tbuckley/cabot-search huggingface dataset:
AB0319 Mood disorders and chronic rheumatologic diseases: about 200 cases
Authors: W. Hamdi, M. Boudokhane, I. Cherif, K. Maatallah, D. Kaffel, M. Kchir
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Mood disorders are frequently associated to chronic diseases. They are reported in inflammatory diseases as well as in mechanical rheumatologic disorders but they are not systematically recognised and assessed by clinicians. It is necessary to identify the frequency of moods disorders in order to reduce their impact on patient's compliance to treatment. <h3>Objectives</h3> The aim of this study was to assess the impact of chronicrheumatologic diseases on the mood of patients by comparing inflammatory and mechanical diseases and to identify factors correlated with anxiety and depression. <h3>Methods</h3> We conducted a cross-sectional study during 1 year including Tunisian patients with chronic inflammatory rheumatism (rheumatoid arthritis: RA according to the criteria ACR 1987 and axial spondyloarthritis: AS according to modified NEW YORK criteria)and patients with mechanical disorders (chronic low back pain and primitive knee osteoarthritis). Anxiety and depression were assessed by the BAI (Beck anxiety index) and the BDI (Beck depression index), respectively. <h3>Results</h3> We included 120 patients with chronic inflammatory rheumatism (group 1) and 80 patients with mechanical disease (group2). Group 1 was composed of 70 patients with RA and 50 patients with AS including 65 women and 55 men. Their average age was 46.95 [18.75]. Group 2 was composed of 40patients with chronic low back pain and 40 patients with knee osteoarthritis including 48 women and 32 men. Their average age was 51.95 [18.82]. Anxiety was significantly more frequent in group 1 than group 2: 15.52% vs 9.37% (p=0.000). Depression was significantly more noted in group 1 than group 2:16.29%vs 7.16% (p=0.009). The risk factors for anxiety and depression were respectively in the rheumatoid arthritis:tender and swollen joint count, DAS 28, the HAQ and the sharp erosion score. In AS, factors associated to mood disorders were the visual scale of pain, BASFI, BASDAI, ASDAS<sub>ESR</sub> and CRP. In the chronic low back pain mood disorders are associated to functional impairment assessed by the Eiffel questionnaire and the reduced mobility of the lumbar spine assessed by the distance finger-soil. Finally knee in osteoarthritis moods disorders were associated to Lequesne index and the reduction of knee extension. <h3>Conclusions</h3> Patients with chronic rheumatologic diseases suffer very often from anxiety and depression which was related in majority of cases to functional impairment, hence the need for multidisciplinary management. <h3>Disclosure of Interest</h3> None declared
FRI0718-HPR Are pain, activity limitations and quality of life associated with objectively measured physical activity in patients with end-stage osteoarthritis of the hip or knee?
Authors: C. Leichtenberg, F. van Tol, T. Krom, C. Tilbury, H. Horemans, H. Bussmann, R. Nelissen, M. Gademan, T. Vliet Vlieland
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background:</h3> Hip and knee osteoarthritis (OA) are a major cause of disability, yet relatively little is known on the relationship between OA and the total amount of physical activity. <h3>Objectives:</h3> We investigated if OA-associated pain, functional disability and quality of life (QoL) are associated with objectively measured physical activity in patients with end-stage hip/ knee <h3>Methods:</h3> Preoperative data from the Longitudinal Leiden Orthopaedics Outcomes of Osteoarthritis Study (LOAS) were used. All patients were scheduled for primary total hip or knee arthroplasty. Patients wore an accelerometer (Activ8 Remedy Distribution Ltd) which assessed levels of physical activity (LPA) in activity counts and %time spent on physical activity (%PA, i.e. time spent on walking, cycling or running) and sedentary behaviour (%SB, i.e. time spent in lying/sitting). Pain, functional disability and health related QoL were assessed with the Hip Disability and Knee Injury Osteoarthritis Outcome Scores (HOOS/KOOS) and Short-Form 12 (SF12). Multivariate linear regressions models adjusted for confounding were conducted. The physical activity outcomes were transformed to Z-scores. <h3>Results:</h3> 9 hip OA and 48 knee OA patients were included. When awake, mean LPA was 18.787± 7.247 for hip OA patients and 21.193± 6.164 for knee OA patients. In hip OA patients %PA was on average 14±6.4, while %SB was 66±10.5. In knee OA patients these percentages were 15±5.0 and 68±8.7, respectively. In hip OA, the HOOS-QoL and SF12-PCS were positively associated with more LPA, (β0.028; 95%CI:0.007 – 0.048, β0.041; 95%CI:0.010 – 0.071) and better SF12-PCS scores also with PA (β 0.040, 95%CI:0.007 – 0.073). No other associations were found. <h3>Conclusions:</h3> Whereas QoL was associated with physical activity in end-stage hip OA patients, no associations between pain or functional disability and physical activity were observed in either hip or knee OA. <h3>Acknowledgements:</h3> This study was supported by the Dutch Arthritis Association [grant number LLP13]. <h3>Disclosure of Interest:</h3> None declared
