tbuckley/cabot-search huggingface dataset:
SAT0730-HPR Differences in perception of the disease constraints between the child and parents in jia
Authors: E Ünal, P Kısacık, G Arin, NB Karaca, ED Batu, ZS Arıcı, E Sönmez, Y Bilginer, S Özen
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Studies suggest that the perception of the child and family differ in juvenile idiopathic arthritis. This may pose a problem for the clinician9s assessment (1,2). <h3>Objectives</h3> The aim of this study was to evaluate the differences in perception of the disease constraints between child and parent in JIA. <h3>Methods</h3> 129 children with JIA were included in the study.The main complaint about the illness was requested to be expressed in writing by both the child and the family. Individuals9 expressions and demographic data were recorded. The main complaints were considered nominal. The correlation between the parent9s and child9s expressions were examined. The types of complaints were classified as none, functional, symptomatic, and both functional and symptomatic. <h3>Results</h3> The age range of children ranged from 6 to 21 (mean±SD; 12,86±3,68). When the complaints of the 129 children and the parents were compared, 45,7% were different, 20,2% were partially similar and 34,1% were perfectly similar complaints.When the answers of the 59 children and parents who differ in their complaints were examined; 67.8% of the children expressed a milder disease than their parents (Table1). While the family mostly concentrated on the symptoms, the children were worried on functional complaints (Table1). <h3>Conclusions</h3> As a result of this study, there was a difference between parent9s and child9s perception of the disease. While children report functional complaints, parents focus on symptomatic complaints. Exercise approaches should focus on the child9s functional complaints and identify common goals with the family. Families should be educated about the importance of the complaints that the child expresses. <h3>References</h3> Manczak, M. Rutkowska-Sak, L. Raciborski, F. Health-related quality of life in children with juvenile idiopathic arthritis – child9s and parent9s point of view. Reumatologia 2016; 54(5):243–250. Consolaro, A. Negro, G. Lanni, S. et al. Toward a treat-to-target approach in the management of juvenile idiopathic arthritis. Clin Exp Rheumatol 2012; 30(4 Suppl 73): 157–162. <h3>Acknowledgements</h3> The author declare that they have no conflict of interest. <h3>Disclosure of Interest</h3> None declared
AB0844 Falls: risk and prevention in private rheumatology practice
Authors: L Poulain, V Strotz, X Grapton, P Lemesle
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Menopaused and osteoporotic women have a higher risk of fractures when falling. Fall prevention is important when taking care of those women. <h3>Objectives</h3> After identifying fall risk factors, defining the % of fractured fallers and the part of osteoporotic women in this population, we want to evaluate the relevance of balance tests and to favor prevention measures for those patients. <h3>Methods</h3> 110 patients, 60 years and older (including 24 controls), having falled within a year, have seen 28 private practice rheumatologists in the larger Paris area and were subject of our multicentre retrospective study. <h3>Results</h3> Fallers mean age was 75 years. 37% of the fallers within a year fractured after falling. Among them, 95% were post-menopausal fractures versus 64% when including the control group. Post-fall or post menopausal fracture sites are identical. In order: spine 26%, wrist 18%, ribs 6%. The circumstances of the falls are: 1) lack of attention 2) slipping 3) stumbling. 61% of the fallers have at least one physical activity, among them 71% a weight-bearing physical activity. The most common physical activity was walking (46%). Among the fractured, 32% had physical activity, 42% hadn9t. 72% of the fallers had at least one fall risk factor: 94% had intrinsic risk factors, 28% extrinsic, and an average of 2 risk factors within the older than 80 years. Intrinsic factors: 1) osteo-articular 2) eye-vision 3) postural 4) drugs, out of 13 items. Extrinsic: 1) obstacles 2) footwear out of 7 items. 45% of the fallers within a year had at least one osteoporosis risk factor: 1) low body mass index 2) cortico-steroids 3) early menopausis 4) smoking, out of 9 items. 