pinecone/core-2020-05-10-deduplication huggingface dataset:
Development and validation of a short form of the valued life activities disability questionnaire for rheumatoid arthritis

Objective  To develop and validate a shortened version of the Valued Life Activities disability and accommodations scale (VLA) for individuals with rheumatoid arthritis (RA).    Methods  To shorten the existing VLA measure, item response theory analyses were conducted using data from 449 patients with RA. Next, the resulting 14‐item shortened version of the VLA scale (S‐VLA) was evaluated by structured interviews among 20 RA patients. Lastly, the S‐VLA was administered to 150 RA patients along with other measures, including the Health Assessment Questionnaire (HAQ) and Short Form 36 (SF‐36). A random sample of 50 patients completed the S‐VLA 2 weeks later to assess reliability. Item statistics were calculated to evaluate correlations between individual items and the S‐VLA total score. Correlations between the S‐VLA and other measures were used to evaluate validity.    Results  Test–retest reliability was 0.91, while Cronbach's alpha for the S‐VLA was 0.95. None of the 14 items was associated with improved alpha coefficients when omitted. All of the items were strongly correlated with the S‐VLA total score. S‐VLA scores were highly positively correlated with the HAQ (r = 0.81,  P  ≤ 0.001), patient‐reported disease activity (r = 0.71,  P  ≤ 0.001), satisfaction with abilities (r = 0.82,  P  ≤ 0.001), and number of days with activity limitations (r = 0.65,  P  ≤ 0.001). In addition, as hypothesized, the S‐VLA was inversely correlated with the SF‐36 physical component summary score (r = −0.78,  P  ≤ 0.001) and the physical functioning (r = −0.80,  P  ≤ 0.001), role physical (r = −0.67,  P  ≤ 0.001), and social functioning (r = −0.72,  P  ≤ 0.001) subscales.    Conclusion  The S‐VLA is a short, valid, and reliable instrument that may prove useful for monitoring disability among individuals with RA
Assessment of health‐related quality of life as an outcome measure in granulomatosis with polyangiitis (Wegener's)

Objective  To assess a generic measure of health‐related quality of life (HRQOL) as an outcome measure in granulomatosis with polyangiitis (Wegener's) (GPA).    Methods  Subjects were participants in the Wegener's Granulomatosis Etanercept Trial (WGET) or the Vasculitis Clinical Research Consortium Longitudinal Study (VCRC‐LS). HRQOL was assessed with the Short Form 36 (SF‐36) health survey that includes physical and mental component summary scores (PCS and MCS, respectively). Disease activity was assessed with the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG).    Results  The data from 180 subjects in the WGET (median followup 2.3 years, mean number of visits 10) and 237 subjects in the VCRC‐LS (median followup 2.0 years, mean number of visits 8) were analyzed. A 1 unit increase in the BVAS/WG corresponded to a 1.15 unit (95% confidence interval [95% CI] 1.02, 1.29) decrease for the PCS and a 0.93 (95% CI 0.78, 1.07) decrease for the MCS in the WGET, and to a 1.16 unit decrease for the PCS (95% CI 0.94, 1.39) and a 0.79 unit decrease for the MCS (95% CI 0.51, 1.39) in the VCRC‐LS. In both arms of the WGET study, SF‐36 measures improved rapidly during the first 6 weeks of treatment followed by gradual improvement among patients achieving sustained remission (0.5 improvement in PCS per 3 months), but worsened slightly (0.03 decrease in PCS every 3 months) among patients not achieving sustained remission ( P  = 0.005).    Conclusion  HRQOL, as measured by the SF‐36, is reduced among patients with GPA. SF‐36 measures are modestly associated with other disease outcomes and discriminate between disease states of importance in GPA
Foot Pain, Impairment and Disability in Patients With Acute Gout Flares; A Prospective Observational Study