FRI0533 Asian mitochondrial dna haplogroup b is associated with the development of knee osteoarthritis in korean
Authors: B.S. Koo, Y. Song, S. Lee, Y.-K. Sung, K.-J. Shin, N.H. Cho, J.-B. Jun
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> In our previous study, we have conducted a case-control study to demonstrate the mitochondrial DNA (mtDNA) haplogroups in the development of knee osteoarthritis (OA). However, there were no mtDNA haplogroups associated with the development of knee OA. <h3>Objectives</h3> The objective of this study was to elucidate the role of mtDNA haplogroups in the development of knee OA in prospective on-going community-based cohort. <h3>Methods</h3> This cohort was established in 2001 to investigate the epidemiologic characteristics of major chronic diseases in Korea by the Korean Genome and Epidemiology Study, Centre for Disease Control (KCDC). The epidemiologic data and knee radiographs were obtained from the second follow-up (2005–2006) and the sixth follow-up (2013–2014), and DNA was distributed from the fourth follow-up (2009–2010). The Kellgren-Lawrence (K/L) score was measured using a knee X-ray taken at each visit. The mtDNA was analysed by multiplex mutagenetically separated polymerase chain reaction to determine the mtDNA haplogroups (M, G, D, D4, D5, M7, M8, M9, M10, N, A, N9, R, F, B). The frequency of the mtDNA haplogroup was compared between the group with knee OA (K/L≥2 or underwent total knee replacement arthroplasty) and the group without knee OA (K/L&lt;2) at the 6th follow-up in the cohort of K/L=0 at the second follow-up. Multiple logistic regression was used to determine relative risk (RR) of mtDNA haplogroups for OA by adjusting sex, age, and body mass index (BMI). <h3>Results</h3> A total of 1115 epidemiological data, knee radiographs, and DNA samples were distributed. Of these, 572 were cohorts with K/L=0 in the second follow-up, and 438 underwent knee X-ray examination at the sixth follow-up visit. Among them, 160 were classified as Knee OA by K/L grading and 278 were classified as control group. The mean age (59.4±8.5 and 64.3±6.8), the number of male patients (61 [21.9%] and 11 [6.9%]), and the mean BMI (24.0±3.1 and 25.0±3.0) were significantly different between normal and OA group (p&lt;0.001). In comparison of frequency of mtDNA haplogroup between two groups, haplogroup B was significantly higher in OA group (unadjusted RR=1.794, p=0.030 and adjusted RR=2.346, p=0.005). <h3>Conclusions</h3> Our data suggested that mtDNA haplogroup B contributed to the development of knee OA in Korean. Further study is ongoing to confirm the relationship between the progression of knee OA and mtDNA haplogroups. <h3>Acknowledgements</h3> Our previous study was presented at 4th International Congress on Controversies in Rheumatology and Autoimmunity, Bologna, Italy, 9–11 March 2017. <h3>Disclosure of Interest</h3> None declared
Activation of the Lysosome-Associated Membrane Protein LAMP5 by DOT1L Serves as a Bodyguard for MLL Fusion Oncoproteins to Evade Degradation in Leukemia
Authors: Wen-Tao Wang, Cai Han, Yu-Meng Sun, Zhen-Hua Chen, Ke Fang, Wei Huang, Lin-Yu Sun, Zhan-Cheng Zeng, Xue-Qun Luo, Yue-Qin Chen
Journal: Clinical Cancer Research (2019)

Despite many attempts to understand mixed-lineage leukemia (MLL leukemia), effective therapies for this disease remain limited. We identified a lysosome-associated membrane protein (LAMP) family member, LAMP5, that is specifically and highly expressed in patients with MLL leukemia. The purpose of the study was to demonstrate the functional relevance and clinical value of LAMP5 in the disease.We first recruited a large cohort of leukemia patients to validate LAMP5 expression and evaluate its clinical value. We then performed in vitro and in vivo experiments to investigate the functional relevance of LAMP5 in MLL leukemia progression or maintenance.LAMP5 was validated as being specifically and highly expressed in patients with MLL leukemia and was associated with a poor outcome. Functional studies showed that LAMP5 is a novel autophagic suppressor and protects MLL fusion proteins from autophagic degradation. Specifically targeting LAMP5 significantly promoted degradation of MLL fusion proteins and inhibited MLL leukemia progression in both an animal model and primary cells. We further revealed that LAMP5 is a direct target of the H3K79 histone methyltransferase DOT1L. Downregulating LAMP5 with a DOT1L inhibitor enhanced the selective autophagic degradation of MLL oncoproteins and extended survival in vivo; this observation was especially significant when combining DOT1L inhibitors with LAMP5 knockdown.This study demonstrates that LAMP5 serves as a "bodyguard" for MLL fusions to evade degradation and is the first to link H3K79 methylation to autophagy regulation, highlighting the potential of LAMP5 as a therapeutic target for MLL leukemia.
