Rheumatology International最新文献

The ANCA-associated vasculitis (AAV) has an exceptionally high morbidity and mortality especially in patients with diffuse alveolar hemorrhage (DAH). Data on DAH in elderly AAV patients is still very limited. To investigate the impact of DAH on patient survival, relapse-free survival, death from infectious complications, and the incidence of pneumonia in one of the most vulnerable but often underrepresented AAV subpopulation-elderly patients. We included 139 AAV patients in this retrospective cohort study and performed a 5-year follow-up. AAV patients were divided into patients ≤ 65 and > 65 years ("elderly"). Elderly AAV patients were further subdivided into patients with and without DAH. Relapse-free survival was comparable (P = 0.49) whereas overall patient survival (P = 0.01) was significantly lower in patients > 65 as compared to ≤ 65 years. Death due to infectious complications occurred more frequently in the elderly cohort (log-rank P = 0.02). Especially the incidence of pneumonia (including opportunistic pathogens) was considerably higher in elderly AAV patients (log-rank P = 0.001). Overall survival in elderly patients was significantly lower in patients with as compared to patients without DAH [8/18 (44%) versus 9/52 (17%) deaths (P = 0.02)] while relapse-free survival was again comparable (P = 0.87) between both groups. Notably, 6 out of 8 fatal outcomes in elderly DAH patients were associated with severe infections. In multivariate analyses, age and glucocorticoid (GC) dose at 3 months were the only predictors of death from infectious complications, whereas this could not be independently demonstrated for DAH. Life-threatening infections with (opportunistic) pneumonia are common in elderly AAV patients with DAH during the first 12 months and higher GC dose was an independent predictor of death from infectious complications.

Sarcopenia is characterized by loss of muscle mass and reduced muscle function, presenting various adverse events, especially when inflammation is present. The present study aimed to determine the prevalence of sarcopenia and sarcopenic obesity among patients with rheumatic and musculoskeletal diseases (RMDs) and identify the risk for sarcopenia using two screening tools the Strength, assistance with walking, rising from a chair, climbing stairs, and falls (SARC-F) and the Mini Sarcopenia Risk Assessment (MSRA). In this single-center cross-sectional study, 220 consecutive patients visiting the Department of Rheumatology and Clinical Immunology at the University General Hospital of Larissa were interviewed. The EWGSOP criteria were used for the diagnosis of sarcopenia, while sensitivity, specificity, positive predictive values, number needed to screen, and positive and negative likelihood ratios were used to validate the diagnostic validity of the SARC-F and the MSRA. Univariate and multivariate logistic regression analyses were also applied to model the relationship between sarcopenia and other variables. In the total sample, 15.9% of patients were diagnosed with sarcopenia and one patient with sarcopenic obesity. The SARC-F (sensitivity 22.2%, specificity 75.6%), the 5-item (sensitivity 88.9%, specificity 18.9%), and the 7-item MSRA (sensitivity 91.7%, specificity 9.2%) presented poor clinical performance when used for screening alone. Univariate logistic regression analyses showed that underweight status, systemic sclerosis and appetite loss are strong contributors to sarcopenia diagnosis. Sarcopenia is prevalent among RMDs, and screening is essential within RMD clinics. None of the screening tools (SARC-F and MSRA) can stand alone in assessing sarcopenia in patients with RMDs. More research is required to understand sarcopenia in RMDs and validate the wide-using screening tools.

This study investigates the thickness of retinal structures in patients with neurosarcoidosis (NS) and ocular sarcoidosis (OS). We compared the central macular thickness (CMT), retinal thickness (RT), central nerve fiber layer (RNFL) thickness, and ganglion cell layer (GCL) thickness using optical coherence tomography. In a cross-sectional study, we categorized 97 sarcoidosis patients (185 eyes) into four groups: patients without ocular or central nervous system sarcoidosis (Non-Ocular/Non-CNS, n = 53), patients with OS (Ocular, n = 13), patients with NS (CNS, n = 16), and patients with combined OS and NS (Ocular/CNS, n = 15). The mean age was 51 (14) years. We found no overall difference between the groups in the CMT (p = 0.3), RT (p = 0.9), RNFL (p = 0.3), and GCL measurements (p = 0.9). Only in patients with a disease duration of more than five years, the CMT was significantly thicker in the Ocular group (278 μm, p < 0.001), the CNS group (267 μm, p = 0.04), and the Ocular/CNS group (268 μm, p = 0.04), compared to the Non-Ocular/Non-CNS group (249 μm). The RT was significantly thicker in the Ocular group (296 μm, p = 0.008) and the Ocular/CNS group (291 μm, p = 0.03) compared to the Non-Ocular/Non-CNS group (283 μm). In the RNFL measurements, the Ocular group (33.7 μm, p = 0.002) was thicker than the Non-ocular/Non-CNS group (29.1 μm). We found an increased retinal thickness in patients with ocular sarcoidosis and long disease duration.

