Objective
Juvenile Sjögren's syndrome (SS) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and ...clinical findings of juvenile SS and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS.
Methods
A cross‐sectional multicenter study recruited patients with disease onset until age 18 years (n = 67). Disease characteristics were recorded, and unstimulated whole sialometry and SGUS examination of the parotid and submandibular salivary glands were performed.
Results
The female:male ratio was 58:9. The mean age at first symptom was 10.2 years and 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42 of 67 patients (63%) and 53 of 66 patients (80%), respectively. The American‐European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary SS were fulfilled by 42 of 67 patients (63%). Pathologic SGUS findings were observed in 41 of 67 patients (61%); 26 of 41 SGUS+ patients (63%) fulfilled primary SS criteria. Salivary gland enlargements/parotitis were noted in 37 of 58 patients and were nonsignificantly associated with SGUS+ status (P = 0.066). The mean levels of saliva were 5.6 ml/15 minutes in SGUS– patients compared to 3.3 ml/15 minutes in the SGUS+ patients (P = 0.049). A total of 36 of 41 SGUS+ patients (88%) were anti‐Ro/La+ compared to 14 of 26 SGUS– patients (54%) (P = 0.001). In addition, 24 of 39 SGUS+ patients (62%) were positive for rheumatoid factor (RF), whereas only 5 of 25 SGUS– patients (20%) were RF+ (P = 0.001).
Conclusion
Juvenile SS is characterized by a large spectrum of clinical symptoms and findings. Several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies are associated with pathologic SGUS findings.
To determine the usefulness of power Doppler (PD) ultrasound (US) to predict rheumatoid arthritis (RA) development in patients with clinically suspect arthralgia (CSA).
Retrospective analysis of a US ...unit cohort over a 1-year period. Patients with CSA and no previous diagnosis of inflammatory arthritis (IA) were included for analysis. All underwent bilateral US examination of the hands and/or feet according to the EULAR guidelines. Active US inflammation was defined as PD synovitis and/or tenosynovitis ≥1 at any location. RA diagnosis according to clinician criteria 6 months after the US examination was checked. Univariate and multivariate logistic regression models were employed to investigate possible predictive factors of RA development.
A total of 110 CSA patients (80 females, mean age 53.6 years) were included for analysis. After 6 months of follow-up, 14 (12.7%) developed RA and 34 (30.9%) IA. US active inflammation was present in 38 (34.5%) patients (28.2% showed PD synovitis and 18.2% PD tenosynovitis). Multivariate analysis showed that ACPA (OR 1.0003; 95% CI 1.002-1.006) and ESR (OR 1.054; 95% CI 1.016-1.094) were significantly associated with the detection of US active inflammation at baseline. Only PD tenosynovitis was found to be an independent predictive factor of an evolution towards RA (OR 6.982; 95% CI 1.106-44.057) and IA (OR 5.360; 95% CI 1.012-28.390).
US is able to detect features of subclinical inflammation in CSA patients, especially in those with higher ESR and ACPA values. Only PD tenosynovitis at baseline US assessment was found to be an independent predictor of RA and IA development in CSA patients.
Objective
To evaluate the impact of musculoskeletal ultrasound (MSUS) in the management of rheumatoid arthritis (RA) patients and to investigate factors affecting treatment strategy by the referring ...rheumatologist.
Methods
Prospective study of RA patients evaluated at a MSUS clinic over a 6‐month period. Data extraction included demographics, current treatment and MSUS findings. Pre‐ and post‐MSUS follow‐up of 3 months data were analyzed. Patients were classified into 2 groups based on the decision of the referring rheumatologist to change the treatment after the MSUS examination. Comparisons between groups were performed in a univariate analysis. We used logistic regression models to investigate factors associated with changes in clinical management.
Results
A total of 64 RA patients were included. Mean age was 61.9 years and 83.6% were female. Main referral indication was assessment of disease activity (89%). Overall, MSUS led to subsequent therapeutic actions by the referring rheumatologist in 41 (64.1%) patients, and to a change in the clinical impression of the complaint that generated the referral in 7 (11.5%) patients. The detection of power Doppler (PD), the 28 swollen joint count and the presence of radiographic erosions were significantly associated with a subsequent clinical action. In the multivariate analysis only PD remained significant (odds ratio = 3.29; 95% CI: 1.05‐10.26).
