tbuckley/cabot-search huggingface dataset:
SAT0620 Further evidences of secondary amyloidosis in alkaptonuria
Authors: B. Frediani, L. Millucci, G. Bernardini, D. Braconi, M. Bardelli, B. Marzocchi, O. Spiga, M. Orlandini, S. Sestini, M. Galeazzi, A. Santucci
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Alkaptonuria (AKU) is an ultra-rare inborn error of metabolism due to a deficient activity of homogentisate 1,2-dioxygenase. Patients suffer from a severe arthropathy. Evidence was provided on the presence of a secondary serum amyloid A (SAA)-based amyloidosis[.<sup>1</sup>Here a complete microscopic and ultrastructural analysis of different AKU tissues, taken from six differently aged patients, is presented. <h3>Objectives</h3> SAA amyloidosis is a complication in AKU[,<sup>2</sup> making the detection of amyloid deposits at an early phase,important for treatment[.<sup>3</sup> We present a study of tissues from patients of different age and relevance of symptoms, providing a detailed overview of AKU amyloidosis. <h3>Methods</h3> Different tissues were obtained from a cohort of 6 AKU patients: 4 M (63,68,42,44 y) and 2 F (66,71 y) with different severity of symptoms. Histology and amyloid were investigated. A complete microscopic and ultrastructural analysis is presented and patient features as radiological examination, mild-to-severe degenerative changes as joint space narrowing, cartilage irregularities, sub-chondral sclerosis or peripheral osteophytes and linear intervertebral disk calcifications were reported. SAAserum levels and other serological markers were measured too.Specimens were analysed by Congo Red, Immunofluorescence, Transmission Electron Microscopy. <h3>Results</h3> The analysis of all AKU specimens confirmed the massive presence of amyloid fibrils even in young patients. Alterations in collagen composition, strictly associated to amyloid bundles deposition, were observed especially in labial salivary gland, cartilage, tendons and infrapatellar fat pad. Histological analysis showed depletion of glycosaminoglycans in young patients, whereas, at light microscopy, calcification and fibrillation were observed only in elderly patients. Immunofluorescence assessed undoubtedly the presence of SAA in amyloid deposits in AKU, and we reported for the first timethe finding of amyloid deposition in young AKU patients and in less common regions. <h3>Conclusions</h3> We provide the first detailed overview of amyloidosis in AKU. Overall, our findings depicted a novel biological framework underlining the pathological role of amyloidosis in several AKU tissues. Furthermore, we found that degradation of extra-cellular matrixin AKU is not limited to elderly.The clinical burden of AKU may notably increase, since amyloidosis was foundeven in young AKU patients, whereas degeneration of cartilage and tendons was limited to older subjects. <h3>References</h3> [1] Millucci L, et al. J Inherit Metab Dis2015Sep;38(5):797–805. [2] Braconi D, et al. Free RadicBiol Med2015Nov;88(Pt A):70–80. [3] Millucci L, et al. DiagnPathol. 2014Sep 26;9:185. <h3>Disclosure of Interest</h3> None declared
SAT0379 Vacuum-assisted closure versus conventional wound care in the management of chronic ulcers in patients with tophaceous gout: a prospective analysis
Authors: X. Pan, Z. Huang, M. Liu, D. Li, Z. Huang, Y. Huang, X. Huang, M. Liu, X. Li, X. Yao, Y. Wang, Q. Huang, X. Guo, S. Zheng, T. Li
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> With the rising epidemic of gout, an increasing number of patients suffer from chronic ulcers associated with tophaceous gout in China, causing poor quality of life and disability. Such ulcers are very difficult conditions to deal with. Vacuum-assisted closure (VAC) has been proved to be effective in treating a variety kinds of wounds such as diabetic foot ulcers. However, its use in chronic ulcers associated with tophaceous gout has been seldom reported. <h3>Objectives</h3> In the present study, we evaluated the use of VAC in the treatment of chronic ulcers associated with tophaceous gout in comparison to conventional wound care (CWC). <h3>Methods</h3> We performed a 12 week prospective study that included 13 patients treated with VAC and 14 patients treated with CWC. We collected the clinical outcomes of these patients and data from a satisfaction survey. Chronic ulcers were treated until wound closure, or until the end of 12 weeks. Study will discontinue when the ulcer worsens or remains unchanged by the end of week 4. <h3>Results</h3> Granulation tissue appeared in 12 (92.31%) patients by the end of week 2 in the VAC group, while it appeared in 6 (42.86%) patients by that time in the CWC group (p=0.013). 