Disease Duration Research Articles

Comparative early effectiveness across 14 PsA drugs and 5 classes of PsA treatment: 3-month results from the PRO-SPIRIT study

BackgroundThe psoriatic arthritis (PsA) Observational Study of Persistence of Treatment (PRO-SPIRIT) assesses effectiveness and persistence of real-world PsA treatments. Ixekizumab (IXE) is an interleukin (IL)-17A inhibitor (i) (IL-17Ai), approved for...

Serum markers of microbial translocation and intestinal damage in assessment of gastrointestinal tract involvement in systemic sclerosis

Gastrointestinal (GI) tract involvement affects up to 90% of Systemic sclerosis (SSc) patients. The presence of GI symptoms is assessed by the University of California, Los Angeles, and Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT 2.0). Microbial translocation (MT) is reported in SSc patients consequently to increased intestinal permeability due to intestinal damage (ID) and dysbiosis. Aim of this study was to assess circulating levels of LBP and EndoCab IgM (markers of MT), IL-6 (marker of inflammation), I-FABP and Zonulin (markers of ID) in a cohort of SSc patients and healthy controls (HC). Moreover, we aimed to correlate these parameters with severity of GI symptoms. UCLA SCTC GIT 2.0 questionnaire was administered to 60 consecutive SSc patients. Markers of MT, inflammation and ID were evaluated in SSc patients and HC. SSc patients had higher median value of markers of MT, inflammation and ID than HC. The logistic regression analysis showed LBP as the only variable associated with an UCLA total score “moderate-to-very severe” [OR 1.001 (CI 95%: 1.001–1.002), p < 0.001]. The logistic regression analysis showed LBP [OR 1.002 (CI 95%: 1.001–1.003), p < 0.01] and disease duration [OR 1.242 (CI 95%: 1.023–1.506), p < 0.05] as variables associated with UCLA distension/bloating “moderate-to-very severe”. The logistic regression analysis showed LBP as the only variable associated with UCLA diarrhea “moderate-to-very severe” [OR 1.002 (CI 95%: 1.001–1.003), p < 0.01]. SSc patients with dysregulation gut mucosal integrity expressed by high levels of MT and ID biomarkers had more severe GI symptoms.

The application and clinical translation of the self-evolving machine learning methods in predicting diabetic retinopathy and visualizing clinical transformation

ObjectiveThis study aims to analyze the application and clinical translation value of the self-evolving machine learning methods in predicting diabetic retinopathy and visualizing clinical outcomes.MethodsA retrospective study was conducted on 300 diabetic patients admitted to our hospital between January 2022 and October 2023. The patients were divided into a diabetic retinopathy group (n=150) and a non-diabetic retinopathy group (n=150). The improved Beetle Antennae Search (IBAS) was used for hyperparameter optimization in machine learning, and a self-evolving machine learning model based on XGBoost was developed. Value analysis was performed on the predictive features for diabetic retinopathy selected through multifactor logistic regression analysis, followed by the construction of a visualization system to calculate the risk of diabetic retinopathy occurrence.ResultsMultifactor logistic regression analysis revealed that being male, having a longer disease duration, higher systolic blood pressure, fasting blood glucose, glycosylated hemoglobin, low-density lipoprotein cholesterol, and urine albumin-to-creatinine ratio were risk factors for the development of diabetic retinopathy, while non-pharmacological treatment was a protective factor. The self-evolving machine learning model demonstrated significant performance advantages in early diagnosis and prediction of diabetic retinopathy occurrence.ConclusionThe application of the self-evolving machine learning models can assist in identifying features associated with diabetic retinopathy in clinical settings, enabling early prediction of disease occurrence and aiding in the formulation of treatment plans to improve patient prognosis.

Influencing factors of self-management ability among dry eye patients in west China.

