Higher Disease Duration Research Articles

AB0730 ASSOCIATION OF SPONDYLOARTHRITIS AND FAMILIAL MEDITERRANEAN FEVER AND IMPACT ON DISEASE PHENOTYPE: A SYSTEMATIC REVIEW OF THE LITERATURE

Background:Spondyloarthritis (SpA) and Familial Meditaerranean fever (FMF) may co-exist in certain populations, and have some overlapping manifestations (oligo-arthritis, hip involvement). Their association may impact disease phenotype and may affect disease management.Objectives:To evaluate the association of SpA and FMF and its impact on disease phenotype and management.Methods:A systematic literature search was conducted with the keywords spondyloarthritis and familial mediterranean fever from Janurary 1990 to January 2020 in PubMed and using manual cross-reference methods.Results:The search retrieved 74 articles, out of which 37 articles were relevant to the study question; most of the articles were case reports, with some large cohort studies of FMF and SpA (Flowchart in Figure 1).In large FMF cohorts, the prevalence of SpA was higher compared to the general population (7.5-13%, OR around 10). M694V was a risk factor for SpA. These FMF-SpA patients were older at diagnosis, had lower fever attacks, and higher disease duration, inflammatory back pain, chronic arthritis, enthesopathy, persistent inflammation and higher resistance to Colchicine. In case series, they were responsive to anti-TNF therapy.In large SpA cohorts, MEFV mutation, particularly M694V, was found in 15-35% (even without associated FMF). In most cohorts, MEFV mutation carriers didn’t have any distinct disease phenotype, except for some reports of higher ESR, more hip involvement, higher BASFI and higher BASDAI. Genome-wide association studies and case reports suggest an implication for IL-1 and thus a role for Anakinra therapy in these patients.Conclusion:In FMF or SpA patients with resistance to conventional therapy, the evaluation of disease association should be performed as it may have significant impact on disease management.

The clinical value of the delta finger to palm distance in systemic sclerosis.

Systemic sclerosis (SSc) is a collagen-vascular disorder characterized by fibrosis and vasculopathy. Delta finger to palm distance (delta FTP) is an index measuring the distance between the tip of the third finger to the distal palmar crease in the flexed and extended position. The present study aimed to evaluate the clinical value of delta FTP and to assess the correlation of delta FTP with modified Rodnan skin score (mRSS) and forced vital capacity (FVC) over the 12-month follow-up. This prospective longitudinal study began with 50 participants who were followed for twelve months. Lowess smoothing and linear regression were applied to detect and assess the relationship between delta FTP and mRSS. p-values were adjusted by the Benjamini-Hochberg method (BHM) as a control for false discovery rate. Delta FTP was lower among patients with higher disease duration (p-valueadj: 0.008), diffuse cutaneous SSc (p-valueadj: 0.006), digital ulcers (p-valueadj: 0.003), telangiectasia (p-valueadj: 0.006) and dysphagia (p-valueadj: 0.036). The mRSS has a significant negative linear effect on the delta FTP at the baseline and the end of the follow-up (r: -0.31 and -0.40, respectively). Moreover, changes of mRSS and delta FTP showed a negative linear association over time (r: -0.22). These linear effects remained significant after regrouping the patients based on their SSc subtype. Delta FTP and FVC were not correlated either at the baseline or at the end. It seems that the delta FTP can be a valuable clinical index, supported by its correlated changes with mRSS and other SSc clinical manifestations over the one-year follow-up.

Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study

BackgroundHereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities. In this study, we aimed to assess the effect of the disease across health-related quality of life dimensions, in both carriers of the mutation and patients, to compare health-related quality of life with general population, as well as to explore health-related quality of life prognostic factors among patients, including disease progression and treatment.MethodsThis study was a multi-institutional, longitudinal, prospective, observational study of hereditary Transthyretin Amyloidosis Polyneuropathy Portuguese adult subjects (621 asymptomatic carriers and 733 symptomatic patients) enrolled in the Transthyretin Amyloidosis Outcomes Survey. Health-related quality of life was captured with the preference-based instrument EQ-5D-3 L. For general population the dataset included all subjects enrolled in a representative national study (n = 1500). Different econometric models were specified; multivariate probit, generalized linear model and generalized estimating equations model; including demographic and clinical covariates.ResultsHereditary Transthyretin Amyloidosis Polyneuropathy patients have their health status severely impaired in all quality of life dimensions and more anxiety/depression problems were found among asymptomatic carriers. No differences on utility were found between carriers and general population (p = 0.209). Among patients, the utility value is estimated to be 0.51 (0.021), a decrement of 0.27 as compared with general population utility. Higher disease duration, advanced disease stage and not receiving treatment are associated with impaired health-related quality of life. No differences were found between genders (p = 0.910) or between late (≥50 years) and early-onset patients (p = 0.254). The utility estimate ranged from 0.63 (0.009) in stage I to 0.01 (0.005) in stage IV.ConclusionsHereditary Transthyretin Amyloidosis Polyneuropathy symptoms and progressive associated disabilities substantially decrease patient’s health-related quality of life. Clinical strategies focused on health-related quality of life preservation such as close follow-up of asymptomatic carriers, prompt diagnosis and adequate, early treatment would benefit patient’s long-term outcomes, slowing the progressive decline in health-related quality of life.

Hereditary Ataxias in Cuba: A Nationwide Epidemiological and Clinical Study in 1001 Patients.

The prevalence estimations of hereditary ataxias are biased since most epidemiological studies are confined to isolated geographical regions and few nationwide studies are available. The study aims to assess the prevalence, distribution, and neurological features of the Cuban population with hereditary ataxias. A nationwide epidemiological study of hereditary ataxias was conducted in Cuba between March 2017 and June 2018. Patients were scheduled at the Cuban ataxia research center, various hospitals, or at their homes. Demographic and clinical variables were obtained through standardized questionnaires and validated clinical tools. Overall, 1001 patients were diagnosed with hereditary ataxias for a nationwide prevalence of 8.91 cases/100.000 inhabitants. Spinocerebellar ataxia type 2 (SCA2) was the commonest subtype, with highest prevalences at Holguín province (47.86/100.000), and a broad dissemination in the whole country. Most of neurological features were common between all SCA cohorts, but the frequencies of some of them varied between distinct subtypes. Within the SCA2 cohort, significant influences of long mutation size and higher disease duration over the muscle atrophy and oculomotor disorders were observed. Besides, higher disease durations were associated with resting tremor and dysphagia, whereas shorter disease durations were associated with hyperreflexia. The spreading of SCA2 to whole country and the documented raising of its prevalence set the rationales for higher-scope medical care and research strategies, supported in collaborative research networks. The wide epidemiological, clinical, and genetic characterization of this founder SCA2 population identifies this homogeneous cohort as an attractive source for the development of future clinical-genetic and therapeutic researches.

P397 Surveillance in ulcerative colitis: Can we predict the risk for intraepithelial neoplasia?

Abstract Background Ulcerative colitis (UC) has been associated with an increased risk of colorectal cancer (CRC). Although dye spray chromoendoscopy showed superiority to standard colonoscopy in surveillance studies, with the availability of higher-resolution colonoscopes, the utility of chromoendoscopy (CE) has been questioned. We aimed to evaluate the risk of intraepithelial neoplasia (IN) after a high-quality screening colonoscopy (making use of CE or random biopsies (RB) and removing all detected lesions) in a population with longstanding UC and to identify potential risk factors for dysplasia incidence. Methods In a previous study, 145 patients with clinically and endoscopic longstanding (≥8 yr) distal/extensive UC without primary sclerosing cholangitis and/or history of IN were prospectively randomised to undergo CE or RB. In this study, after a median follow-up of 5 additional years, we evaluated subsequent IN incidence in these patients, submitted to surveillance colonoscopy. Patients without high-quality surveillance colonoscopy (with good bowel preparation and cecum intubation) using high-definition were excluded. Results One hundred and twenty-one patients were included. Nine had removed adenomas with low-grade dysplasia in the index colonoscopy. Now, in surveillance colonoscopy, we detected 9 (7.4%) IN: low-grade dysplasia was found in 8 (6.6%) patients and a colorectal adenocarcinoma in 1 (0.008%) patient. After multivariate analysis, IN was significantly associated with older age (68 vs. 52 years, p < 0.05) and higher disease duration (26 vs. 20 years, p < 0.05). No association was found between IN and previous detection of IN in screening colonoscopy sex, the CE or RB use in index colonoscopy, extent of disease, The presence of pseudopolyps, smoking habits, familial history of CRC or maintenance therapy for UC. Conclusion In this study, older patients and higher disease duration were associated with a higher risk of IN in surveillance colonoscopy.