A significant proportion of patients with discoid lupus erythematosus (DLE) experience joint pain, yet its underlying pathomechanism remains unclear. Recent ultrasound studies in systemic lupus erythematosus (SLE) patients indicate synovitis in up to 90%, even in clinically silent cases, with interferon-mediated immune responses implicated in joint inflammation. This study aimed to investigate joint pathology in DLE, focusing on synovitis and its immunological profile. We analyzed 23 patients with histologically confirmed DLE and joint pain, all treated for ≥ 5 months with prednisone (≤ 10 mg/day) and hydroxychloroquine (HCQ, 200 mg/day). Ultrasonographic assessment (24 joints per patient) was performed using power Doppler (PD) ultrasonography, with synovitis graded using the OMERACT-EULAR PDUS synovitis score. Serum levels of 37 cytokines were measured via Bio-Plex Pro™ Human Inflammation Panel (37Plex). Synovitis (OMERACT-EULAR score ≥ 1) was identified in 30% (7/23) of DLE patients, with minimal (grade 1) synovitis in six cases and moderate (grade 2) synovitis in one. Patients with synovitis had significantly higher levels of IFN-α2, IFN-γ, MMP-1, MMP-3, sTNF-R1, and sTNF-R2 (p < 0.05), more painful joints, and poorer response to HCQ treatment (71.4% vs. 25% non-responders). Joint pain in DLE may result from synovitis, with an interferon-mediated immune response contributing to inflammation. Patients with synovitis exhibited elevated interferon levels and a worse response to HCQ therapy. These findings suggest a shared pathogenic mechanism between DLE and SLE-related arthritis, warranting further investigation into targeted therapeutic strategies.

Objective: Benefits of physical activity (PA) on sleep in people with axial SpondyloArthritis (axSpA) are largely unknown. Our aim is to explore the relationships between PA and sleep on both a group level and an individual level using Wearable Activity Trackers (WATs) and machine learning.

Methods: A sample of 64 axSpA participants received a WAT to monitor their PA and sleep. Participants with more than 30 days data of PA and sleep duration were included in the analyses. Spearman's correlation and the machine learning technique Subgroup Discovery were used to determine relationships between PA during the three prior days and light and deep sleep duration.

Results: Number of daily steps (n = 64) was (median (first quartile (Q1) - third quartile (Q3) )) 4026 (1915 - 6549), total sleep (daily light and deep sleep) duration of the participants was 7 h 29 min (6 h 41 min - 8 h 8 min). Nearly 30% (n = 18) of the participants were eligible for inclusion in analyses (> 30 days of data). No significant relationships between prior PA and sleep were obtained on a group level. On an individual level, for 8 of the 18 included participants, significant relationships (p < 0.05) could be identified between PA during the three prior days and daily sleep duration. These significant relationships differed from participant to participant with a varying qualification of PA (number of steps, intensity level PA) and relevant time window (previous one, two or three days).

Conclusion: Significant relationships between PA and daily sleep duration could be obtained on an individual level with details of the significant relationships varying between participants.

Registration number: Netherlands Trial Register NL8238, included in the International Clinical Trial Registry Platform (ICTRP) ( https://trialsearch.who.int/Trial2.aspx?TrialID=NL8238 ).