Objectives: The aim of this study was to evaluate the impact of acute gout on foot pain, impairment and disability. Methods: This prospective observational study recruited 20 patients with acute gout flares. Patients were recruited from emergency departments, hospital wards and rheumatology outpatient clinics throughout Auckland, New Zealand. Patients were recruited at the time of the flare (baseline visit) and then reassessed at a follow-up visit once the acute flare had resolved 6-8 weeks after the initial assessment. Joint counts, C-reactive protein and serum urate were recorded at both visits. General and foot-specific outcome measures were also recorded at each visit including pain visual analogue scale, Health Assessment Questionnaire (HAQ)-II, Lower Limb Tasks Questionnaire, and the Leeds Foot Impact Scale. Results: The foot was affected by acute gout in 14 (70%) patients. Objective measures of joint inflammation including swollen and tender joint counts and C-reactive protein significantly improved at the follow-up visit, compared with the baseline visit. At baseline, high levels of foot pain, impairment and disability were reported. All patient-reported outcome measures of general and foot-specific musculoskeletal function improved at the follow-up visit compared with the baseline visit. However, pain, impairment and disability scores did not entirely normalise after resolution of the acute gout flare. Conclusions: Patients with acute gout flares experience severe foot pain, impairment and disability. These data provide further support for improved management of gout to prevent the consequences of poorly controlled disease
Work productivity in scleroderma: Analysis from the University of California, Los Angeles scleroderma quality of life study

Objective  To examine the productivity of patients with scleroderma (systemic sclerosis [SSc]) both outside of and within the home in a large observational cohort.    Methods  One hundred sixty‐two patients completed the Work Productivity Survey. Patients indicated whether or not they were employed outside of the home, how many days per month they missed work (employment or household work) due to SSc, and how many days per month productivity was decreased by ≥50%. Patients also completed other patient‐reported outcome measures. We developed binomial regression models to assess the predictors of days missed from work (paid employment or household activities). The covariates included: type of SSc, education, physician and patient global assessments, Health Assessment Questionnaire (HAQ) disability index (DI), Functional Assessment of Chronic Illness Therapy–Fatigue, and Center of Epidemiologic Studies Depression Scale Short Form.    Results  The mean age of patients was 51.8 years and 52% had limited cutaneous SSc. Of the 37% of patients employed outside of the home, patients reported missing 2.6 days per month of work and had 2.5 days per month of productivity reduced by half. Of the 102 patients who were not employed, 39.4% were unable to work due to their SSc. When we assessed patients for household activities (n = 162), patients missed an average of 8 days of housework per month and had productivity reduced by an average of 6 days per month. In the regression models, patients with lower education and poor assessment of overall health by a physician were more likely to miss work outside of the home. Patients with limited cutaneous SSc and high HAQ DI scores were more likely to miss work at home.    Conclusion  SSc has a major impact on productivity at home and at work. Nearly 40% of patients reported disability due to their SSc
Items for developing revised classification criteria in systemic sclerosis: Results of a consensus exercise

Objective  Classification criteria for systemic sclerosis (SSc; scleroderma) are being updated. Our objective was to select a set of items potentially useful for the classification of SSc using consensus procedures, including the Delphi and nominal group techniques (NGT).    Methods  Items were identified through 2 independent consensus exercises performed by the Scleroderma Clinical Trials Consortium and the European League Against Rheumatism Scleroderma Trials and Research Group. The first‐round items from both exercises were collated and redundancies were removed, leaving 168 items. A 3‐round Delphi exercise was performed using a 1–9 scale (where 1 = completely inappropriate and 9 = completely appropriate) and a consensus meeting using NGT was conducted. During the last Delphi round, the items were ranked on a 1–10 scale.    Results  In round 1, 106 experts rated the 168 items. Those with a median score of <4 were removed, resulting in a list of 102 items. In round 2, the items were again rated for appropriateness and subjected to a consensus meeting using NGT by European and North American SSc experts (n = 16), resulting in 23 items. In round 3, SSc experts (n = 26) then individually scored each of the 23 items in a last Delphi round using an appropriateness score ( 1–9 ) and ranking their 10 most appropriate items for the classification of SSc. Presence of skin thickening, SSc‐specific autoantibodies, abnormal nailfold capillary pattern, and Raynaud's phenomenon ranked highest in the final list that also included items indicating internal organ involvement.    Conclusion  The Delphi exercise and NGT resulted in a set of 23 items for the classification of SSc that will be assessed for their discriminative properties in a prospective study
Validation of potential classification criteria for systemic sclerosis