Longitudinal Trend of Fasting Blood Glucose and Related Factors in Patients with Type 2 Diabetes.

Background:Control of diabetes plays an important role in improving complications and disabilities and quality of life. This study aimed to evaluate the 3-year changes in fasting blood glucose (FBG) values and its related factors in patients with type 2 diabetes.Methods:In this retrospective cohort study, 500 patients with type 2 diabetes covered by the National Diabetic Prevention and Care Plan during 2013–2016 were selected based on random cluster systematic sampling. A linear mixed model was used to study changes in FBG levels and their related factors. The data were analyzed using the R3.2.0 software.Results:The patients' mean age was 47.7 years. Among these patients, 58.6% were female, 19.8% had a history of smoking. High FBG was associated with high disease duration, high body mass index (BMI), low age, normal BMI at baseline, insulin therapy, smoking, and family history of diabetes. Trend of FBG in follow-up was decreasing.Conclusions:Given that patients who received insulin therapy had higher mean FBG, it is recommended to examine their insulin dose and modifications should be made in terms of the patients' needs during their continuous follow-up. Weight loss during follow-up and cessation of smoking indicate a favorable prognosis of disease. More attention should be paid to younger patients in care. Patients are encouraged to start treatment and care at the same time diagnose.

Burden of rheumatoid arthritis on patients\u2019 work productivity and quality of life

BackgroundTo determine the burden of Rheumatoid Arthritis (RA) on patients’ work productivity and health related quality of life (HRQoL), and examine the influence of several exposure variables; to analyze the progression of RA over 1 year and its impact on work productivity and HRQoL.MethodsInternational multicenter prospective survey including patients in 18 centers in Argentina, Brazil, Colombia and Mexico with diagnosis of RA and aged between 21-55 years. The following standard questionnaires were completed at baseline and throughout a 1-year follow-up: WPAI:RA, WALS, WLQ-25, EQ-5D-3 L and SF-36. Clinical and demographic variables were also collected through interview.ResultsThe study enrolled 290 patients on baseline visit. Overall mean scores at baseline visit were: WPAI:RA (presenteeism) = 29.5% (SD = 28.8%); WPAI:RA (absenteeism) = 9.0% (SD = 23.2%); WPAI:RA (absenteeism and presenteeism) = 8.6% (SD = 22.6%); WALS = 9.0 (SD = 6.1); WLQ-25 = 7.0% (SD = 5.1%); SF-36 Physical Scale = 39.1 (SD = 10.3) and Mental Scale = 45.4 (SD = 11.3); EQ-5D-3 L VAS = 69.8 (SD = 20.4) and EQ-5D-3 L index = 0.67 (SD = 0.23). Higher educational levels were associated with better results in WLQ-25, while previous orthopedic surgeries reduced absenteeism results of WPAI:RA and work limitations in WLQ-25. Higher disease duration was associated with decreased HRQoL. Intensification of disease activity was associated with decreased work productivity and HRQoL, except in WLQ-25. In the longitudinal analysis, worsening in disease activity was associated with a decrease in both work productivity and HRQoL.ConclusionsRA patients are dealing with workplace disabilities and limitations and loss in HRQoL, and multiple factors seems to be associated with this. Worsening of disease activity further decreased work productivity and HRQoL, stressing the importance of disease tight control.

