenter study, we retrospectively evaluated the safety of nintedanib in a real-world population of patients with RA-ILD from the Italian Group for the Study of Early Arthritis (GISEA) registry and the possible role of comorbidities and DMARDs on drug safety and withdrawal. Our secondary aim was to investigate the causes of nintedanib discontinuation.

Methods: Sixty-five patients treated with nintedanib in accordance with the current therapeutic indications were enrolled in the study. Nintedanib was prescribed in combination with DMARDs and/or steroids in 62 patients (95.4%).

Results: The 12-month retention rate of nintedanib was 76.7% and the drug was effective in about 80% of patients with ≥ 6 months of follow-up. Adverse events (AEs) were recorded in 36 subjects (55.3%), and these were mainly gastroenteric. Thirty-one subjects required a reduction of the nintedanib dose; among them, a transient or permanent reduction of the daily dose of nintedanib allowed the continuation of the treatment in 22, whereas 15 (23.1%) withdrew from the drug. All reductions and discontinuations were owing to treatment-related AEs. Comorbidities were significantly associated with side effects in multivariate analysis, whereas AEs due to nintedanib were the main cause of discontinuation.

Conclusion: Combination therapy with DMARDs did not reduce the safety and effectiveness of nintedanib, and AEs were the main cause of drug withdrawal or dose reduction, mainly owing to comorbidities.

Objective: Our aim was to compare the incidence of malignancy and its effect on mortality between hospitalized patients with rheumatoid arthritis (RA) and controls.

Methods: We conducted a population-level observational study of patients with RA (International Classification of Diseases, 9th revision, Clinical Modification [ICD-9-CM] code 714 and International Statistical Classification of Diseases and Related Health Problems, 10th revision, Australian Modification [ICD-10-AM] codes M05-M06) in the Hospital Morbidity Data Collection (HDMC) in Western Australia (WA) between 1985 and 2015, as well as nonexposed hospitalized controls matched on sex, age, and year of index admission. HDMC data were linked to the WA Cancer Registry and the WA Death Registry data, and cancer incidence rates (CIRs) per 1000 person-years, incidence rate ratios (IRR) with 95% CIs, and Kaplan Meier survival were estimated.

Results: Among 14,041 patients with RA (67.56% female, median age 65.1 years) and 33,785 controls (65.16% female, median age 65.3 years), preexisting cancer in patients with RA was less prevalent than in controls (7.6% vs 14.2%; P < 0.01). In participants without prior cancer, the overall post index CIR was lower in those with RA (CIR 19.68 vs 24.77; IRR 0.79, 95% CI 0.76-0.83) and stable over 3 study decades. CIR was higher in patients with RA for lung (CIR 1.17, 95% CI 1.04-1.34) and hematological cancer (CIR 1.21, 95% CI 1.03-1.43) but lower for most other cancer types. Overall median survival was lower for patients with RA than controls (3.3 vs 5.3 years; P < 0.001) with increased mortality rates observed for most cancer subtypes.

Conclusion: Overall CIR in patients with RA was consistently lower over time than in matched controls. CIR was only increased for lung and hematological cancer. Despite the overall lower CIR, post cancer mortality was higher for patients with RA in most cancer subtypes.

Objective: In patients with systemic sclerosis (SSc), fatigue is the highest-ranked symptom affecting quality of life (QOL), followed by Raynaud phenomenon (RP). We report results from a pilot study of the Apollo wearable device in patients with SSc.

Methods: Twenty-five adult participants with SSc, moderate fatigue, and RP were enrolled. Participants completed a 4-week intervention, during which they wore the Apollo device daily for a minimum of 15 minutes. The primary outcome was change on the Patient Reported Outcomes Measurement Information System Fatigue 13a (PROMIS Fatigue) at 4 weeks.

Results: After 4 weeks of using the Apollo wearable, participants reported less fatigue on the PROMIS Fatigue (P < 0.001) scale. The average daily number of RP attacks declined (P = 0.007), as did the Raynaud Condition Score (P = 0.007) after 4 weeks of use. Average device usage (2.87 hours/day) far exceeded the requested time, and no adverse events occurred. The PROMIS-29 subscores assessment demonstrated QOL improvement in physical function (P = 0.01), depression (P = 0.03), fatigue (P = 0.01), sleep disturbance (P = 0.002), and ability to participate in social roles and activities (P < 0.001). Significant improvements were also noted for depression (P = 0.004) and disability (P < 0.05) measures.

Conclusion: Use of the Apollo wearable for 4 weeks was associated with improvement in fatigue and RP symptoms in patients with SSc, with improved QOL measures, lower depression scores, and improved disability measures. Future studies should further test the efficacy of the Apollo wearable in these domains and QOL of patients with SSc. (ClinicalTrials.gov: NCT04854850).