Objective  Classification criteria for systemic sclerosis (SSc; scleroderma) are being updated jointly by the American College of Rheumatology and European League Against Rheumatism. Potential items for classification were reduced to 23 using Delphi and nominal group techniques. We evaluated the face, discriminant, and construct validity of the items to be further studied as potential criteria.    Methods  Face validity was evaluated using the frequency of items in patients sampled from the Canadian Scleroderma Research Group, 1000 Faces of Lupus, and the Pittsburgh, Toronto, Madrid, and Berlin connective tissue disease (CTD) databases. Patients with SSc (n = 783) were compared to 1,071 patients with diseases similar to SSc (mimickers): systemic lupus erythematosus (n = 499), myositis (n = 171), Sjögren's syndrome (n = 95), Raynaud's phenomenon (RP; n = 228), mixed CTD (n = 29), and idiopathic pulmonary arterial hypertension (PAH; n = 49). Discriminant validity was evaluated using odds ratios (ORs). For construct validity, empirical ranking was compared to expert ranking.    Results  Compared to mimickers, patients with SSc were more likely to have skin thickening (OR 427); telangiectasias (OR 91); anti–RNA polymerase III antibody (OR 75); puffy fingers (OR 35); finger flexion contractures (OR 29); tendon/bursal friction rubs (OR 27); anti–topoisomerase I antibody (OR 25); RP (OR 24); fingertip ulcers/pitting scars (OR 19); anticentromere antibody (OR 14); abnormal nailfold capillaries (OR 10); gastroesophageal reflux disease symptoms (OR 8); antinuclear antibody, calcinosis, dysphagia, and esophageal dilation (all OR 6); interstitial lung disease/pulmonary fibrosis (OR 5); and anti–PM‐Scl antibody (OR 2). Reduced carbon monoxide diffusing capacity, PAH, and reduced forced vital capacity had ORs of <2. Renal crisis and digital pulp loss/acroosteolysis did not occur in SSc mimickers (OR not estimated). Empirical and expert ranking were correlated (Spearman's ρ = 0.53,  P  = 0.01).    Conclusion  The candidate items have good face, discriminant, and construct validity. Further item reduction will be evaluated in prospective SSc and mimicker cases
Neighbourhood disadvantage, individual-level socioeconomic position, and self-reported chronic arthritis : a cross-sectional multilevel study

Objective:\ud
To examine the association between individual- and neighborhood-level disadvantage and self-reported arthritis.\ud
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Methods:\ud
We used data from a population-based cross-sectional study conducted in 2007 among 10,757 men and women ages 40–65 years, selected from 200 neighborhoods in Brisbane, Queensland, Australia using a stratified 2-stage cluster design. Data were collected using a mail survey (68.5% response). Neighborhood disadvantage was measured using a census-based composite index, and individual disadvantage was measured using self-reported education, household income, and occupation. Arthritis was indicated by self-report. Data were analyzed using multilevel modeling.\ud
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Results:\ud
The overall rate of self-reported arthritis was 23% (95% confidence interval [95% CI] 22–24). After adjustment for sociodemographic factors, arthritis prevalence was greatest for women (odds ratio [OR] 1.5, 95% CI 1.4–1.7) and in those ages 60–65 years (OR 4.4, 95% CI 3.7–5.2), those with a diploma/associate diploma (OR 1.3, 95% CI 1.1–1.6), those who were permanently unable to work (OR 4.0, 95% CI 3.1–5.3), and those with a household income <$25,999 (OR 2.1, 95% CI 1.7–2.6). Independent of individual-level factors, residents of the most disadvantaged neighborhoods were 42% (OR 1.4, 95% CI 1.2–1.7) more likely than those in the least disadvantaged neighborhoods to self-report arthritis. Cross-level interactions between neighborhood disadvantage and education, occupation, and household income were not significant.\ud
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Conclusion:\ud
Arthritis prevalence is greater in more socially disadvantaged neighborhoods. These are the first multilevel data to examine the relationship between individual- and neighborhood-level disadvantage upon arthritis and have important implications for policy, health promotion, and other intervention strategies designed to reduce the rates of arthritis, indicating that intervention efforts may need to focus on both people and places
Is there a higher genetic load of susceptibility loci in familial ankylosing spondylitis?