100% granulation was achieved in 11 (84.62%) patients by the end of week 8 in the VAC group as compared to 5 (35.71%) patients by that time in the CWC group (p=0.018). By the end of week 12, decreasing in wound size was achieved in 12 (92.31%) patients in the VAC group, while it was achieved in 10 (71.43%) patients in the CWC group (p=0.326). Among them, wound closure was achieved in 9 (69.23%) patients in the VAC group, while it was achieved in 3 (21.43%) patients in the CWC group (p=0.021). None developed local infection in both groups during the treatment. More patients in the VAC group were satisfied with treatment as compared to the CWC group. <h3>Conclusions</h3> Our preliminary study suggests that VAC therapy is effective and safe in treating chronic ulcers associated with tophaceous gout and appears to be superior to CWC therapy. Large-scale studies are needed to further evaluated the efficacy and safety of the VAC therapy in the treatment of chronic ulcers associated with tophaceous gout. <h3>Reference</h3> [1] Agarwal J, Ogilvie ML, Lohman R, et al. Vacuum-assisted closure for sternal wounds: a first-line therapeutic management approach. [J]. Plastic &amp; Reconstructive Surgery2015;116(4):1035–1040. <h3>Acknowledgements</h3> We are grateful to all the participants in our study. <h3>Disclosure of Interest</h3> None declared
AB0332 Comorbidities prevalence and charlson index in a cohort of patients with rheumatoid arthritis
Authors: A.B. Azuaga-Piñango, R. Castellanos-Moreira, S.C. Rodriguez-Garcia, S. Mandelikova, B. Frade-Sosa, A. Diaz-Castillo, N. Sapena, C. Gonzalez-Delaurens, V. Ruiz-Esquide, O. Camacho, A. Cuervo, J. Ramirez, M.V. Hernández, J.A. Gómez Puerta, J.D. Cañete, R. Sanmarti
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The Charlson comorbidity index (CCI) is a prognostic scale, which gives a numerical value that indicates the burden of comorbidities in a patient. This index is obtained from the sum of 19 medical conditions that have been related to mortality and has been validated in several studies. Patients with rheumatoid arthritis (RA) are more at risk than the general population of developing comorbidities. However, these often go unnoticed despite the impact on the disease activity and to treatment response, as shown by different studies such as COMORA. <h3>Objectives</h3> To determine the prevalence of comorbidities in a cohort of patients with RA and estimate CCI. <h3>Methods</h3> Cross-sectional descriptive study, patients diagnosed with RA according to the EULAR/<sup>ACR 2010</sup> classification criteria were included. All patients were followed up in a rheumatology service in a tertiary hospital. Comorbidities were obtained from the medical records. To measure comorbidities, CCI was calculated, the diagnosis of RA was not included in the index. We defined three categories of comorbidity according to CCI: 0 (no comorbidity, applied to patients with no previous record of conditions included in the CCI), 1 to 2 (moderate) and 3 or more (severe). Others comorbidities not included in CCI, such as hypertension (HTN), dyslipidemias (DLP), thyroid disease (TD), osteoporosis (OP) were collected. <h3>Results</h3> 130 patients (103 women) were analysed; mean age was 58.6±12.9 years and disease duration of 6.0±4.4 years. 82.8% were seropositive for rheumatoid factor (n: 83) and/or anti-CCP (n: 97). 44.6% had previous smoking history, 22 were current smokers. The most observed comorbidities in our cohort were: overweight and obesity (BMI ≥25; 63%), DLP (38.8%), HTN (31.5%), chronic kidney disease (32.3%; 6.9% ≥Stage III) and chronic lung disease (23.8%). Other diseases included TD (18.5%), OP (17.7%), diabetes mellitus (9.2%) and liver disease (9.2%). Five patients with a history of tumour (2 metastases) and 2 lymphomas in the last 5 years. Four patients had a heart disease, in 3 as an ischaemic event. According to CCI, 20.8% of the patients had a Charlson 0, 43.8% Charlson 1–2, and 35.4% Charlson ≥3. <h3>Conclusions</h3> In our cohort, despite being a relatively young population, the presence of comorbidities and cardiovascular risk factors is relatively high, in agreement with what has been observed in other studies. 1 out of 3 patients has a severe comorbidity burden. <h3>Disclosure of Interest</h3> None declared
Greediest players in US healthcare “honoured” in awards
Authors: Mary Chris Jaklevic
Journal: BMJ (2019)

A drug company topped a list of the worst actors in US healthcare for the second year in a row, but other industry sectors—such as hospitals, physicians, and insurers—gained ground in the second annual Shkreli Awards.1

“This year we quite intentionally cast a wider net than just the well known and insane greed of the pharmaceutical sector,” said Vikas Saini, president of the Lown Institute, an advocacy group that bestows the awards, in an email. “We wanted to make the point that profiteering has become rampant and is not confined to any one particular sector of the healthcare enterprise.”