Serum RANKL, osteoprotegerin (OPG) and RANKL/OPG ratio in children with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) patients have lower bone mineral density (BMD) compared with healthy individuals because of general, genetic, disease and medication-related factors. The disturbance of the receptor activator of nuclear factor-κB ligand (RANKL)/osteoprotegerin (OPG) ratio has been reported to be associated with low BMD in many disorders in adults and children alike. The objectives of this study were (i) to assess serum OPG, RANKL and RANKL/OPG ratio levels in SLE children and controls, (ii) to determine whether the cumulative glucocorticoid (CGCS) dose had any effect on the concentration of serum RANKL, OPG and RANKL/OPG ratio, and (iii) to determine the relation of these parameters to BMD. We evaluated 50 SLE children and 50 age- and sex-matched healthy controls. RANKL and OPG were assessed in serum and compared between patients and controls. For SLE patients, a univariate followed by multivariable analysis were carried out to detect the possible predictors of the changes in RANKL, OPG and RANKL/OPG ratio levels. Lumbar BMD for all patients was assessed by dual-energy X-ray absorptiometry (DXA) scan and then correlated to different probable correlated factors. RANKL, OPG and RANKL/OPG ratio were significantly higher in SLE patients (p ≤ 0.001). Univariate analysis showed significant correlations of RANKL with CGCS (p ≤ 0.001) and with DXA scan z-score (p = 0.007): OPG was significantly correlated to Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (p = 0.001) and anti-double-stranded DNA (p = 0.001), whereas RANKL/OPG was significantly correlated to duration of illness and DXA z-score (p = 0.002). The multivariable analysis showed that DXA z-score was an independent predictor of RANKL and RANKL/OPG ratio (p = 0.019 and 0.008, respectively), whereas SLEDAI score was an independent predictor of OPG levels. BMD was negatively correlated to disease duration (p = 0.008) and CGCS dose (p = 0.015), but no significant correlation has been found between BMD and cumulative SLEDAI score (p = 0.29). Serum RANKL/OPG ratio is elevated in Egyptian children with SLE and is considered a risk factor for reduced bone mass in these children. Other risk factors for low BMD include high CGCS dose and disease duration, supporting that osteoporosis in SLE is multifactorial.

Study on risk factors of extrapyramidal symptoms induced by antipsychotics and its correlation with symptoms of schizophrenia

BackgroundExtrapyramidal symptoms (EPS) are one of the most common and neglected side effects during the treatment of schizophrenia. The risk factors of EPS in Chinese patients with schizophrenia and its...

Dysmetabolic neuropathy in children with diabetes type I

Neuropathy is the most frequent symptomatic complication of diabetes. Diabetic polyneuropathy (DPN) is the most common variety of neuropathy, which represents chronic symmetrical sensorimotor polyneuropathy. (DPN) typically begins as a generalized asymptomatic dysfunction of peripheral nerve fibers, which may be revealed by electromyography. However, nerve conduction study (NCS) is a sensitive method for early detecting of peripheral neuropathy. We have performed NCS in 69 children with poorly compensated diabetes type I aged 7-18 y. Study protocol included testing of peripheral motor and sensory nerve conduction velocities (NCVs) and compound motor and sensory nerve action potential amplitudes. We revealed subclinical abnormalities, which were symmetric, suggestive of DPN and reflecting disorders of predominantly motor, rather than sensory nerves. In addition, nerve conduction abnormalities were correlated with high HbA1c level, patient age and disease (diabetes) duration. Poor metabolic control was the most important contributor to abnormal electrophysiological parameters.