70% of our patients had vitamin D level&gt;30 ng/l (as recommanded) 69% of the patients had recent bone density measures: bone density was lower only at femoral neck of fractured fallers versus non fractured fallers. The unipodal balance test (positive when standing less than 5 seconds) was relevant in 42% of the patients with or without fractures. Get up and go test was not relevant in our study population. 94% of the rheumatologists participating in the study recommend at least 2 fall prevention measures: 1) correct vitamin D levels 2) physical activity 3) reeducation 4) eye vision correction, out of 12 items. <h3>Conclusions</h3> A significant part of menopaused women with anteriority of falls and fractures will refracture after falling again. Beside treating osteoporosis, we should identify potential fallers by doing unipodal tests, consider and correct fall risk factors, encourage those patients to keep on physical activity and prescribe reeducation. <h3>Disclosure of Interest</h3> None declared
Cholangioscopy-assisted guidewire placement in post-liver transplant anastomotic biliary stricture: efficient and potentially also cost-effective
Authors: Fernanda Martins, Angelo Ferrari
Journal: Endoscopy (2017)

Endoscopy is the first-line therapy for most post-liver transplantation anastomotic biliary strictures. Passage of a guidewire through the stricture is done under fluoroscopic guidance. Although anastomotic biliary strictures are usually short, they can be tight, fibrotic, and angulated, sometimes not allowing guidewire passage. Additional interventions (percutaneous transhepatic cholangiography [PTC], or endoscopic ultrasound-guided biliary drainage [EUS-BD]) can be necessary to avoid re-operation.
THU0402 Ultrasonography and dual-energy CT (DECT) do not provide the same quantification of urate deposition in gout: results from a cross-sectional study
Authors: T Pascart, A Grandjean, L Norberciak, V Ducoulombier, M Motte, H Luraschi, M Vandecandelaere, C Godart, E Houvenagel, N Namane, J-F Budzik
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Gout is due to monosodium urate (MSU) deposition in joints and soft tissues. Ultrasonography (US) and dual-energy CT (DECT) have been shown to be effective in detecting MSU deposits. Both techniques can examine tophi size. DECT is effective to identify soft-tissue MSU deposits and US can show joint deposition with the double contour (DC) sign. It is unknown if these two techniques provide the same quantification of the extent of urate deposition on a given patient. <h3>Objectives</h3> The main objective of this study is to compare the tophus size measured by US and by DECT. The secondary objective is to evaluate the correlation between the prevalence of the US DC sign and the global volume of urate deposits measured by DECT. <h3>Methods</h3> This prospective cross-sectional study included patients fulfilling the 2015 ACR/EUALR criteria for gout. Patients underwent US and DECT examinations of their knees and feet. The largest US tophi was selected as the index tophus. US examination of the DC sign was performed on the femoro-patellar joints, talo-crural joints and 1st metatarsophalangeal joints. Total volume of urate deposits of knees and feet was measured by DECT. The primary endpoint was the intra-class correlation coefficient (ICC) of the volume of the index tophus measured by US and DECT [CI 95%]. <h3>Results</h3> A total of 64 patients were included in the study, of which 35 patients presented with at least one US tophus. Patients were in average 64.5±16.3 years old, 84.4% were male, had an average ACR/EULAR score of 13.6±2.5, and disease duration was 12±14.7 years. Overall, 44 patients (68.8%) were currently taking urate lowering therapy and 22 patients (34.4%) had clinical tophi. Out of the 35 US selected largest tophi, 6 tophi were not seen in DECT. Of the tophi identified with both techniques, 21 were localized in the feet and 8 in the knees. The ICC of the tophus volume assessment by US and DECT was 0.45 [0.12–0.69]. The average volume of the largest US tophi was 2.7±6.5 cm<sup>3</sup> and 1.5±3.3cm<sup>3</sup> when measured by DECT. If the index tophus was localized in the knee, the ICC was 0.36 [0–0.82] and was 0.68 [0.37–0.86] if the tophus was in the foot. The Spearman correlation coefficient between the DECT urate volume and the number of joints with a positive DC sign was 0.15. <h3>Conclusions</h3> US and DECT do not provide the same assessment of tophus size. The correlation is improved when considering tophi localized in the feet. The number of joints with positive DC sign does not correlate to the volume of urate deposition in the soft tissues measured by DECT. <h3>Disclosure of Interest</h3> None declared