Three of the 10 …
Reduced efficacy of low FODMAPs diet in patients with IBS-D carrying sucrase-isomaltase (<i>SI</i>) hypomorphic variants
Authors: Tenghao Zheng, Shanti Eswaran, Amanda L Photenhauer, Juanita L Merchant, William D Chey, Mauro D’Amato
Journal: Gut (2019)

Recently in Gut and elsewhere,1–3 rare and common hypomorphic sucrase-isomaltase ( SI ) gene variants have been linked to an increased risk of IBS. Similar to congenital SI deficiency (a form of carbohydrate malabsorption caused by homozygous SI loss-of-function mutations),4 reduced SI enzymatic activity may trigger IBS symptoms via colonic accumulation of undigested disaccharides from starch and sucrose, resulting in fermentation with gas production and osmotic diarrhoea. This information holds potential for patients’ stratification, and the identification of IBS subgroups better suited to benefit from specific dietary restrictions. For instance, diets low in short chain carbohydrates like the FODMAPs (fermentable oligo-, di-, mono-saccharides and polyols that are poorly absorbed in the small intestine) have been shown to be effective in reducing GI symptoms in some patients with IBS,5 6 though this has not been studied in relation to SI genotype and/or function. While sucrose and, in part, starch are not specifically restricted in a standard low FODMAP diet, such a therapeutic approach may be less effective in individuals whose reduced SI activity contributes …
THU0252 The rate of us verified hip involvement in patients with ankylosing spondylitis
Authors: E. Agafonova, T. Dubinina, A. Dyomina, O. Rumyantseva, S. Erdes
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Hip joint (HJ) involvement is one of the most common extra-axial manifestations of ankylosing spondylitis (AS). <h3>Objectives</h3> To correlate clinical manifestations of coxitis with the ultra-sound findings in AS patients. <h3>Methods</h3> 224 consecutive AS patients (mean age 33,2±14,2 y) meeting 1984 modified N-Y criteria and hospitalised in V.A. Nasonova Research Institute of Rheumatology with hip pain were evaluated. Patients' mean age at the onset of the disease was 26,3±20,3 y., 93% of them were HLA-B27-positive. Median AS duration was 57 [12–444] months, the BASDAI score was 5,7±3,1. The diagnosis of coxitis was based on clinical signs and symptoms, such as inguinal pain and/or limitation in the range of hip motion (ROM) at the time of patients' admission to the clinic. All patients (regardless current complaints) were subjected to ultrasound (US) examination of the HJ (Sono Diagnost 360 (Philips)) using linear (7,5 MHz) and convex (5,0 MHz) transducers. A neck capsular pattern (NCP – the distance between the signals from inner surface of the femoral neck and the joint capsule) more than 7 mm was used as the US criterion, verifying the clinical diagnosis of coxitis. <h3>Results</h3> The US criterion of coxitis was found in 119 (53%) out of 224 patients, and two groups of pts were analysed based on NCP values with 7 mm threshold. <h3>Conclusions</h3> US criterion of coxitis, i..e, increased NCP, was found in 53% of patients with clinical AS manifestations. Patients with US-confirmed coxitis demonstrate more active disease with greater functional impairment and longer history of AS, as compared to AS population without US signs of hip involvement. <h3>Disclosure of Interest</h3> None declared
Stem Cell Transplantation to Treat Multiple Sclerosis
Authors: Harold Atkins
Journal: JAMA (2019)

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SAT0212 The safety and tolerability of intra-articular injection of tolerogenic dendritic cells in patients with rheumatoid arthritis: the preliminary results
Authors: Y. Kurochkina, M. Tikhonova, T. Tyrinova, O. Leplina, A. Sizikov, A. Sulutian, O. Chumasova, A. Ostanin, E. Chernykh