Conclusion
Disease activity evaluation is the most common indication for MSUS examination, with the presence of PD the factor most frequently associated with changes in therapeutic management. This study highlights the impact of MSUS, especially the use of PD, to support treatment decisions in RA routine care.
Abstract
Objective
To evaluate the impact of cardiovascular risk (CVR) on the diagnostic accuracy of the ultrasonographic (US) Halo Score in patients with suspected giant cell arteritis (GCA).
...Methods
Retrospective observational study of patients referred to our US fast track clinic with suspected GCA for a 2-year period. The intima-media thickness (IMT) of cranial and extra-cranial arteries and the Halo Score was determined to assess the extent of vascular inflammation. The European Society of Cardiology Guidelines on CV Disease Prevention were used to define different categories of CVR and patients were classified according to the Systemic Coronary Risk Evaluation (SCORE). The gold standard for GCA diagnosis was clinical confirmation after a 6-month follow-up.
Results
Of the 157 patients included, 47 (29.9%) had GCA after a 6-month follow-up. Extra-cranial artery IMT was significantly higher in patients with high/very high CVR than in those with low/moderate CVR, but only among patients without GCA. Non-GCA patients with high/very high CVR had also a significantly higher Halo Score in contrast with low/moderate CVR 9.38 (5.93) vs 6.16 (5.22);
p
= 0.007. The area under the ROC curve of the Halo Score to identify GCA was 0.835 (95% CI 0.756–0.914), slightly greater in patients with low/moderate CVR (0.965 95% CI 0.911–1) versus patients with high/very high CVR (0.798 95% CI 0.702–0.895). A statistically weak positive correlation was found between the Halo Score and the SCORE (
r
0.245;
c
= 0.002).
Conclusions
Elevated CVR may influence the diagnostic accuracy of the US Halo Score for GCA. Thus, CVR should be taken into consideration in the US screening for GCA.
The distribution and role of tumor‐infiltrating leucocytes in glioblastoma (GBM) remain largely unknown. Here, we investigated the cellular composition of 55 primary (adult) GBM samples by flow ...cytometry and correlated the tumor immune profile with patient features at diagnosis and outcome. GBM single‐cell suspensions were stained at diagnosis (n = 44) and recurrence following radiotherapy and chemotherapy (n = 11) with a panel of 8‐color monoclonal antibody combinations for the identification and enumeration of (GFAP+CD45−) tumor and normal astrocytic cells, infiltrating myeloid cells —i.e. microglial and blood‐derived tumor‐associated macrophages (TAM), M1‐like, and M2‐like TAM, neutrophils. and myeloid‐derived suppressor cells (MDSC)— and tumor‐infiltrating lymphocytes (TIL) —i.e. CD3+T‐cells and their TCD4+, TCD8+, TCD4−CD8−, and (CD25+CD127lo) regulatory (T‐regs) subsets, (CD19+CD20+) B‐cells, and (CD16+) NK‐cells—. Overall, GBM samples consisted of a major population (mean ± 1SD) of tumor and normal astrocytic cells (73% ± 16%) together with a significant but variable fraction of immune cells (24% ± 18%). Within myeloid cells, TAM predominated (13% ± 12%) including both microglial cells (10% ± 11%) and blood‐derived macrophages (3% ± 5%), in addition to a smaller proportion of neutrophils (5% ± 9%) and MDSC (4% ± 8%). Lymphocytes were less represented and mostly included TCD4+ (0.5% ± 0.7%) and TCD8+ cells (0.6% ± 0.7%), together with lower numbers of TCD4−CD8− T‐cells (0.2% ± 0.4%), T‐regs (0.1% ± 0.2%), B‐lymphocytes (0.1% ± 0.2%) and NK‐cells (0.05% ± 0.05%). Overall, three distinct immune profiles were identified: cases with a minor fraction of leucocytes, tumors with a predominance of TAM and neutrophils, and cases with mixed infiltration by TAM, neutrophils, and T‐lymphocytes. Untreated GBM patients with mixed myeloid and lymphoid immune infiltrates showed a significantly shorter patient overall survival versus the other two groups, in the absence of gains of the EGFR gene (p = 0.02). Here we show that immune cell infiltrates are systematically present in GBM, with highly variable levels and immune profiles. Patients with mixed myeloid and T‐lymphoid infiltrates showed a worse outcome.