AB0704 Clinical-analytical characterisation of 52 diagnosed patients of behÇet disease with inclusion of paediatric cases in a spanish tertiary hospital
Authors: S. Zegarra Mondragon, A. Alia-Jimenez, C. Bouroncle Alaluna, A. Boteanu
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Behçet's disease (BD) is a chronic and recurrent inflammatory disease of unknown etiology, classified into polygenic autoinflammatory diseases or variable vessel vasculitis. It has a wide spectrum of symptoms with a very variable range of severity, from mucocutaneous involvement to neurological manifestations, systemic vascular or severe ocular manifestations. About 5.4%–7.6% of Behçet's cases have a paediatric debut. <h3>Objectives</h3> To evaluate and compare the clinical and laboratory manifestations of a series of 52 patients, adults and children, diagnosed with BD according to the classification criteria of the International Study Group of BD (ISGBD-1990). <h3>Methods</h3> Retrospective cross-sectional observational study, which included 43 adult patients and 9 paediatric patients diagnosed with EB in the Rheumatology Department of a Madrid tertiary hospital. The clinical-analytical characteristics of both groups were evaluated, as well as the correlation of HLA-B51 with the described symptomatology. <h3>Results</h3> The mean age at diagnosis of BD was 36.9±11.8 years in adults and 11.4±5.1 years in children. 27.3% of adults and 11.1% of children with BD were male, with oral ulcers close to 90% in both groups. Contrary to what was reported in other series, genital ulcers were more frequent in children (77.8% versus 65.9% of adults), as was the presence of uveitis (44.4% in children compared to 22.7% in adults) and neurological manifestations (22.2% in children versus 6.8% in adults). Joint involvement was also more frequent in children (88.9% versus 52.3% in adults), as well as fever (44% in children versus 14% in adults); being these two manifestations the only parameters that were associated in a statistically significant way with their presentation in the paediatric age in BD. In contrast, skin involvement and vascular manifestations were more frequent in adults. The positivity of HLA-B51 did not correlate statistically with any clinical manifestation, but those who had it had a mean age at diagnosis of 26.5 years compared to a mean of 39 years in those who did not present this genetic marker. <h3>Conclusions</h3> Behçet's disease presents with a wide spectrum of clinical manifestations, potentially serious, ranging from skin lesions to neurological or vascular manifestations. In our series, patients diagnosed at paediatric age most frequently had systemic manifestations (fever), arthritis or severe clinical manifestations such as neurological involvement or uveitis. Limitations: a small number of paediatric cases included in our study. <h3>Disclosure of Interest</h3> None declared
AB1102 Kawasaki disease and giant aneurysm in mexican children: evolution and clinical characteristics: a 5-year experience
Authors: M.T. Brana, S. Osorio, L. Aparicio, A. Rodriguez, E. Faugier, R. Maldonado
Journal: Annals of the Rheumatic Diseases (2018)

Methods: A cross-sectional study was performed in 288 LAPR based on online survey about cSLE practices, which included 21 countries.All physicians are members of Pan-American League of Association for Rheumatology (PANLAR).Results: The response rate of web-based survey by LAPR was 170/288 (59%) and the majority worked in University Hospitals (63%).The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used.Laboratory exams, diagnostic imaging and biopsies were generally available (>75%), however low availability for densitometry (66%).Drug access was excellent for the most common prescribed medications (>75%), except for belimumab (11%).Endemic illnesses were reported by LAPR in at least one cSLE patient during the previous year: tuberculosis (16%) and Hansen disease (2%).Emerging mosquito-borne diseases were also reported: dengue (20%), Chikungunya (11%) and Zika (8%).Groups were further divided in two, according to the number of cSLE patients followed by LAPR in the last year: group A ( 3 25 patients) and group B (<25 patients).Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (69% vs. 48%, p=0.01).The frequencies of reported pregnancy (50% vs. 16%, p<0.001) and non-adherence to therapy were significantly higher in group A (100% vs. 93%, p=0.023).Alcohol intake (42% vs. 21%, p=0.004) and illicit drug use (19% vs. 5%, p=0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient.Conclusions: This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of University Hospitals.Adherence to therapy, pregnancy and substance abuse were identified as major challenges in this population, particularly in larger centres.