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background:</h3> Dendritic cells (DCs) are known to contribute to the pathogenesis of rheumatoid arthritis (RA) through the presentation of cartilage glycoprotein, production of proinflammatory cytokines and activation of Th1/Th17 responses. Along with stimulating activity, DCs may exhibit suppressive functions via capacity to induce T cell apoptosis/anergy and to generate regulatory T cells. Since these DCs have potential to control autoreactive T-lymphocytes, utilization of tolerogenic DCs seems to be a promising immunotherapeutic toolto treat RA. <h3>Objectives:</h3> The aim of our study is to evaluate the safety and tolerability of a single intra-articular injection (into the knee joint) of autologous monocyte-derived dendritic cells generated in the presence of IFN-α/GM-CSF and tolerized with Dexamethasone in RA patients. <h3>Methods:</h3> DCs were generated by culturing blood monocytes for 5 days with GM-CSF and IFN-α in the presence dexamethasone, applied on third day. Azoximer bromide as maturation stimuli was added on fourth day. Ten RA patients with moderate and high disease activity and ultrasound-defined knee synovitis were recruited in this study. All patients fullfield ACR/EULAR criteria (2010) and received methotrexate, leflunomide, sulfasalazine or their combination. The patients received intra-articular injections of 1*10<sup>6</sup>, 3*10<sup>6</sup>, 5*10<sup>6</sup>,8*10<sup>6</sup>DCs in knee joints. Safety was assessed by evaluation of adverse events (AE). Acceptability was assessed by questionnaire. DAS28 and HAQ were used for assessment of treatment efficiency. This trial registered on clinicaltrials.gov (ID:NCT03337165). <h3>Results:</h3> DCs injections were safe and well tolerated. No one participants showed worsening of symptoms in targeting knee, fever, elevation of blood pressure or other AE within 7 days after injection. After one month the median pain VAS score decreased from 45 (40–65) mm to 40 (15–40) mm; p=0.03. Ultrasound and clinical examination did not detect synovitis. At 3 month no patients demonstrated recurrence of synovitis and median DAS28-ESR improved from baseline 5.1 to 4.4 (p=0.008; n=10). Five patients, evaluated at 6 months follow-up showed a good EULAR response (improvement of DAS28-ESR &gt;1.2). The median DAS28-ESR in this group decreased from 5.1to 3.3 (p=0.04).In addition, we detected median HAQ improvement (from 1.45 to1.0; p=0.04). <h3>Conclusions:</h3> The data obtained suggest that single intra-articular injection of autologous tolerogenic dendritic cells is safe, well tolerated, and according to preliminary data have a potential efficiency. However, the final conclusion should be done after the completion of the trial. <h3>Disclosure of Interest:</h3> None declared
SAT0008 Dysregulation of the circulating and renal clusterin in lupus nephritis
Authors: H.-J. Ma, C. Liu, B.-Y. Shi, L.-Y. Sun
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with lupus nephritis (LN) as one of the main causes of poor prognosis. Although the complement system participates in the development of LN, the exact mechanism is not clear. The present study was to investigate the pathogenesis of LN by detecting the levels of Clusterin (CLU, one of complement regulatory proteins) in the plasma and renal specimens of patients with LN. <h3>Objectives</h3> To determine the circulating and renal Clusterin levels and its clinical associations in patients with LN. <h3>Methods</h3> Plasma Clusterin levels were examined by enzyme-linked immunosorbent assay in 66 SLE including 50 LN patients and 40 healthy controls. Immunohistochemical method was used to detect the expression of Clusterin in renal biopsy tissues of 16 LN patients. Severity of LN was assessed according to the abbreviated version of the International Society of Nephrology/Renal Pathology Society classification. <h3>Results</h3> Plasma levels of Clusterin were significantly decreased in active