<b>Objective</b>\ud
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Several genetic risk variants for ankylosing spondylitis (AS) have been identified in genome-wide association studies. Our objective was to examine whether familial AS cases have a higher genetic load of these susceptibility variants.\ud
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<b>Methods</b>\ud
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Overall, 502 AS patients were examined, consisting of 312 patients who had first-degree relatives (FDRs) with AS (familial) and 190 patients who had no FDRs with AS or spondylarthritis (sporadic). All patients and affected FDRs fulfilled the modified New York criteria for AS. The patients were recruited from 2 US cohorts (the North American Spondylitis Consortium and the Prospective Study of Outcomes in Ankylosing Spondylitis) and from the UK-Oxford cohort. The frequencies of AS susceptibility loci in <i>IL-23R, IL1R2, ANTXR2, ERAP-1</i>, 2 intergenic regions on chromosomes 2p15 and 21q22, and HLA–B27 status as determined by the tag single-nucleotide polymorphism (SNP) rs4349859 were compared between familial and sporadic cases of AS. Association between SNPs and multiplex status was assessed by logistic regression controlling for sibship size.\ud
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<b>Results</b>\ud
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HLA–B27 was significantly more prevalent in familial than sporadic cases of AS (odds ratio 4.44 [95% confidence interval 2.06, 9.55], <i>P</i> = 0.0001). Furthermore, the AS risk allele at chromosome 21q22 intergenic region showed a trend toward higher frequency in the multiplex cases (<i>P</i> = 0.08). The frequency of the other AS risk variants did not differ significantly between familial and sporadic cases, either individually or combined.\ud
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<b>Conclusion</b>\ud
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HLA–B27 is more prevalent in familial than sporadic cases of AS, demonstrating higher familial aggregation of AS in patients with HLA–B27 positivity. The frequency of the recently described non–major histocompatibility complex susceptibility loci is not markedly different between the sporadic and familial cases of AS
Assessment of health‐related family role functioning in systemic lupus erythematosus: Preliminary validation of a new measure

Objective  Individuals with systemic lupus erythematosus (SLE) often experience symptoms that affect family relationships, which are important components of quality of life. To assess the impact of SLE on family role functioning, we developed a 6‐domain (Fatigue, Activity participation, Mental health, Isolation, Love and intimacy, and You/fulfilling family roles [FAMILY]) measure. The objectives of this study were to pilot test and achieve preliminary validation for the SLE‐FAMILY questionnaire.    Methods  This was a 3‐phase study. In phase 1 (development), domains were identified and items were generated for evaluation. During phase 2 (pilot test), a pilot test was conducted to assess the performance of candidate items. In phase 3 (initial validation), 52 individuals with SLE completed questionnaires, including the 6‐item SLE‐FAMILY. Data were analyzed for internal consistency reliability, and validity was assessed using correlations between the SLE‐FAMILY questionnaire and well‐validated measures.    Results  The SLE‐FAMILY had good test–retest reliability (0.82) and internal consistency (0.67). Reliability analysis of individual items revealed weakness in the performance of item 5. We reviewed raw data and determined that 9 individuals likely overlooked the reverse scoring of item 5, thus explaining its poor reliability. When these 9 individuals were excluded from analysis, Cronbach's alpha increased to 0.71, while test–retest reliability remained acceptable (0.75). Spearman's rho correlations supported the validity of the SLE‐FAMILY measure. A pilot test of the SLE‐FAMILY questionnaire without the reverse‐scored item was conducted; results suggested that the modified version is superior to the initial form.    Conclusion  The SLE‐FAMILY questionnaire is a promising new instrument for robust measurement of family role functioning
Dyslipidemia and changes in lipid profiles associated with rheumatoid arthritis and initiation of anti–tumor necrosis factor therapy