Diastolic dysfunction in rheumatoid arthritis patients with low disease activity

Introduction/objectivesPatients with rheumatoid arthritis (RA) are at increased risk for congestive heart failure (CHF) and left ventricular diastolic dysfunction (LVDD), as compared to the general population. High disease activity is to be associated with higher incidence of cardiovascular disease (CVD), CHF, and mortality in RA patients. LVDD is not anticipated in RA patients without CVD symptoms and may be underdiagnosed especially in those with low disease activity.MethodThe study group consisted of 70 RA patients (54 women, 16 men) with no CVD and 33 healthy controls, of comparable age. All RA patients had low disease activity (DAS28 ≤ 3.2) from 2 to 7 years. Laboratory and imaging assessments included metabolic, RA-related, and cardiovascular parameters. Echocardiographic and Doppler studies were conducted in patients and controls with assessment of ejection fraction (EF) and diastolic dysfunction (assessed as E/A ratio).ResultsThe mean E/A ratio did not differ significantly between RA patients and healthy controls (1.08 (0.28) vs 0.99 (0.21), NS); comparable numbers of patients and controls had abnormal E/A (< 1.0) (26 (37.1%) vs 10 (30.3%), NS). Patients with decreased E/A were significantly older and had higher disease duration, activity, and presence of bone erosions than their RA counterparts with normal E/A. The mean EF was not significantly different in patients and controls.ConclusionsThe prevalence of DD as expressed by E/A ratio in RA patients with continued low disease activity was not different from that of controls. Higher disease duration and severity may predispose to DD occurrence in patients with preserved EF.

Vision-Related Quality of Life in Iranian Patients With Keratoconus: National Eye Institute Vision Function Questionnaire-25.

Use of patient-reported outcomes to evaluate the impact of ocular conditions on quality of life (QoL) is rising. Hence, the aims of this study were to determine the impact of keratoconus on QoL, and assess the National Eye Institute of the United States Vision Function Questionnaire (NEI-VFQ) performance in an Iranian population. From November 2016 to June 2017, patients with keratoconus definitive diagnosis for more than one year who were being routinely followed at a cornea clinic were recruited. The NEI-VFQ-25 was administered during a face-to-face interview. Ocular examinations comprised best-corrected visual acuity (BCVA) was logged monocularly and binocularly, anterior segment biomicroscopy, refraction, and corneal topography. Keratoconus severity was graded based on steep keratometric (K) reading values. A group of 30 age- and sex-matched subjects with other ocular diseases (except keratoconus) were selected randomly from the cornea clinic. P<0.05 was considered as significant. The mean age of participants was 28.7±7.6 years. The lowest NEI-VFQ subscale scores were related to ocular pain, general vision, mental health, and role difficulty. Almost all NEI-VFQ scores in patients were statistically significant less than mean score of the control group. Composite and all subscales NEI-VFQ scores were lower significantly among patients with higher disease duration (≥5 years). Among clinical data, patients with BCVA ≥0.5 in the better eye had significantly lower NEI-VFQ composite score. Severe keratoconus patients (steep K reading ≥52) had lower NEI-VFQ scores in mental health and dependency subscales (P<0.05). The overall and subscales Cronbach α was above 0.7. Iranian patients with keratoconus had physical, emotional, and social impairment in QoL. The NEI-VFQ-25 might be applicable in further studies.

Slicc damage index score in systemic lupus erythematosus patients and its associated factors.

The aims of this study were to determine damage index in systemic lupus erythematosus (SLE) patients based on Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI) and to determine the laboratory and clinico-demographic factors affecting SDI.This is a retrospective cohort study of 94 SLE patients attending rheumatology clinics in 2 local hospitals in Kelantan, Malaysia. The patients were divided into 2 groups based on SDI score assigned by the attending physician, 0 (without damage) or ≥1 (with damage). Newly diagnosed SLE patients with disease duration less than 6 months were excluded.A total of 45 (47.9%) SLE patients showed damage by SDI score. Majority of the subjects had neuropsychiatric damages (21/94; 22.3%) followed by skin (12/94; 12.8%) and musculoskeletal (6/94; 6.4%) damage. SDI score was significantly associated with higher disease duration (6.2 ± 6.57 years vs 4.5 ± 3.7 years; P = .018), lower prednisolone dose (8.74 ± 10.89 mg vs 4.89 ± 3.81 mg; P < .001), hypertension (P = .007), and exposure to cyclophosphamide (P = .004). Hypertension (P = .020), exposure to cyclophosohamide (P = 0.013), and lower prednisolone dose (P = .023) were significantly associated with damage by multivariable analysis.Higher SDI score was significantly associated with exposure to cyclophosphamide, suggesting that lower cyclophosphamide doses or alternative therapeutic agents are recommended.