Risankizumab vs. ustekinumab for plaque psoriasis: a critical appraisal
Authors: A. Al‐Janabi, Z.K. Jabbar‐Lopez, C.E.M. Griffiths, Z.Z.N. Yiu
Journal: British Journal of Dermatology (2019)

Gordon et al. investigated the efficacy and safety of risankizumab [an anti-interleukin (IL)-23p19 biologic] compared with ustekinumab (anti-IL-12/23p40) and placebo in patients with moderate-to-severe chronic plaque psoriasis. This was a parallel-group controlled study up to 16 weeks with a planned switch of the placebo group to risankizumab at 16 weeks.This study consisted of two replicate phase III, double-blinded randomized controlled trials (UltIMMa-1 and UltIMMa-2) conducted across 139 centres in Australia, Austria, Belgium, Canada, Czech Republic, France, Germany, Japan, Mexico, Poland, Portugal, South Korea, Spain and the U.S.A.Patients with a minimum 6-month history of chronic plaque psoriasis were randomly assigned to receive either 150 mg risankizumab, 45 mg or 90 mg ustekinumab or placebo. Prior to this, each group was also stratified by weight (either more than or less than 100 kg) and previous exposure to tumour necrosis factor inhibitors. Those assigned to receive placebo were transitioned onto risankizumab at week 16. The study drugs were given at weeks 0, 4, 16, 28 and 40.The severity of psoriasis was measured using the Psoriasis Area and Severity Index (PASI) and a static Physician's Global Assessment (sPGA). The authors additionally recorded the number of adverse events in each treatment arm, and a measure of quality of life.The coprimary outcomes were the proportions of patients achieving ≥ 90% reduction in their baseline PASI (PASI 90) and an sPGA score of 0 or 1 (clear or almost clear) at week 16.In total 506 patients were included in UltIMMa-1 and 491 patients in UltIMMa-2. In UltIMMa-1, PASI 90 by week 16 was achieved by 75·3% of patients receiving risankizumab, compared with 42·0% receiving ustekinumab and 4·9% receiving placebo (P < 0·001 vs. placebo and ustekinumab). sPGA of 0 or 1 by week 16 was achieved by 87·6% of patients receiving risankizumab, compared with 63·0% receiving ustekinumab and 7·8% receiving placebo (P < 0·001 vs. placebo and ustekinumab). The results for UltIMMa-2 are similar. The frequencies of adverse events in the risankizumab, ustekinumab and placebo groups were similar in both studies.Gordon et al. conclude that risankizumab has a higher efficacy than placebo and ustekinumab in the treatment of moderate-to-severe chronic plaque psoriasis, and that the adverse-event profiles were similar across all treatment groups.