Objective  To investigate the frequency of lipid testing in clinical practice and to explore the relationship between rheumatoid arthritis (RA), dyslipidemia, and other cardiovascular (CV) risk factors with RA treatment.    Methods  Patients in this retrospective database study were ages ≥18 years and had ≥2 physician diagnoses for RA or osteoarthritis (OA; comparator group) between March 2004 and March 2008. Outcomes of interest included the percentage of RA and OA patients receiving lipid tests, lipid profiles (total cholesterol, low‐density lipoprotein [LDL] cholesterol, and high‐density lipoprotein [HDL] cholesterol) of RA versus OA patients, and lipid profiles of RA patients before and after initiation with a tumor necrosis factor (TNF) inhibitor. We used multivariable regression to control potential confounders between the cohorts.    Results  Over a median ≥2‐year followup, fewer RA patients than OA patients had ≥1 lipid test (62.0% [95% confidence interval (95% CI) 61.5–62.5] versus 69.8% [95% CI 69.5–70.1]). Mean total cholesterol and LDL cholesterol were each 4 mg/dl lower in the RA cohort ( P  < 0.0001); HDL cholesterol was similar between the cohorts. Across the RA cohort, 25.2% of patients had suboptimal LDL cholesterol levels (≥130 mg/dl). Among RA patients not receiving lipid‐lowering therapy who initiated TNF inhibitor therapy (n = 96), mean total cholesterol and LDL cholesterol increased by 5.4 and 4.0 mg/dl, respectively.    Conclusion  Patients with RA were less likely to be tested for hyperlipidemia and had more favorable lipid profiles than patients with OA. TNF inhibitor therapy modestly increased all lipid parameters. Additional studies are needed to determine the effect of traditional CV risk factors and inflammation and the impact of biologic agents on CV outcomes in RA patients
Long‐term followup for rheumatoid arthritis patients in a multicenter outcomes study of silicone metacarpophalangeal joint arthroplasty

Objective  Rheumatoid arthritis (RA) often results in deformities at the metacarpophalangeal (MCP) joints. Patients with severe deformities can be treated by silicone metacarpophalangeal joint arthroplasty (SMPA). The objective of the study is to prospectively compare long‐term outcomes for an SMPA surgical and a nonsurgical cohort of RA patients.    Methods  A total of 67 surgical and 95 nonsurgical patients with severe subluxation and/or ulnar drift of the fingers at the MCP joints were recruited from 2004–2008 in this multicenter prospective cohort study. Patients could elect to undergo SMPA or not. Outcomes included the Michigan Hand Outcomes Questionnaire (MHQ), Arthritis Impact Measurement Scales 2 (AIMS2), grip/pinch strength, Jebsen‐Taylor Test, ulnar deviation, extensor lag, and arc of motion measurements at the MCP joints.    Results  There was no significant difference in the mean age, race, education, and income at baseline between the 2 groups. Surgical subjects had worse MHQ function and functional measurements at baseline. At 3 years, the mean overall MHQ score and the MHQ function, activities of daily living, aesthetics, and satisfaction scores showed significant improvement in the surgical group compared to the nonsurgical group. Ulnar deviation, extensor lag, and arc of motion in the MCP and proximal interphalangeal joints also improved significantly in the surgical group. No improvement was seen in the mean AIMS2 scores and grip/pinch strength. Complications were minimal with a fracture rate of 9.5%.    Conclusion  RA patients with poor baseline functioning showed long‐term improvement in hand function and appearance following treatment with SMPA compared to nonsurgical controls
Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Objective  Systemic juvenile idiopathic arthritis (JIA) is characterized by fevers, rash, and arthritis, for which interleukin‐1 (IL‐1) and IL‐6 inhibitors appear to be effective treatments. Pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and alveolar proteinosis (AP) have recently been reported with increased frequency in systemic JIA patients. Our aim was to characterize and compare systemic JIA patients with these complications to a larger cohort of systemic JIA patients.    Methods  Systemic JIA patients who developed PAH, ILD, and/or AP were identified through an electronic Listserv and their demographic, systemic JIA, and pulmonary disease characteristics as well as their medication exposure information were collected. Patients with these features were compared to a cohort of systemic JIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry.    Results  The patients (n = 25) were significantly ( P  < 0.05) more likely than the CARRA registry cohort (n = 389) to be female; have more systemic features; and have been exposed to an IL‐1 inhibitor, tocilizumab, corticosteroids, intravenous immunoglobulin, cyclosporine, and cyclophosphamide. Twenty patients (80%) were diagnosed with pulmonary disease after 2004. Twenty patients (80%) had macrophage activation syndrome (MAS) during their disease course and 15 patients (60%) had MAS at pulmonary diagnosis. Sixteen patients had PAH, 5 had AP, and 7 had ILD. Seventeen patients (68%) were taking or recently discontinued (<1 month) a biologic agent at pulmonary symptom onset; 12 patients (48%) were taking anti–IL‐1 therapy (primarily anakinra). Seventeen patients (68%) died at a mean of 10.2 months from the diagnosis of pulmonary complications.    Conclusion  PAH, AP, and ILD are underrecognized complications of systemic JIA that are frequently fatal. These complications may be the result of severe uncontrolled systemic disease activity and may be influenced by medication exposure