To investigate the current situation and influencing factors of self-management ability in dry eye patients in west China. A total of 265 patients clinically diagnosed with dry eye received a convenience survey questionnaire at West China Hospital of Sichuan University. All participants completed the rating scale of health self-management skill for adults (AHSMSRS), Huaxi Emotional-Distress Index (HEI), e-health literacy scale (e-HEALS) and Brief Illness Perception Questionnaire (Brief-IPQ). A generalized linear model was employed to establish a multivariate linear model with demographic data, psychological state, e-HEALS, and illness perception as independent variables and health self-management skill score as the dependent variable. The mean score for health self-management skill was 165.58±15.79. Multivariate analysis revealed that advanced age, better illness perception and improved psychological state were associated with better health self-management ability among dry eye patients. Furthermore, the health self-management ability of patients with a disease duration less than 1y was found to be higher compared to those with a disease duration exceeding 1y. The health self-management ability of dry eye patients in west China is relatively high. Age, duration of disease, illness perception and psychological state are the influencing factors on the health self-management ability of dry eye patients.

Exploring Sleep Architecture in Polish Patients with Multiple Sclerosis: A Polysomnography Study

Background: Sleep disturbances are a prevalent phenomenon in patients with multiple sclerosis (PwMS). The present study employs polysomnography (PSG) to quantify sleep efficiency and architecture in PwMS, aiming to elucidate the relationships between PSG parameters and factors including gender, disability level, brain lesion location, and subjective measures of insomnia, excessive daytime sleepiness (EDS), fatigue, pain, and mood disorders. Methods: The study cohort comprised 51 adult PwMS, of whom 31 underwent overnight PSG. The demographic and clinical characteristics, including age, gender, and Expanded Disability Status Scale (EDSS), were collated. The Athens Insomnia Scale, the Epworth Sleepiness Scale, the Fatigue Severity Scale, the Modified Fatigue Impact Scale (MFIS), the Numerical Pain Rating Scale, and the Hospital Anxiety and Depression Scale were employed for the assessment of insomnia, EDS, fatigue, pain, and mood disorders. The brain and spinal cord magnetic resonance imaging (MRI) were evaluated. Results: A reduced sleep efficiency was observed among 30 PwMS (aged 38.9 ± 12.9), with a mean of 80 ± 12%, especially in those with brainstem demyelinating lesions. In those PwMS aberrant sleep onset latency (SOL) and wake after sleep onset were also noted (p &lt; 0.05). The prevalence of sleep fragmentation, as measured by the total arousal index, was greater in male PwMS than in female (p &lt; 0.05). Higher disability according to the EDSS correlated with longer SOL (ρ = 0.48, p &lt; 0.05), and reduced N2 sleep stage correlated with cognitive fatigue according to MFIS (ρ = −0.46, p &lt; 0.05). Age, disease duration, insomnia, EDS, physical fatigue, and mood disorders did not impact PSG parameters. Conclusions: The study demonstrated the disruption of sleep architecture in PwMS, and highlighted the importance of a comprehensive PSG assessment of sleep disturbances in this population.

Static and temporal dynamic alterations of local functional connectivity in chronic insomnia.

Several studies have revealed altered intrinsic neural activity in chronic insomnia (CI). However, the temporal variability of intrinsic neural activity in CI is rarely mentioned. This study aimed to explore static and temporal dynamic alterations of regional homogeneity (ReHo) in CI and excavate the potential associations between these changes and clinical characteristics.Eighty-seven patients with CI and seventy-eight healthy controls (HCs) were included. Resting-state functional magnetic resonance imaging was performed on all subjects and both static and dynamic ReHo were used to detect local functional connectivity. We then tested the relationship between altered brain regions, disease duration, and clinical scales. The receiver operating characteristic curve analysis was used to reveal the potential capability of these indicators to screen CI patients from HCs.CI showed increased dynamic ReHo in the right precuneus and decreased static ReHo in the right cerebellum_6. The dynamic ReHo values of the right precuneus were negatively correlated with the self-rating depression score and the static ReHo values of the right cerebellum_6 were positively correlated with the Montreal Cognitive Assessment-Naming score. In addition, the combination of the two metrics showed a potential capacity to distinguish CI patients from HCs, which was better than a single metric alone.The present study has revealed the altered local functional connectivity under static and temporal dynamic conditions in patients with CI, and found the relationships between these changes, mood-related scales, and cognitive-related scales. These may be useful in elucidating the neurological mechanisms of CI and accompanying symptoms.