Objective: This prospective study investigates the efficacy of biologics in combination with methotrexate (MTX) or leflunomide (LEF) on juvenile idiopathic arthritis (JIA)-related temporomandibular joint (TMJ) arthritis measured by magnetic resonance imaging (MRI)-based inflammation score and deformity score.

Methods: A prospective, single-center observational cohort study of 18 consecutive patients was performed between September 2018 and April 2023. Inclusion criteria were (1) diagnosis of JIA, (2) MRI-verified TMJ arthritis leading to treatment with tumor necrosis factor inhibitor (TNFi), (3) MRI at 6 months and 24 months after treatment initiation, and (4) clinical follow-up together with an MRI by a pediatric rheumatologist and an orthodontist.

Results: We included 18 patients (89% female). At the time of the first MRI, median age was 13.2 years (IQR 9.9-17.4), median disease duration was 7.8 years (IQR 3.4-11.1), and 4 received MTX or LEF. During the observation period, significant improvements were observed in TMJ movement pain (P = 0.01), morning stiffness (P = 0.004), opening capacity (P = 0.03), and maximal incisal opening P = 0.006). The inflammation score decreased significantly from a median of 2 (IQR 1-3) at baseline to a median of 1 (IQR 0-2) at 24 months (P = 0.009). In 17 of 36 TMJs (47%), the deformity score improved or remained stable and no significant increase in the median score was observed.

Conclusion: This is the first prospective, observational study with evidence to support that the orofacial signs, symptoms, and MRI-derived inflammation score in TMJ arthritis can be reduced by treatment with TNFi.

Objective: Pneumocystis jirovecii pneumonia (PJP) is an opportunistic infection that may affect patients with systemic lupus erythematosus (SLE). The objective of this project was to describe the incidence of PJP among patients with SLE.

Methods: A retrospective cohort analysis of the TriNetX database was conducted. Included patients had ≥ 2 International Classification of Diseases, 9th or 10th revision, Clinical Modification (ICD-9-CM/ICD-10-CM) codes for SLE separated by at least 30 days and were new users of mycophenolate mofetil (MMF) and/or cyclophosphamide (CYC). The incidence of PJP over the first 6 months of therapy was calculated; adverse events were assessed using incidence rate ratios (IRR) and Cox proportional hazards regressions.

Results: A total of 6017 patients with SLE were identified. Most were female (n = 5176, 86%) and Black or African American (n = 2138, 35.5%). Induction medications included MMF (n = 5208, 86.6%), CYC (n = 505, 8.4%), or both (n = 304, 5.1%); the most common PJP prophylaxis was trimethoprim-sulfamethoxazole (n = 1126, 18.7%). Five PJP cases were identified over 2752 person-years (PYs), one of whom received PJP prophylaxis, for an incidence rate of 1.8 cases/1000 PYs. In the adjusted analysis, patients who received prophylaxis had a higher risk of neutropenia (hazard ratio [HR] 2.5, 95% CI 1.4-4.4), leukopenia (HR 1.9, 95% CI 1.3-2.8), nephropathy (HR 1.7, 95% CI 1.4-2.1), and hyperkalemia (HR 1.4, 95% CI 0.9-2.0).

Conclusion: PJP rarely affects patients with SLE undergoing therapy with MMF and/or CYC; prophylaxis against PJP is associated with adverse events. The majority of patients with SLE and PJP had structural lung disease. These data do not support universal prescribing of PJP prophylaxis for patients with SLE without lung disease.

Objective: Patients with inflammatory articular diseases, such as psoriatic arthritis (PsA), report weather changes in their symptoms. Our objective was to investigate the correlation between weather variation, disease activity (DA), and patient-reported outcomes (PROs) in patients with PsA.

Methods: Hourly measurements of temperature, relative humidity, and pressure were obtained from 2015 to 2020 in Montreal (through Environment Canada) and were matched with DA and PROs of patients with PsA enrolled in Rhumadata. The differences in mean DA and PROs were examined between winter and summer. Pearson correlation coefficients were calculated between clinical profile and weather measurements.

Results: Among patients with PsA, 2665 PROs were collected for a total of 858 patients. The Clinical Disease Activity Index (P = 0.001) and Simplified Disease Activity Index (P < 0.001) were lower in winter. In summer, positive correlations were found between humidity and symptoms (using patient global assessment, fatigue, pain, C-reactive protein, Bath Ankylosing Spondylitis Disease Activity Index, Bath Ankylosing Spondylitis Functional Index), whereas negative correlations between temperature and Health Assessment Questionnaire-Disability Index were reported. In winter, positive correlations were observed between temperature, fatigue, and pain.

Conclusion: This is the first study to investigate weather variations through subjective and objective PROs matched with patients with PsA. Statistically significant differences in clinical profile were evident between winter and summer, as well as in their correlation with weather measurements. However, these distinctions lack clinical significance, suggesting a small impact on patients with PsA.