LN patiens when compared to healthy controls (<i>p</i>&amp;x003C;0.05). Plasma Clusterin levels were negatively correlated with CRP, SLEDAI, and 24 hours proteinuria (<i>p</i>&amp;x003C;0.01,<i> p</i>&amp;x003C;0.05, and <i>p</i>&amp;x003C;0.05). In sixteen patients with lupus nephritis, we found that the expression of Clusterin in glomeruli was significantly enhanced in severe LN when compared to mild LN (figure 1, <i>p</i>&amp;x003C;0.05). <h3>Conclusions</h3> Decreased Clusterin could involve in the pathogenesis of LN, and the role of renal Clusterin need to be further explored. These findings suggested that Clusterin would be a therapeutic target for lupus nephritis in the future. <h3>Reference</h3> [1] Weening JJ, D'Agati VD, Schwartz MM, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol2004Feb;15(2):241–50. <h3>Disclosure of Interest</h3> None declared
Abstract 16732: Sympathomimetic Effects of Acute E-Cigarettes are Attributable to Nicotine, Not Non-Nicotine Constituents
Authors: Roya Moheimani, May Bhetraratana, Kacey Peters, Fen Yin, Jeffrey Gornbein, Jesus A Araujo, Holly Middlekauff
Journal: Circulation (2017)

Background: Electronic cigarettes (EC) release nicotine and non-nicotine constituents. The non-nicotine constituents contain far lower levels of carcinogens than tobacco cigarette smoke. However, ECs generate particulate matter comparable in size and concentration to the levels present in tobacco cigarette smoke - with uncertain cardiac effects. Chronic EC users have increased cardiac sympathetic activity and increased susceptibility to oxidative stress, both risk factors for cardiac disease. Hypothesis: We tested the hypothesis that the nicotine, rather than non-nicotine constituents, in EC emissions produce increased sympathetic tone and oxidative stress in otherwise healthy humans. Methods and Results: Thirty-three healthy volunteers, all non-EC or tobacco cigarette smokers, were studied. On different days separated by a 4-week washout, each participant used an EC with nicotine, an EC without nicotine, or a sham-control. Cardiac sympathetic activity was determined by heart rate variability, and oxidati...
Poly I:C-based rHBVvac therapeutic vaccine eliminates HBV via generation of HBV-specific CD8<sup>+</sup> effector memory T cells
Authors: Hua-Jun Zhao, Qiu-Ju Han, Guan Wang, Ang Lin, Dong-Qing Xu, Ya-Qun Wang, Lian-Hui Zhao, Zhi-Gang Tian, Jian Zhang
Journal: Gut (2019)

Objective Chronic hepatitis B (CHB) virus infection is a global health problem. Finding a cure for CHB remains a challenging task. Design In this study, poly I:C was employed as an adjuvant for HBV therapeutic vaccine (referred to as pHBV-vaccine) and the feasibility and efficiency of pHBV-vaccine in CHB treatment were evaluated in HBV-carrier mice. Results We found that pHBV-vaccine decreased HBsAg and HBV DNA efficiently and safely in HBV-carrier mice. Further investigation showed that pHBV-vaccine promoted maturation and antigen presentation ability of dendritic cells in vivo and in vitro. This vaccine successfully restored the exhaustion of antigen-specific CD8 + T cells and partly broke the immune tolerance established in HBV-carrier mice. pHBV-vaccine also enhanced the proliferation and polyfunctionality of HBV-specific CD11a hi CD8α lo cells. Importantly, we observed that T cell activation molecule KLRG1 was only expressed on HBV specific CD11a hi CD8α lo cells. Furthermore, pHBV-vaccine reduced the expression of Eomes and increased the serum IL-12 levels, which in turn promoted the generation of effector memory short-lived effector cells (SLECs) to exhibit a critical role in HBV clearance. SLECs induced by pHBV-vaccine might play a crucial role in protecting from HBV reinfection. Conclusions Findings from this study provide a new basis for the development of therapeutic pHBV-vaccine, which might be a potential candidate for clinical CHB therapy.