Factors associated with health-related quality of life in Peruvian patients with systemic lupus erythematosus.

Objective In this paper, we aim to define factors associated with health-related quality of life (HRQoL) in Mestizo patients with systemic lupus erythematosus (SLE). Methods We evaluated patients with SLE from Peru's two largest hospitals between October 2012 and July 2015 to ascertain HRQoL. Using a standard protocol, we incorporated demographic characteristics, clinical manifestations and treatment in our analysis. HRQoL was measured with the LupusQoL, disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and damage was appraised with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) damage index (SDI). The associations between the LupusQoL and these variables were examined using linear regression models. Model selection was based on backward elimination. Results A total of 277 patients fit the inclusion criterion. Of these, 254 (91.7%) were female, the median (interquartile range, IQR) age at diagnosis was 41.5 (33.8-51.8) years, disease duration was 6.5 (2.7-11.3) years. The HRQoL domains most affected were the following: burden to others, fatigue, and intimate relationships. Through multivariate analysis, we determined that older age at diagnosis, higher disease activity, damage, and immunosuppressive drug use were negatively associated with HRQoL. Further, we found that higher socioeconomic status, disease duration, and antimalarial use were positively associated with HRQoL. Conclusion Age at diagnosis, disease activity, damage, and use of immunosuppressive drugs were negatively associated with HRQoL; high socioeconomic status, disease duration, and use of antimalarials were positively associated with HRQoL.

Cognitive and behavioral comorbidities in Rolandic epilepsy and their relation with default mode network's functional connectivity and organization

ObjectiveRolandic epilepsy (RE) is characterized by typical interictal-electroencephalogram (EEG) patterns mainly localized in centrotemporal and parietooccipital areas. An aberrant intrinsic organization of the default mode network (DMN) due to repeated disturbances from spike-generating areas may be able to account for specific cognitive deficits and behavioral problems in RE. The aim of the present study was to investigate cognitive development (CD) and socioemotional development (SED) in patients with RE during active disease in relation to DMN connectivity and network topology. MethodsIn 10 children with RE and active EEG, CD was assessed using the Wechsler Intelligence Scale for Children-IV (WISC-IV); SED was assessed using the Fünf-Faktoren-Fragebogen für Kinder (FFFK), a Big-Five inventory for the assessment of personality traits in children. Functional connectivity (FC) in the DMN was determined from a 15-minute resting state functional magnetic resonance imaging (fMRI), and network properties were calculated using standard graph-theoretical measures. ResultsMore severe deficits of verbal abilities tended to be associated with an earlier age at epilepsy onset, but were not directly related to the number of seizures and disease duration. Nonetheless, at the network level, disease duration was associated with alterations of the efficiency and centrality of parietal network nodes and midline structures. Particularly, centrality of the left inferior parietal lobe (IPL) was found to be linked with CD. Reduced centrality of the left IPL and alterations supporting a rather segregated processing within DMN's subsystems was associated with a more favorable CD. A more complicated SED was associated with high seizure frequency and long disease duration, and revealed links with a less favorable CD. SignificanceAn impaired CD and – because of their interrelation – SED might be mediated by a common pathomechanism reflected in an aberrant organization, and thus, a potential functional deficit of the DMN. A functional segregation of (left) parietal network nodes from the DMN and a rather segregated processing mode within the DMN might have positive implications/protective value for CD in patients with RE.