THU0328 Predictors of severe damage in anca-associated vasculitis: data from a monocentric inception cohort
Authors: M Felicetti, R Padoan, M Cerchiaro, M Zen, L Punzi, F Schiavon
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are multi-systemic diseases associated with anti-neutrophil cytoplasmic antibodies (ANCA). Patients develop severe and irreversible damage, even in early stages of the disease, but data about prognostic factors are limited. <h3>Objectives</h3> To assess items and predictors of severe damage in a monocentric cohort of ANCA-associated vasculitis (AAV) patients, classified as GPA or MPA. <h3>Methods</h3> Clinical and serological data of patients, followed-up in a daily practice setting, were retrospectively revised. Severe damage, defined as a Vasculitis Damage Index (VDI) <sup>3</sup> 5, was assessed after 12 months and at last examination (LE). The five most important items, predictors of severe damage and their increase rate were analysed at the beginning and at the last examination. <h3>Results</h3> 69 patients were included, 53 (76.8%) classified as GPA and 16 (23.2%) as MPA. According to EUVAS classification 7 (10.1%) were affected by limited disease, 14 (20.3%) by early systemic disease, 35 (50.7%) by generalized disease and 13 (18.9%) by severe disease. Patients were middle aged at diagnosis (54.6±15 years) with a diagnostic latency of 9.6±16.4 months, mostly caucasian (98.6%) and equally represented in sex (female 53.6%). Mean follow-up was 54.5±41.9 months. The number of flare/year at LE was significantly higher in anti-PR3 patients if compared to anti-MPO (1.0±1.1 vs 0.3±0.5, p=0.028). The VDI items most frequently observed are listed in Figure 1. We measured their frequencies and increase rate from diagnosis to the LE: arterial hypertension increased from 3 (4.7%) to 41 (65.1%) (+60.3% (IC 95% 48–71%), p&lt;0.0001), GFR&lt;50% from 1 patient (1.6%) to 28 (44.4%) (+42.8 (IC 95: 31–55), p&lt;0.0001), neuropathy from 4 (6.3%) to 22 (34.9%) (+28.6% (IC 18–41), p&lt;0.0001), diabetes from 0 (0%) to 11 (17.5%) (+17.5% (IC 10–29); p=0.0011) and proteinuria&gt;0.5 g 24 h from 1 (1.6%) to 13 (20.6%) (+19% (IC 11–31); p=0.0006). Severe damage, defined as VDI≥5, increased over time, being present in none patient at diagnosis and in 14 (25.9%) at last examination. At univariate analysis, number of flares/year (p=0.012), BVASv3 at diagnosis (p=0.03), steroid pulses therapy (p=0.04) and steroid cumulative dose (p=0.03) were identified as predictive factors of severe damage in the first year. Conversely, creatinine at LE (p=0.04), numbers of hospitalizations (p=0.009) and steroid cumulative dose at LE (p=0.47) were found to be predictors of long-term severe damage. Interestingly, ANCA pattern did not influence damage severity and only slightly differences in VDI items was noted between anti-PR3 and anti-MPO positive patients. <h3>Conclusions</h3> Our results highlights that knowing severe damage predictors is key in short and long term treat to target patient9s management. <h3>Disclosure of Interest</h3> None declared
SAT0326 The asscociation of serum type 1 interferon activity and autoantibodies in inflammatory myositis
Authors: A Maruyama, N Ono, M Sakai, Y Sadanaga, S Koarada, Y Tada
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Recent reports had shown that most of clinically amyopathic dermatomyositis (CADM), which showed poor prognosis, was positive for anti-Melanoma differentiation-associated gene 5 (MDA5) antibody (Ab). It had been shown that patients not only with lupus but with dermatomyositis (DM) also showed increased type 1 interferon (IFN) signature. MDA5 acts as a cytosolic RNA sensor, which drives type 1 IFN production. These facts suggested that type 1 IFN might have some roles in anti-MDA5 Ab positive patients. <h3>Objectives</h3> We evaluated the association of serum type 1 IFN signature and autoantibodies in patients with inflammatory myositis, in particular anti-MDA5 Ab positive patients and anti-aminoacyl-tRNA synthetases (ARS) Ab positive patients. <h3>Methods</h3> Sera from 33 inflammatory myositis patients (13 DM, 10 PM and 10 CADM) were studied for type 1 IFN activity, using a functional reporter cell assay. Briefly WISH cells were incubated with serum containing media for 6 hours. Serum IFN signature scores of the incubated cells were evaluated by the sums of gene expressions of Mx1, IFIT3, IFI44L and IFI44 by real time PCR (Reference). Anti-MDA5 Ab and Anti-ARS Ab were measured by ELISA. We divided these patients into three groups, anti-MDA5 Ab positive group (MDA5 group), anti-ARS Ab positive group (ARS group) and double negative group (DN group). We included double positive patients into MDA5 group. The presence of interstitial lung disease (ILD) and the prognosis were also investigated. <h3>Results</h3> MDA5 group had 12 patients (8 CADM and 4 DM), ARS group had 8 patients (4 DM, 1 CADM and 3 PM), and DN group had 13 patients (5 DM, 1 CADM and 7 PM). 