Can we differentiate patients with dysferlinopathies and inflammatory myopathies by ultrasound? A discriminant analysis study.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of diseases that are characterized by inflammation and muscle weakness. Dysferlinopathies are autosomal recessive limb-girdle muscular dystrophies caused by mutations in DYSF, which share a similar clinical presentation and histopathological inflammatory changes. To determine the sonographic differences between dysferlinopathies and IIM and whether these differences allow their classification. This observational, cross-sectional, and analytical study evaluated 20 muscles from 11 patients with dysferlinopathies and 11 patients with IIM. The patients were matched for age, sex, and disease duration. Clinical and laboratory variables were analyzed. Semi-quantitative scales were used to weigh the gray scale and power Doppler muscle abnormalities. Descriptive statistics were computed and discriminant analysis was performed to determine the ultrasound variables that best predicted the final diagnosis. Forty muscles were evaluated. Atrophy and higher Heckmatt scale scores were observed in patients with dysferlinopathies. A set of three muscles (biceps/brachialis, quadriceps, and gastrocnemius) had a diagnostic accuracy of 100% (sensitivity, 100%; specificity, 100%; canonical coefficient, 0.733 p < 0.001). A set of two formulas was used to classify both diseases correctly. In the present study, scanning of three muscle groups showed high diagnostic accuracy in differentiating dysferlinopathies from MII. Ultrasound can be used as an initial test in patients with suspected muscle disease or as an additional tool to support diagnosis in controversial cases.

Beliefs of Moroccan patients with chronic inflammatory rheumatic diseases regarding medication: related factors and correlation with therapeutic adherence

BackgroundMedication adherence is one of the key elements of the management of patients with chronic inflammatory rheumatic diseases (CIRDs), adherence/medication regimes are prone to being influenced by beliefs about medicines; such beliefs can influence the management and quality of life of patients. Several factors may be associated with these beliefs, including demographic and clinical factors, as well as socio-psychological factors. The aim of this study is to assess beliefs regarding medications among Moroccan patients with CIRDs, the factors associated with these beliefs, and the correlation of these factors with medication adherence.Material and methodThis cross-sectional study included patients with CIRDs. Sociodemographic data, comorbidities, and information about CIRDs (type, disease duration, pain evaluation, disease activity and treatments) were collected. Beliefs regarding medication were assessed by the Belief about Medicine Questionnaire (BMQ). Therapeutic adherence was assessed using the Arabic version of the Compliance Questionnaire in Rheumatology (CQR). Sociopsychological factors, such as catastrophism and trust in physicians, were assessed by the Pain Catastrophizing Scale (PCS) and the Trust in Physicians Scale (TPS), respectively.ResultOur sample included 189 patients. The average age was 47.49 ± 13.7; 52.4% had comorbidities; and 49.2% had a low level of education. Of the patients, 49.7% were on glucocorticoids, 61.9% on conventional synthetic disease-modifying antirheumatic drugs and 6.3% on biologics. The median necessity-concern differential was 6 [1–12]. Of the patients, 67.4% strongly believed that medication was essential to maintain their health. The long-term side effects were the main concerns about medicines (51.3%). In a multivariate analysis, there was a statistically significant association between low level of education, catastrophizing, methotrexate use, and trust in the physician as independent factors and the BMQ necessity-concern differential as the dependent factor. There was also a significant correlation between CQR and the BMQ necessity score.ConclusionMoroccan patients with CIRDs have a rather positive perception of their medication. This perception seems to influence their adherence to treatment. Low levels of education, catastrophizing, methotrexate use, and trust in physicians are the most important factors associated with patients’ beliefs regarding medication.