The Influence of Income and Formal Education on Damage in Brazilian Patients With Systemic Lupus Erythematosus.

The aim of this study was to evaluate the association of socioeconomic status and American College of Rheumatology/Systemic Lupus International Collaborating Clinics Damage Index (SDI) score in Brazilian patients with systemic lupus erythematosus (SLE). Five hundred twenty-three patients (SLE ACR criteria) 18 years or older who were at 12 months or greater since diagnosis were included. Socioeconomic status was assessed by per-capita income and years of education. Race was categorized as white and nonwhite. The SDI and Mexican SLE Disease Activity Index were used. Mean ± SD and median were used for descriptive analysis. Student t test, Mann-Whitney U test, χ test, and Spearman rank correlation coefficient and univariate and multivariate regression analyses were performed. The level of significance was set at 5% for all statistical tests. Ninety-six percent were female, 51.2% were nonwhite, and the mean age was 37.8 ± 1.4 years. Disease duration was 8.2 ± 10.3 years and formal education was 10.2 ± 3.5 years. Unemployment among patients was 63.7%, with median monthly per-capita income of US $276. Mean SDI score was 1.4 ± 1.52, and 65.6% had some type of damage (SDI ≥1). Patients with SDI of 1 or greater had lower income (P = 0.039). Nonwhite patients had higher SDI than did white patients (P = 0.005). The SDI presented a positive correlation with disease duration (P < 0.001) and age (P < 0.001) and a negative correlation with years of education (P = 0.001). Working patients had lower SDI than did inactive ones (P ≤ 0.001). In a multivariate analysis, older age, higher disease duration, nonwhite race, low income, and out-of-work profile were associated with damage. Besides nonmodifiable characteristics such as longer disease duration and older age, low income was also associated with damage. Therefore, interventions to give adequate socioeconomic support are necessary to improve outcome, mainly in poorer and nonwhite SLE patients.

Notalgia paresthetica: factors associated with its perceived severity, duration, side, and localization.

Notalgia paresthetica (NP) is a disorder characterized by pruritus localized to the patient's back. Little is known about predictors of severity, duration, side, and localization. To identify factors associated with perceived severity, duration, side, and localization of NP. In a cross-sectional study, disease severity, duration, side, and localization were compared among the considered variables. Multivariate analysis was used to assess each variable contribution in predicting disease severity and its duration. Sixty-five patients were recruited. Disease involved more female than male patients (Female/Male: 1.6) and after adjusting for all potential confounders, it was perceived as more severe in females (OR = 7.3, 95% CI = 2-26.3, P < 0.01). NP was reported more frequently on the right side among patients sleeping on the left side and conversely (P < 0.05). A higher disease duration was significantly linked to a higher body mass index (OR = 4.8, 95% CI = 1.3-17.1, P < 0.05). Our study linked female gender to worse disease severity, a higher body mass index to longer disease duration, and prevailing sleep position to NP side expression. The sleeping position should be considered a possible target for treating the disease.

Predictors of the response to etanercept in patients with juvenile idiopathic arthritis without systemic manifestations within 12 months: results of an open-label, prospective study conducted at the National Scientific and Practical Center of Children's Health, Russia