FRI0230 Crowned dens syndrome, yet another rheumatic disease imposter
Authors: A. Awisat, M. Rozenbaum, D. Rimar, N. Boulman, L. Kaly, G. Slobodin, N. Jiries, H. Hussien, I. Rosner
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Crowned Dens Syndrome (CDS) is defined as acute cervical or occipital pain, usually associated with elevated acute phase reactants, due to local inflammatory reaction related to calcifications in the ligaments surrounding the odontoid process. Virtually all previous descriptions of CDS have been related to calcium pyrophosphate dehydrate (CPPD) arthropathy. <h3>Objectives</h3> To evaluate patients admitted with acute neck pain and/or headacke for CDS not only in those with CPPD arthropathy but also in patients with other rheumatic diseases <h3>Methods</h3> Twenty four cases of CDS treated in Zion Medical Centre in 2016–2017 were prospectively reviewed. Patients were evaluated according to clinical and laboratory features, background rheumatic disease, response to treatment and invase investigational procedures undertaken. <h3>Results</h3> All patients (age range 54 to 87 years, 67% females) presented with acute onset pain in upper neck and/or occiput accompanied with extreme neck stiffness. Nineteen of 24 patients (79%) had elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Five patients (21%) complained on concomitant severe diffuse headache. Four of those underwent temporal artery biopsy, which was negative for arteritis in all cases, and one was subjected to lumbar puncture, which was non-contributory. The diagnosis of CDS was based on computed tomography imaging in all patients, with precipitations of calcium pyrophosphate dehydrate seen most often in transverse ligament (figure 1) or in alar ligaments. Seventeen patients (71%) had known rheumatic disease on presentation: 10 patients had the diagnosis of CPPD arthropathy, 3 patients had ankylosing spondylitis, 2 patients had rheumatoid arthritis, 1 patient had Behcet's disease and 1 suffered from Familial Mediterranean Fever (table 1). In 4 more patients CDS was the presenting symptom of CPPD disease, diagnosed during hospitalisation. All patients were treated with glucocorticoids as 0.5 mg/kg prednisone plus colchicine 0.5 mg bid resulting in dramatic improvement in both clinical (head/neck pain alleviated and cervical spinal mobility regained) and laboratory measures. <h3>Conclusions</h3> CDS should be considered and craniocervical junction exposed in the context of acute cervical or occipital pain with stiffness and elevated inflammation markers not only in patients previously diagnosed with CPPD, but rather in diverse clinical settings. Particularly, CDS should be recognised as a possible alternative diagnosis in older patients referred with suspicion to giant cell arteritis because of new headache and elevated ESR/CRP. While generally believed to be a rare phenomenon, CDS was seen in 24 patients in 400-bed general hospital within 2 years and is probably widely underdiagnosed. <h3>Disclosure of Interest</h3> None declared
SAT0719-HPR Performance-based physical function measure was more sensitive to detect responders than self-reported measure after a physical therapy exercise intervention in patients with axial spondyloarthritis
Authors: C. Fongen, S.H. Sveaas, A. Bilberg, E. Pedersen, M.W. Johansen, S. van Weely, K.B. Hagen, H. Dagfinrud
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Physical function is regarded as an important outcome in axial spondyloarthritis (axSpA) and the self-reported questionnaire Bath Ankylosing Spondylitis Functional Index (BASFI) is recommended for the evaluation. However, it is shown that BASFI may not be sufficiently sensitive to detect changes after physical therapy. Based on BASFI a disease-specific performance-based measure has been developed; the Ankylosing Spondylitis Performance-based Improvement (ASPI). <h3>Objectives</h3> To compare the proportion of patients with axSpA considered as responders in the performance-based function measure ASPI and the self-reported BASFI after a physical therapy exercise intervention. <h3>Methods</h3> This study was part of the ESpA-study (Exercise for SpondyloArthritis) which examines the effect of 12 weeks, supervised high intensity exercise intervention. Patients with axSpA were included from 4 centres in Scandinavia. Physical function was assessed with ASPI and BASFI at baseline and after 3 months. The ASAS20 response criteria were used to categorise patients as intra-individual responders or non-responders. In BASFI, patients were classified as responders if they showed an improvement of ≥20% and≥1 unit. In ASPI, patients were classified as responders if they showed an improvement of ≥20% on 1 or more subtest(s) and the absence of deterioration on the potential remaining test. Deterioration was defined as a worsening of ≥20% in 1 or more subtest(s). The proportion of patients categorised as intra-individual (non-) responders was examined with Chi square test. <h3>Results</h3> A total of 58 patients (intervention n=30, control n=28) with complete data on ASPI and BASFI were included in the analyses, 41% were male, mean age (SD) was 45 (10.7) years, 55% had radiographic axSpA and mean disease activity (ASDAS) (SD) was 2.6 (0.7). In BASFI, a score of &lt;1 was present in 14% at baseline and in 22% at 3 months, indicating a ceiling effect (figure). The proportion of responders in the total group was 53% in ASPI and 36% in BASFI, p=0.13 (table 1). In the intervention group, 70% were responders in ASPI and 43% were responders in BASFI, p=0.02. <h3>Conclusions</h3> The performance-based physical function measure ASPI was more sensitive to detect responders than the self-reported measure BASFI according to the ASAS20 response criteria in patients with axSpA after a high intensity exercise intervention. Our findings suggest that the performance-based physical function (ASPI) measure is preferable when evaluating physical function after exercise interventions with physical therapy. <h3>Disclosure of Interest</h3> None declared