9 of MDA5 group, 5 of ARS group, and 1 of DN group were complicated with ILD. Serum IFN signature scores of MDA5 group were significant higher than those of ARS group and DN group (12.43 1.406, 2.407, p=0.0005). The most of ARS group showed low serum IFN signature activities. The overall survivals of ARS group were fairer than MDA5 group, but not significantly. The deceased cases of MDA5 group showed especially high serum IFN signature activities. <h3>Conclusions</h3> We characterized two major groups in inflammatory myositis patients. ARS group was characterized by low IFN signature with the susceptibility to DM and ILD. MDA5 group was characterized by high serum IFN signature with the high susceptibility to CADM. Our results suggest that these two entities may have different onset mechanisms, leading to different outcomes. <h3>References</h3> Niewold TB, Rivera TL, Buyon JP, Crow MK. Serum type I interferon activity is dependent on maternal diagnosis in anti-SSA/Ro-positive mothers of children with neonatal lupus. Arthritis Rheum. 2008 Feb;58(2):541–6. <h3>Disclosure of Interest</h3> None declared
Srebp-controlled glucose metabolism is essential for NK cell functional responses
Authors: Nadine Assmann, Katie L O'Brien, Raymond P Donnelly, Lydia Dyck, Vanessa Zaiatz-Bittencourt, Róisín M Loftus, Paul Heinrich, Peter J Oefner, Lydia Lynch, Clair M Gardiner, Katja Dettmer, David K Finlay
Journal: Nature Immunology (2017)
FRI0164 Differences between ra patients with and without ild from a united states tertiary referral center
Authors: R Meehan, J Solomon, J Crooks, D Muram, E Hoffman, P Zelarney
Journal: Annals of the Rheumatic Diseases (2017)

Background: Rheumatoid arthritis (RA) and periodontitis (PD) are common chronic inflammatory diseases with remarkable pathological and clinical similarities.A lot of similarities exist between RA and PD at cellular and molecular levels.Objectives: To analyse the PD incidence among RA patients, measure the level of tumor necrosis factor-alpha (TNF-α), and in their serum and gingival crevicular fluid (GCF), and to investigate their relationship.Methods: The experimental group was composed of 350 patients with RA and the control group consisted of 426 age and gender matched healthy individuals,check and record periodontal condition by dentist,analyse the PD incidence of the two groups,then select 64 PD patients without systematic diseases and 47 PD patients with a stable condition of RA,Detect and compare their periodontal status and the level s of TNF-α, IL-1β and IL-6 in their serum and GCF.Results: The percentage of PD was 67.7%,which was statistically significant higher than control group (43.6%) (P<0.001).The periodontal disease index (PDI),Probing depth (PD)and Clinical attachment loss (CAL) in patients with PD and RA are all significantly higher than those with simple PD (P<0.05).But the bleeding on probing (BOP) in the two groups was not statistically significant (P>0.05).Meanwhile, the level of TNF-α, IL-1β and IL-6 in serum and GCF are significantly higher in all PD patients than oral healthy individuals (P<0.01).Although those inflammatory mediators in serum are much higher in patients with PD and RA than in those with simple PD (P<0.01),there is no difference in the two group about those inflammatory mediators in GCF.The serum level of TNF-α,IL-1β and IL-6 in patients with PD and RA are positively correlated with the corresponding inflammatory mediators in their GCF (r value was 0.531,0.422and 0.770 respectively,p<0.01).Conclusions: Individuals with RA are more likely to experience periodontitis compares to healthy subjects, and the periodontitis is much more serious in patients with RA and PD than those with simple PD. the serum and GCF inflammatory factors' level increased significantly in patients with RA and PD,even during their RA stable period.The serum level of inflammatory factors in patients with PD and RA are positively correlated with the corresponding inflammatory mediators in their GCF.There is correlation between periodontitis and RA,and they may be the risk factors for eachother, the improvement of the periodontal status in patients with RA and PD may help to control systemic inflammation level and to make comprehensive treatment measure for RA patients.