Study of Eye Movements Abnormalities in Epilepsy

ABSTRACT Idiopathic epilepsy (IE), is a group of epileptic syndromes with no structural brain lesion, but with microstructural changes in neuronal networks leading to neuropsychological consequences. Therefore, the assessment of saccadic eye movements can provide insight into the integrity of cerebral networks as it involves large cortical and subcortical brain areas and circuitries. Describe saccadic eye movement abnormalities in patients with IE and correlate them with disease characteristics and antiseizure medication. Case–control study including IE patients followed in the Neurology Department of Razi University Hospital and healthy controls matched. Participants underwent a recording of saccadic eye movements. Pursuit, prosaccade, and anti-saccade tasks were performed. 115 patients and 98 matched healthy controls were included. The gender ratio (male to female) was 0.6. The mean age at onset was 16.3 ± 12 years. Diagnosed epileptic syndromes were juvenile myoclonic epilepsy (JME), epilepsy with generalized tonic-clonic seizures, childhood absence epilepsy, temporal lobe epilepsy, frontal lobe epilepsy, and rolandic epilepsy. Saccadic eye movements were impaired in 52.2% of our patients and significantly more altered in those with JME (p = .021). Prolonged horizontal saccades latencies were the most frequent eye movement abnormalities (32.1%), followed by altered horizontal smooth pursuit (22.6%). A positive correlation was found between age at eye movements recording, age at onset, disease duration, global cognitive impairment, and saccadic eye movements. However, no definite relationship was identified. Saccadic eye movement illustrates extending anatomic alterations in IE including frontal and temporoparietal cortical areas and cortico-subcortical circuits. Eye movement recording is a useful and reproducible tool in the assessment of epileptic patients and provides a better understanding of neuronal mechanisms in epilepsy.

Pain catastrophizing negatively impacts drug retention rate in patients with Psoriatic Arthritis and axial Spondyloarthritis: results from a 2-years perspective multicenter GIRRCS (Gruppo Italiano di Ricerca in Reumatologia Clinica) study

BackgroundChronic pain and inflammation are common features of rheumatic conditions such as Psoriatic Arthritis (PsA) and Axial Spondyloarthritis (axSpA), often needing prolonged medication treatment for effective management. Maintaining drug retention is essential for both achieving disease control and improving patients' quality of life. This study investigates the influence of pain catastrophizing, a psychological response to pain, on the drug retention rates of PsA and axSpA patients.MethodsA two-year prospective multicenter observational study involved 135 PsA and 71 axSpA patients. Pain Catastrophizing Scale (PCS) was employed to assess pain catastrophizing. Univariable and multivariable regression analyses were utilized to identify factors associated with drug retention.ResultsIn the PsA group, patients early discontinuing therapy showed higher baseline disease activity as well as higher incidence of comorbid fibromyalgia. Notably, pain catastrophizing, specifically the domains of Helplessness, Magnification, and Rumination, were significantly elevated in PsA patients who interrupted the treatment. Multivariable analysis confirmed pain catastrophizing as an independent predictor of drug suspension within two years.In axSpA, drug discontinuation was associated with female gender, shorter disease duration, higher baseline disease activity as well as elevated levels of pain catastrophizing. Univariable analysis supported the role of pain catastrophizing, including its domains, as predictors of treatment interruption. However, limited events in axSpA patients precluded a multivariate analysis.ConclusionThis prospective study emphasizes the impact of pain catastrophizing on drug retention in patients with PsA and axSpA.

Assessment of the Multiple Sclerosis Severity Score and the Age-Related Multiple Sclerosis Severity Score as health indicators in a population-based cohort.