Objectives: Decreased quality of life (QoL) is a significant complication of most rheumatic conditions in adults, whereas data on health-related quality of life (HRQoL) in children with juvenile idiopathic arthritis (JIA) are limited. Patient-Reported Outcomes Measurement Information System^® (PROMIS^® ) instruments assessing quality of life (QoL) components can be scored using disease-specific severity thresholds. Our study aimed to compare the results of generic and one of the JIA-specific scorings regarding PROMIS^® Pediatric Mobility, Pain Interference, and Fatigue questionnaires, introduce PROMIS^® Pediatric Global Health 7 questionnaire as a valuable method to assess QoL specifically in patients with JIA and determine differences in self-reported QoL in juvenile idiopathic arthritis (JIA) patients and their healthy peers in the Polish population.

Methods: In this single-center cross-sectional study, four self-reported questionnaires derived from the PROMIS^® Pediatric Item Bank (Global Health, Pain Interference, Fatigue, Mobility) were administered to 52 patients with JIA (8-17 years; mean 13.2 ± 2.9; girls 59.6%) and 101 age-matched healthy controls. Questionnaires were scored based on generic or JIA-specific cut-off points.

Results: Regardless of the domain (PROMIS^® Pain Interference, Fatigue, or Mobility), JIA patients assigned by generic cut-off points to a more severe outcome were classified into milder outcomes when cut-off points for JIA were used. The usefulness of the PROMIS^® Pediatric Global Health 7 questionnaire was evidenced in children with JIA. No significant differences were found in QoL assessment between children with JIA and healthy children; however, self-reported impaired mobility was more prevalent in patients with JIA. A negative correlation was found between overall QoL and the assessment of three domains: pain, fatigue, and mobility impairment.

Conclusions: Our study underscores the importance of JIA-specific scoring in clinical practice. While more research is needed to establish a single disease-specific scoring, our findings provide valuable insights into the negative influence of pain, fatigue, and mobility impairment on QoL in JIA. These results have the potential to significantly impact patient care and improve the health-related quality of life in children with JIA.

Background: Introduction of anti-TNF treatment has greatly improved prognosis of Behcet's disease (BD). Withdrawal of anti-TNF treatment in chronic inflammatory arthritis or bowel disease has been associated with sustained remission in subsets of patients. Herein, we examined the probability of very long-term quiescence after withdrawal of TNF inhibitors in BD.

Methods: Retrospective longitudinal outcome single-center study focusing on BD patients who discontinued successful ant-TNF treatment since 2001. Endpoint was their proportion with sustained clinical remission for 5 years after withdrawal.

Results: Thirty-three patients with severe BD refractory to non-biologic treatment (mean age 47.5 ± 11.5 years, 55% men) achieved TNF inhibitor-induced remission for a median of 2 years (IQR [1-2.6]). TNF inhibitors had been given for sight-threatening disease (28/33, 82%), for mucocutaneous (3/33), central nervous system (2/33) and gastrointestinal involvement (1/33). After withdrawal, BD remained in remission in 15/33 patients for 6.6 to 20.6 years (mean 13.5 ± 3.8). Conversely, 18/33 patients relapsed after a median of 9.5 months [IQR 8-12, range 4-32] following withdrawal but retreatment with TNF inhibitors was effective in 13/18. Of them, 9/13 discontinued for a second time and achieved again the study's endpoint, remaining in remission for median of 7.4 years ([IQR 5-9.3, range 5-15 years). Study's end-point was met by 24/33 patients (73%); 17and 7 patients remain any-drug free or on azathioprine only, respectively.

Conclusion: Discontinuation of successful anti-TNF treatment is frequently associated with durable very long-term remission in severe BD. Additional studies are needed since relapses not responding to anti-TNF re-treatment may occur.