Intraocular Infection with a Trematode
Authors: Pablo J Guzman-Salas, Juan C Serna-Ojeda
Journal: New England Journal of Medicine (2017)

A 17-year-old boy from a rural town in Mexico presented with decreased visual acuity and pain in his right eye. Slit-lamp examination, shown in a video, revealed an intraocular infection with a trematode.
SAT0497 What are the patients' experience, needs and expectations in hand osteoarthritis? a qualitative evaluation
Authors: M-A Offroy, A Frazier-Mironer
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> The objective of this study was to evaluate the patients9 experience and needs with HOA, in order to establish a therapeutic education program for HOA patients. <h3>Objectives</h3> As the subject was about descriptive, not quantifiable elements, qualitative methodology was chosen. Patients were submitted to individual semi-directive interviews. Verbatim were analyzed following the grounded theory until saturation of data. <h3>Methods</h3> Twelve HOA patients accepted to participate to the study. There were 10 women and 2 men, aged 45 to 79 years. Body-mass indexes varied from 18.7 to 31.6 kg/m<sup>2</sup>. Clinical and radiological severity of HOA varied among patients. They provided data on the experience of HOA, which is influenced by clinical and functional signs and the evolution of the disease. Pain and deformity are the main clinical signs, and lead to severe functional impairment. The functional, psychological, social consequences of HOA also have an impact on the patient experience. Patients develop adaptive strategies, mainly recourse to medical management, and pharmacological and non-pharmacological therapies. The needs of HOA patients were also explored, and three main ideas emerged. First, they want to be better informed on HOA. Second, they have a feeling of failure of conventional medicine, and often use alternative medicines. Third, the fear of disability with the course of the disease is very strong. They have difficulty accepting pharmacological treatments, but often do not realize the therapeutic nature of non-pharmacological treatments. <h3>Results</h3> The main concerns of HOA patients are: information, non-pharmacological treatments and evolutionary risks. These themes should be included in the development of therapeutic education programs for HOA. <h3>References</h3> We thank Pr François Rannou, Pr Pascal Richette and Pr Eric Roulot for providing access to HOA consultation in their centers for recruitment. We thank Dr Laurence Baumann-Coblenz for her advice in qualitative methodology. <h3>Acknowledgements</h3> We thank Pr François Rannou, Pr Pascal Richette and Pr Eric Roulot for providing access to HOA consultation in their centers for recruitment. We thank Dr Laurence Baumann-Coblenz for her advice in qualitative methodology. <h3>Disclosure of Interest</h3> None declared
Sudden Flank Pain in a Patient Receiving Ibrutinib for Mantle Cell Lymphoma
Authors: Yu Xi Terence Law, Lui Shiong Lee
Journal: JAMA Oncology (2017)

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Clinical presentation and outcomes in light chain amyloidosis patients with non-evaluable serum free light chains
Authors: S Sidana, N Tandon, A Dispenzieri, M A Gertz, F K Buadi, M Q Lacy, D Dingli, A L Fonder, S R Hayman, M A Hobbs, W I Gonsalves, Y L Hwa, P Kapoor, R A Kyle, N Leung, R S Go, J A Lust, S J Russell, S R Zeldenrust, S V Rajkumar, S K Kumar
Journal: Leukemia (2017)