People with multiple sclerosis (MS) present varying degrees of disability throughout their disease course. The Multiple Sclerosis Severity Score (MSSS) and the Age-Related Multiple Sclerosis Severity Score (ARMSSS) adjust the Expanded Disability Status Scale (EDSS) according to disease duration and age, respectively. These measures could be useful for quantifying MS severity and as health outcome indicators for benchmarking in population-based settings. The aim of this study was to describe the severity of MS in our health district using the MSSS and ARMSSS and to assess their consistency over time. This population-based study included patients from our health district who were diagnosed with MS according to the 2010 McDonald criteria, had a disease duration of at least one year and were followed up in our MS unit. Sex, age at onset, disease duration, clinical course, age and irreversible EDSS at the last follow-up visit were collected, and the MSSS and ARMSSS were calculated at two time points: 2017 and 2020. One hundred seventy-seven patients were included in 2017, and 208 in 2020. The prevalence of MS was 90 and 104 per 100,000 inhabitants, respectively. The median MSSS was 1.77 (IQR 0.76-4.28) in 2017 and 2.03 (IQR 0.82-4.36) in 2020. The median ARMSSS was 2.90 (IQR 1.47-5.72) in 2017 and 2.93 (IQR 1.51-5.56) in 2020. No significant differences were found. According to the MSSS and ARMSSS, the severity of MS in our area is mild, and these instruments are consistent. These measures could be reliable health outcome measures.

Comparison of kidney and hepatic outcomes among sodium-glucose cotransporter-2 inhibitors: a retrospective study using multiple propensity scores

BackgroundSodium-glucose cotransporter-2 inhibitors (SGLT2i) have been reported to have effects beyond lowering blood glucose levels, with certain SGLT2i expanding their indications to chronic kidney disease and chronic heart failure. We focused on the hepatoprotective and renoprotective effects of six SGLT2i and assessed whether the effects were unique to each drug or common class effects, in addition to whether the renal and hepatoprotective effects vary based on renal and hepatic status.MethodsPatients with diabetes (ipragliflozin: 837, empagliflozin: 850, canagliflozin: 922, dapagliflozin: 590, tofogliflozin: 288, and luseogliflozin: 193) who initiated SGLT2i treatment and were monitored for one year were included. The propensity score (PS) was calculated using patient backgrounds (age, sex, height, weight, body mass index [BMI], disease duration, concomitant diabetes medications, underlying conditions, glycated hemoglobin [HbA1c], estimated glomerular filtration rate [eGFR], aspartate aminotransferase [AST], alanine aminotransferase [ALT], high-density lipoprotein [HDL], low-density lipoprotein [LDL], and triglyceride [TG] levels) as covariates. Additionally, the inverse probability of treatment weighting (IPTW) approach was used to compare liver and renal function test values.ResultsPre- and 12-month post-treatment comparisons demonstrated a significant reduction in hepatic function (AST and ALT) and an increase in renal function (eCcr and eGFR) for all SGLT2i. Comparison of differences between pre- and 12-month post-treatment using the IPTW approach demonstrated no significant differences in AST, ALT, and eGFR levels between SGLT2i. At 12 months post-treatment, 67 patients were classified as having a more severe CKD than those at pre-treatment, representing only 1.8% of all patients (67/3,680). Similarly, 107 patients with AST and 147 patients with ALT were classified as having progressed to a more severe grade than at pre-treatment, representing only 2.9 and 4.0%, respectively.ConclusionsRenoprotective and hepatoprotective effects are class effects of SGLT2i, and their effects are thought to be independent of kidney or liver status.

Erosion regression in patients with rheumatoid arthritis after upadacitinib-a pilot study using high resolution peripheral quantitative computed tomography.

To evaluate whether inhibition of Janus kinases (JAK) 1 could lead to erosion repair on high-resolution peripheral quantitative computer tomography (HR-pQCT) in patients with active rheumatoid arthritis (RA). This was a prospective, non-randomized pilot study. We enrolled 20 adult patients with active RA with ≥1 bone erosion on HR-pQCT. They were given upadacitinib 15 mg once daily for 24 weeks. HR-pQCT of the metacarpophalangeal joint was performed at baseline and 24-week. The serum bone biomarkers level was evaluated before and after treatment. Twenty age-and-sex matched RA patients from another study treated with conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs) were included as active controls. Nineteen patients in the upadacitinib group completed the study procedures. After 24 weeks, despite similar improvement in disease activity, a reversed trend in the mean erosion volume change on HR-pQCT was observed comparing the upadacitinib and active control group (upadacitinib group: -0.23 ± 3.26mm3 vs control group: 1.32 ± 6.05mm3, p= 0.131). A greater proportion of erosions in the upadacitinib group demonstrated regression (27% vs 12%, p= 0.085). Using general estimating equation (GEE), the use of upadacitinib was significantly associated with erosion regression (OR: 3.61, 95% CI: 1.00-13.00, p= 0.049) after adjusting for the difference in disease duration. The serum levels of bone resorption markers reduced after upadacitinib treatment. No new safety signal was noted. Despite a similar improvement in RA disease activity after upadacitinib compared with csDMARDs, a differential regression of erosion on HR-pQCT was observed in patients received upadacitinib. The potential role of JAK1 inhibition in erosion repair should be investigated.