To investigate the predictive value of synovitis detected by Doppler US in relation to failed tapering of biologic therapy (BT) in RA patients in sustained clinical remission.
A total of 77 RA ...patients (52 women, 25 men) in sustained clinical remission, treated with a stable dosage of BT were prospectively recruited. BT was tapered according to an agreed strategy implemented in clinical practice (i.e. increasing the interval between doses for s.c. BT and reducing the dose for i.v. BT). BT tapering failure was assessed at 6 and 12 months. Doppler US investigation of 42 joints for the presence and grade (0-3) of B-mode synovial hypertrophy and synovial power Doppler signal (i.e. Doppler synovitis) was performed at baseline by a rheumatologist blinded to clinical and laboratory data. Hand and foot radiographs were obtained at baseline and at 12-month follow-up.
Of the 77 patients, 46 (59.7%) were on s.c. BT and 31 (40.3%) on i.v. BT. At 12 months, 35 patients (45.5%) presented BT tapering failure, 23 of them (29.9% of all patients) in the first 6 months of BT tapering. In logistic regression analysis, the baseline DAS28 and the global score of Doppler synovitis were identified as independent predictors of BT tapering failure at 12 and 6 months. The presence of Doppler synovitis was the strongest predictor for BT tapering failure. No patient showed radiographic progression.
Our results suggest that the presence of Doppler-detected synovitis may predict BT tapering failure in RA patients in sustained clinical remission.
Batteries play a key role in achieving the target of universal access to reliable affordable energy. Despite their relevant importance, many challenges remain unsolved with regard to the ...characterization and management of batteries. One of the major issues in any battery application is the estimation of the state-of-charge (SoC). SoC, which is expressed as a percentage, indicates the amount of energy available in a battery. An accurate SoC estimation under realistic conditions improves battery performance, reliability, and lifetime. This paper proposes an SoC estimation method based on a new hybrid model that combines multivariate adaptive regression splines (MARS) and particle swarm optimization (PSO). The proposed hybrid PSO-MARS-based model uses data obtained from a high-power load profile (dynamic stress test) specified by the United States Advanced Battery Consortium (USABC). The results provide comparable accuracy to other more sophisticated techniques but at a lower computational cost.
Objective
Salivary gland ultrasound (SGU) is a reliable technique for assessing the salivary glands in patients with primary Sjögren’s syndrome (pSS). The aim of this study was to elucidate the ...relationship between SGU findings and autoimmunity in patients with pSS.
Methods
Patients with pSS underwent an SGU assessment. The patients were classified into three groups according to their autoimmunity profile: the complete positive group (positive rheumatoid factor, antinuclear antibodies, and anti-Ro/anti-La antibodies), the partial seropositive group (positivity of at least one autoantibody but not all), and the seronegative group.
Results
In total, 93 patients were evaluated. Eighty-six (92.5%) were female, and their median age was 49.5 years. The median disease duration was 12.3 years. Pathological SGU findings were present in 32 (34.4%) patients 25 of 36 (78.1%) in the complete positive group and 7 of 44 (21.9%) in the partial positive group. Patients with pathological SGU findings had a shorter disease duration and slightly higher European League Against Rheumatism Sjögren’s syndrome disease activity index.
Conclusions
The autoimmunity profile and pathological SGU findings are strongly associated with each other in patients with pSS. However, the disease duration does not seem to be related to pathological SGU findings.
Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disease that often requires biological therapy to control its activity. Medication persistence and adherence are important aspects on ...which we have scarce information. We performed a longitudinal, retrospective, and observational study based on data from the daily clinical management of JIA patients. We recorded clinical remission at 6 and 12 months. Persistence of biological therapy was evaluated using Kaplan-Meier curves, and adherence was assessed using the medication possession ratio (MPR). We included 68 patients who received biological therapy. Of these, 11 (16.2%) and 5 (7.4%) required a second and third drug, respectively. The persistence rate for biological therapy at 5 years was 64%, with no differences between the first and second lines. Adherence was high during the first year of treatment (MPR80: 96.3%) and also in the second and third years (MPR80: 85.2% and 91.8%, respectively). Persistence and adherence to biological therapy were remarkably high in our JIA cohort. Adherence to biological treatments could be related to a higher probability of fulfilling the Wallace remission criteria at 6 months, although this was not confirmed at 12 months.