The systemic associations with knee osteoarthritis (KOA) are incompletely understood. This study explores aortic disease, musculoskeletal and organ findings in patients with KOA in relation to their symptoms or radiographic abnormalities. Full body computed tomography (CT) scans of 255 IMI-APPROACH participants were investigated using an automated analysis of multislice CT (Voronoi Health Analytics) that extracts aortic size and calcifications, and volumes and densities of bones, muscles, fat compartments and thoracic and abdominal organs. The CT measurements were primarily related to KOA as measured with Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), visual scores and automated knee radiograph analysis of osteophytes, bone sclerosis and joint space width. The median age was 67 years, body mass index (BMI) 26.8 kg/m² and 78% were female. About half had Kellgren-Lawrence grade ≥ 2. Larger knee osteophyte area was associated with a larger aortic volume (R_Spearman=0.21,P = 0.001), which can be due to elongation or dilatation. We observed an association between more symptoms and increased psoas (R_Spearman=-0.23,P < 0.001) and lower leg (R_Spearman=-0.23,P < 0.001) muscle density, suggesting less microscopic muscle fat. Symptomatic KOA was associated with substantially lower lung volume (771 ml difference between 50% worst and 50% best WOMAC), but not with visible lung disease. Lung volume and density were significantly associated with the physical functioning WOMAC component. These associations remained significant after adjustment for age, sex and BMI. KOA is associated with significant systemic changes, including altered aortic and organ volumes. These correlations suggest that KOA's impact may extend beyond the joints. Future research should explore the causal relationships and therapeutic implications associations.

COVID-19 was an emerging pandemic in 2020 which resulted in millions of deaths worldwide. It has been known that COVID-19 can cause secondary vasculitis. However, the impact of large vessel vasculitis, giant cell arteritis (GCA) and Takayasu (TAK), on COVID-19 infection is not known. This retrospective analysis used data from the US National Inpatient Survey 2020. Patients of all ages hospitalized due to COVID-19 in 2020 were identified on the database. A primary diagnosis of COVID-19 and a secondary diagnosis of LVV were included. Characteristics of patients, comorbidities, and clinical outcomes were compared. The primary outcome was the mortality rate. Secondary outcomes included resource utilization and acute in-hospital complications of COVID-19 infection. Multivariate logistic regression and univariate logistic regression analyses were conducted, with P values < 0.05 considered statistically significant. A total of 675 patients hospitalized with COVID-19 had concurrent LVV. Patients with LVV were older (73.70 vs 62.61; P < 0.001) and more likely female (75.00% vs 48.20%; p < 0.001). There is no difference in in-hospital mortality of COVID patients with and without LVV (aOR 0.95, p = 0.834), GCA (aOR 0.94, p = 0.827), or TAK (aOR 2.30, p = 0.394). There is an increase in the in-hospital risk of developing acute MI in COVID patients with LVV (aOR = 1.54; p = 0.04) but not with subgroup GCA and TAK. There were no significant differences in resource utilization and other acute in-hospital complications. Hospitalized COVID-19 patients with LVV and GCA were more likely to develop acute MI than those without. Further studies are required to minimize confounders to better explore the causal relationship of COVID-19 and LVV.

The aim of this study was to evaluate deep tendon reflex responses and associated electrophysiological parameters of the muscles in patients with fibromyalgia. This cross-sectional study included 38 patients with fibromyalgia and 32 age- and sex-matched controls. Deep tendon reflexes of the rectus femoris and triceps brachii were tested using a reflex hammer. Electromyographic (amplitude and duration of activation), inertial measurement unit (angular velocity, acceleration), and electromechanical (delay) analyses were performed using a surface electromyography (sEMG) device. Comparative analyses were carried out between patients and controls. Additionally, Receiver Operating Characteristic (ROC) analysis was performed to evaluate the ability of hyperreflexia in distinguishing fibromyalgia patients from controls. Hyperactive deep tendon reflexes in the right/left rectus femoris and/or triceps brachii were observed in more than 85% (ranging from 86.8 to 94.7%) of the fibromyalgia group. Patients with fibromyalgia exhibited significantly increased deep tendon reflex responses compared to controls (p < 0.001). Patients revealed significantly higher amplitude, longer duration of muscle activation, greater sagittal acceleration and angular velocity, and shorter electromechanical delay. Normalized muscle activation (right and left rectus femoris and right triceps brachii) in response to deep tendon reflex test showed acceptable ability in differentiating fibromyalgia patients from controls (Area under curve (AUC) = 0.890, 0.784 and 0.782, respectively). For the right rectus femoris, values ≤ 28.3 (clinically corresponding to normoactive/hypoactive deep tendon reflexes) appeared to rule-out fibromyalgia with 94.1% sensitivity and 61.3% specificity. Patients with fibromyalgia exhibit increased deep tendon reflex responses. Normal or decreased deep tendon reflex responses may probably be used as a rule-out criterion for fibromyalgia.