ITPR3-associated neuropathy: Report of a further family with adult onset intermediate Charcot-Marie-Tooth disease.

ITPR3 encodes type 3 inositol-tri-phosphate receptor (IP3R3), a protein expressed in Schwann cells, predominantly in the paranodal region, and involved in the regulation of Ca2+ release from the endoplasmic reticulum. Dominant variants in ITPR3 have recently been recognized as a rare cause of intermediate Charcot-Marie-Tooth disease (CMT). We collected the clinical data of a family with autosomal dominant neuropathy whose proband was diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) for many years. The genetic diagnosis was achieved by whole exome sequencing. The proband developed symmetrical sensory-motor neuropathy with demyelinating features at 32 years old. He was diagnosed with CIDP and received numerous immunomodulatory treatments. However, his condition progressed, leading to severe proximal leg and hand atrophy that confined him to a wheelchair at 60 years. The patient's two sons began to exhibit symptoms suggestive of neuropathy shortly after age 30 years, and the condition was reoriented as inherited. Exome sequencing identified a heterozygous c.4271C > T variant in the ITPR3 gene segregating with the disease. Nerve conduction studies showed a combination of demyelinating and axonal features that vary by nerve, disease duration, and patient. A uniform thickening of the nerves was identified on nerve echography, as was distal symmetric fatty infiltration in lower limb muscle imaging. The c.4271C > T ITPR3 variant causes a late onset CMT that can be considered an intermediate CMT. Considering the electrophysiological findings and the distribution of IP3R3, we hypothesize that this variant could start as nodal dysfunction that progresses to widespread nerve degeneration.

Associations between Thyroid Hormones and Cognitive Impairment in Patients with Parkinson's Disease.

This study aims to explore the correlation of serum thyroid hormone levels to cognitive impairments in Parkinson's disease (PD) patients. In this retrospective study, 106 Chinese patients without cognitive impairments and 94 patients with cognitive impairments, including 55 with mild cognitive impairment (PD-MCI) and 39 with Parkinson's disease dementia (PDD), were analyzed. Clinical data regarding the PD assessments, including disease duration, Unified Parkinson's Disease Rating Scale (UPDRS) Part III scores, and Hoehn and Yahr staging, were analyzed. Cognitive functions were evaluated using the Montreal Cognitive Assessment (MoCA) score. Serum levels of thyroid-stimulating hormone (TSH), free thyroxine (FT4) and free triiodothyronine (FT3), were measured using ELISA. Significantly altered Hoehn and Yahr staging, disease duration, and UPDRS Part III scores were observed in PD patients with cognitive impairment compared to those without. Serum levels of FT3 were significantly decreased, while FT4 and TSH levels were significantly elevated in PD patients with cognitive impairment compared to those without. Combined detection of TSH, FT3, and FT4, showed value in distinguishing PD patients with and without cognitive impairment. Furthermore, a comparison of serum levels between PD-MCI and PDD patients revealed significant association between thyroid hormone levels and the degree of cognitive impairment in PD patients. Our findings suggest a relationship between changes in serum thyroid hormone levels and cognitive impairments in PD patients. Thyroid hormone levels, particularly FT3, may serve as potential markers for cognitive dysfunction in PD.Significance Statement The correlation of serum thyroid hormone levels to cognitive impairments in Parkinson's disease (PD) patients remains unclear. The current results demonstrate a relationship between changes in serum thyroid hormone levels and cognitive impairments in PD patients. Our findings suggest that thyroid hormone levels, particularly FT3, may serve as potential markers for cognitive dysfunction in PD.