BackgroundThe aim of this study was to investigate the efficacy of etanercept treatment and to identify predictors of response to therapy within 12 months in patients with juvenile idiopathic arthritis (JIA) without systemic manifestations.MethodsA total of 197 juvenile patients were enrolled in this study. Response to therapy was assessed using the ACRPedi 30/50/70/90 criteria, the Wallace criteria, and the Juvenile Arthritis Disease Activity Score 71 (JADAS-71). Univariate and multivariate logistic regression analyses were performed to identify potential baseline factors associated with treatment response in different JIA categories.ResultsOne year after treatment initiation, 179 (90.9%) patients achieved ACRPedi30; 177 (89.8%) patients achieved ACRPedi50; 168 (85.3%) patients achieved ACRPedi70; and 135 (68.5%) patients achieved ACRPedi90 response. A total of 132 (67.0%) and 92 (46.7%) patients achieved inactive disease according to the Wallace criteria and the JADAS-71 cut-off point, respectively. Excellent response (achieving ACRPedi90 and clinically inactive disease according both to the Wallace criteria and the JADAS71 cut-off point) was associated with persistent oligoarticular JIA category, shorter disease duration before the start of etanercept, a lower number of DMARDs used before the introduction of etanercept, a lower number of joints with limited motion, and lower C-reactive protein at baseline. Poor response (failure to achieve ACR 70 or active disease according to both the Wallace criteria and JADAS71 even when ACR 70 was achieved) was associated with the polyarticular or enthesitis-related JIA categories, higher disease duration before the start of etanercept, and older age at disease onset.ConclusionAlmost half (45.7%) of the patients who initiated etanercept treatment achieved an excellent response (inactive disease and ACRPedi90) after 1 year. What may be novel is our finding that the response to etanercept therapy was strongly associated with the JIA category. The response to etanercept therapy was also associated with the disease duration before the start of etanercept treatment.

Knowledge, attitudes and perceptions of Parkinson's disease: A cross-sectional survey of Asian patients

BackgroundA knowledge gap about Parkinson's disease (PD) often exists amongst PD patients, the level and accuracy of which can influence their attitude and perceptions about the disease. Increased awareness of this information deficit can help physicians identify interventions that will improve patient's knowledge and perceptions about PD. ObjectivesTo evaluate general understanding of PD and identify knowledge gaps amongst PD patients using a validated PD knowledge questionnaire. MethodsA cross-sectional, questionnaire-based study was conducted in 108 PD patients (34 working; 74 retired). The questionnaire covers three domains of PD, including diagnosis, therapeutic options, and disease course with a total knowledge score ranging from 0 to 26. Subjects with dementia were excluded. ResultsPatient misconceptions were reflected in the mean (SD) total, diagnosis, therapeutic options, and disease course scores: 14.64 (3.32), 7.76 (1.93), 3.85 (1.28), and 3.03 (1.41) respectively. The working age subgroup had significantly higher knowledge scores than the retired age group for the whole questionnaire (p=0.004), as well as the diagnosis (p=0.001) and therapeutic sections (p=0.023). Three dependent variables, including younger age at recruitment (<60years old), female gender, and higher disease duration (≥4years), were identified as predictors of a higher level of PD knowledge amongst PD patients. ConclusionSignificant knowledge gaps were identified amongst PD patients in all three aspects of the questionnaire. Timely identification of patients with inaccurate or insufficient disease-related knowledge could help healthcare professionals choose more suitable multimodal educational interventions.

Elevated glycated hemoglobin levels impair blood pressure in children and adolescents with type 1 diabetes mellitus.

BackgroundDeregulation of glycemic and glycated hemoglobin (HbA1) levels accelerate the progression of cardiovascular complications in type 1 diabetes mellitus (T1DM). The aim of this study was to investigate the association between HbA1 and changes in blood pressure of children and adolescents with T1DM.MethodsA total of 60 children and adolescents were recruited and allocated into two groups (prehypertension and control group). Blood pressure and HbA1 were measured by the oscillometric method and high-performance liquid chromatography, respectively.ResultsThe prehypertensive group had (P < 0.05) higher disease duration, body weight, Z score for body weight, systolic blood pressure (SBP), diastolic blood pressure (DBP) and a higher HbA1 when compared with the control children and adolescents. Multiple regression to predict alterations in DBP from HbA1 adjusted for age, disease duration, and body mass index demonstrated a positive relationship with DBP (P < 0.05). A 1 % increase in HbA1 was associated with 1.73 mmHg increase in DBP.ConclusionsHigh levels of HbA1 may be associated with increased blood pressure in T1DM. A tight control of HbA1 levels may provide long-term cardiovascular protection in children and adolescents with T1DM.