The phenotype of the pediatric patient with Cushing syndrome but without obesity.

Cushing syndrome (CS) often presents with obesity that is not as severe in children as it is in adults. The role of obesity in the severity of metabolic syndrome in children with CS has not been studied. This study evaluates whether pediatric patients with CS have obesity-specific differences in their demographic, biochemical and presenting findings. Cohort study. We analyzed 273 patients with young onset of CS at ≤ 18 years old and classified as patients with or without obesity based on their BMI z-scores. Patients without obesity (n=84, 31%) were more frequently females with an older age of onset compared to patients with obesity (n=189, 69%). Consistent with their older age, patients without obesity were also more likely to have advanced Tanner stages. Patients with and without obesity had similar duration of disease, but patients with obesity showed higher markers of hypercortisolemia (urinary free cortisol). A higher prevalence of hypertension and insulin resistance was seen in patients with obesity than those without obesity, adjusting for urinary free cortisol (UFC) (p < .05 for all comparisons). While fatty liver disease was not statistically different among the entire cohort, elevated alanine transaminase (ALT) and metabolic dysfunction-associated steatotic liver disease (MASLD) scores were more common in ACTH-dependent CS patients with obesity (p < .05). Weight gain appears to mediate some but not all the cortisol-associated complications in pediatric CS. Therefore, obesity may be a modifiable risk factor among these patients.

The real-world applicability of the 2023 international myelin oligodendrocyte glycoprotein antibody-associated disease criteria in a Latin American cohort.

The diagnostic criteria for myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) were published in 2023. We aimed to determine the performance of the new criteria in Latin American (LATAM) patients compared with the 2018 criteria and explore the significance of MOG-IgG titers in diagnosis. We retrospectively reviewed the medical records of LATAM (Argentina, Chile, Brazil, Peru, Ecuador, and Colombia) adult patients with one clinical MOGAD event and MOG-IgG positivity confirmed by cell-based assay. Both 2018 and 2023 MOGAD criteria were applied, calculating diagnostic performance indicators. Among 171 patients (predominantly females, mean age at first attack = 34.1 years, mean disease duration = 4.5 years), 98.2% patients met the 2018 criteria, and of those who did not fulfill diagnostic criteria (n = 3), all tested positive for MOG-IgG (one low-positive and two without reported titer). Additionally, 144 (84.2%) patients met the 2023 criteria, of whom 57 (39.5%) had MOG-IgG+ titer information (19 clearly positive and 38 low-positive), whereas 87 (60.5%) patients had no MOG-IgG titer. All 144 patients met diagnostic supporting criteria. The remaining 27 patients did not meet the 2023 MOGAD criteria due to low MOG-IgG (n = 12) or lack of titer antibody access (n = 15), associated with the absence of supporting criteria. The 2023 MOGAD criteria showed a sensitivity of 86% (95% confidence interval = 0.80-0.91) and specificity of 100% compared to the 2018 criteria. These findings support the diagnostic utility of the 2023 MOGAD criteria in an LATAM cohort in real-world practice, despite limited access to MOG-IgG titration.

Is Baricitinib Effective and Safe for Patients with Difficult-to-Treat Rheumatoid Arthritis? Comparative Data with the RA Group of Rheumatoid Arthritis not Difficult to Treat.

This study investigates the efficacy and safety of baricitinib, an oral targeted synthetic Disease Modifying Anti-Rheumatic Drugs (DMARD), in patients with difficult-to-treat rheumatoid arthritis (D2T RA) compared to those without, aiming to determine its potential as an alternative treatment for D2T RA. A total of 78 patients participated in this retrospective cohort study, with 33 meeting the D2T RA criteria and 45 were in the non-D2T RA group. Various clinical and laboratory parameters, adverse events, and disease activity indices were assessed, alongside drug efficacy and survival rates. Patients with D2T RA exhibited higher seronegativity, prior use of bDMARDs and cDMARDs, and longer disease duration. Both groups experienced reductions in VAS and DAS28 scores, as well as SDAI, CDAI, HAQ, CRP, and ESR levels at baseline and 3, 6, and 12 months post-baricitinib initiation, with sustained efficacy observed over 12 months. The most prevalent adverse event was infection (28.21%). Although initial drug survival rates were similar between groups, the non-D2T RA group demonstrated higher rates at 24 months (46.70% vs. 59.40%). Subgroup analyses showed comparable survival rates between D2T RA and non-D2T RA groups, whether treated with baricitinib alone or in combination with methotrexate or leflunomide. Despite potential treatment resistance, patients meeting the D2T RA criteria shared similar safety and efficacy profiles with those non-D2T RA. Baricitinib emerges as a promising treatment option for D2T RA patients, offering effectiveness and safety comparable to the non-D2T RA group.

The examination of detrusor underactivity in multiple system atrophy

AimsThe urinary dysfunction in multiple system atrophy (MSA) is characterized by large post-void residuals (PVR) due to impaired bladder contractility. However, the evaluations of bladder contractility are not well validated in MSA. Because the bladder contractility index can be generally represented as Pdet Qmax (detrusor pressure at maximum urinary flow rate) + kQmax (maximum urinary flow rate), we aim to examine which “k” value is suitable for representing bladder contractility concerning its correlations to PVR and voided percentage (VOID%).MethodsWe retrospectively reviewed 133 patients with MSA (74 males, 59 females, mean disease duration 3.2 years) who underwent an urodynamic study. We calculated bladder contractility using the formula PIPk = Pdet Qmax + kQmax by increasing the “k” value from 0.1 to 10 by increments of 0.1. We calculated the correlations between each PIPk (k = 0.1–10.0) and PVR and VOID%.ResultsThe correlational coefficients between PIPk and VOID% were larger than those between PIPk and PVR. The correlational coefficients between PIPk and VOID% reached a plateau level at a “k” value &amp;gt;5.0 in male patients, suggesting that currently used formulas such as Pdet Qmax + 5Qmax for males might be appropriate for male MSA patients. However, the correlational coefficients between PIPk and VOID% reached a plateau level in female patients when the “k” values were &amp;gt;6.0, which might overestimate bladder contractility in female patients.ConclusionAlthough currently used formulas such as Pdet Qmax + 5Qmax might be appropriate for male MSA patients, formulas for female patients need further evaluation.

Orbital Adipose Tissue: The Optimal Control for Back-Table Fluorescence Imaging of Orbital Tumors

Control tissue is essential for ensuring the precision of semiquantitative analysis in back-table fluorescence imaging. However, there remains a lack of agreement on the appropriate selection of control tissues. To evaluate the back-table fluorescence imaging performance of different normal tissues and identify the optimal normal tissue, a cohort of 39 patients with orbital tumors were enrolled in the study. Prior to surgery, these patients received indocyanine green (ICG) and following resection, 43 normal control tissues (34 adipose tissues, 3 skin tissues, 3 periosteal tissues, and 3 muscle tissues) were examined using back-table fluorescence imaging. The skin tissue demonstrated significantly elevated fluorescence intensity in comparison to the diseased tissue, whereas the muscle tissue exhibited a broad range and standard deviation of fluorescence signal intensity. Conversely, the adipose and periosteum displayed weak fluorescence signals with a relatively consistent distribution. Additionally, no significant correlations were found between the signal-to-background ratio (SBR) of adipose tissue and patients’ ages, genders, weights, disease duration, tumor origins, dosing of administration of ICG infusion, and the time interval between ICG infusion and surgery. However, a positive correlation was observed between the SBR of adipose tissue and its size, with larger adipose tissues (&gt;1 cm) showing an average SBR 27% higher than smaller adipose tissues (≤1 cm). In conclusion, the findings of this study demonstrated that adipose tissue consistently exhibited homogeneous hypofluorescence during back-table fluorescence imaging, regardless of patient clinical variables or imaging parameters. The size of the adipose tissue was identified as the primary factor influencing its fluorescence imaging characteristics, supporting its utility as an ideal control tissue for back-table fluorescence imaging.