tbuckley/cabot-search huggingface dataset:
AB0676 Evolution over Thirty Years of The Profile of Inpatients with Reactive Arthritis in A Tertiary Rheumatology Unit:
Authors: A. Brinster, X. Guillot, C. Prati, D. Wendling
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Reactive arthritis (ReA) are sterile arthritis occurring after extra articular bacterial infection, mainly located in gut or genito urethral mucosa <h3>Objectives</h3> The aim of this study was to analyze, over 30 years, frequency as well as clinico-biological and therapeutic characteristics of ReA, comparing two periods. <h3>Methods</h3> In this retrospective monocentric study, the charts of all the patients followed in our unit between January 1st 1984 and April 2014 with the diagnosis or ReA, according to International Classification Criteria (1), were recorded and clinic biological features, management and outcome were analyzed, and compared between two periods: from January 1984 to December 1993, and from January 2004 to December 2013. <h3>Results</h3> 62 patients fulfilling international diagnosis criteria were analyzed. We found no significant differences (Table) between the two periods in incidence of new cases, clinical presentation (rheumatologic and extra articular features), biological and microbiological data or outcome. Change in therapeutic management was obvious with occurrence of anti TNF use in the recent period. <h3>Conclusions</h3> Reactive arthritis is still a current rheumatologic problem, with an apparently stable incidence in a developed country, with a need of early and tailored rheumatologic management. <h3>References</h3> Kingsley G, Sieper J. Third International Workshop on Reactive Arthritis. 23–26 September 1995, Berlin, Germany. Report and abstracts. Ann Rheum Dis. 1996;55:564–84. <h3>Disclosure of Interest</h3> None declared
The in Vitro and In Vivo Oncogenic Activity of Homodimeric Mutant of Interleukin-7 Receptor a Chain (IL7Rα) Highlights the Significance of the IL7Rα/Jak1 Pathway in T-Cell Acute Lymphoblastic Leukemia
Authors: Kazuaki Yokoyama, Nozomi Yokoyama, Kiyoko Izawa, Ai Kotani, Ratanakanit Harnprasopwat, Akira Harashima, Seiichiro Kobayashi, Arinobu Tojo
Journal: Blood (2011)
Smoldering Multiple Myeloma (SMM) at High-Risk of Progression to Symptomatic Disease: A Phase III, Randomized, Multicenter Trial Based On Lenalidomide-Dexamethasone (Len-Dex) as Induction Therapy Followed by Maintenance Therapy with Len Alone Vs No Treatment
Authors: María-Victoria Mateos, Lucía López-Corral, Miguel Hernández, Pilar Giraldo, Javier De La Rubia, Felipe de Arriba, Laura Rosiñol, Juan-Jose Lahuerta, Luis Palomera, Joan Bargay, Albert Oriol, Felipe Prosper, Jose Garcia-Laraña, Eduardo Olavarría, Maria Luz Martino, Ana-Isabel Teruel, Jose Hernández, Graça Esteves, Jose Mario Mariz, Fernando Leal-da-Costa, Adrian Alegre, Jose-Luis Guzman, Ana López de la Guía, Nuria Quintana, Jose Baquero, Jose Luis García, Jesús F. San Miguel
Journal: Blood (2010)
Pott's disease: a major issue for an unaccompanied refugee minor
Authors: Cihan Papan, Ulrich von Both, Matthias Kappler, Birgit Kammer, Matthias Griese, Johannes Huebner
Journal: Thorax (2016)

The incidence of TB in children in Germany has been on a rise since 2008, especially among foreign-born individuals. With rapidly increasing numbers of refugees from the numerous areas of conflict, this increase in incidence is not expected to halt, neither in Germany nor in Europe in general. We report a case of insufficient tracking in a 16-year-old unaccompanied refugee minor from Somalia who had a positive interferon γ release assay on arrival in Germany. No actions were undertaken, until 6 months later, an X-ray showed prominent hilar enlargement. Nine months later, the patient presented to our hospital with abdominal pain, vomiting and B symptoms. Workup revealed a paravertebral abscess due to Pott9s disease, a skeletal manifestation of <i>Mycobacterium tuberculosis</i> disease. The patient made a full recovery after a combination therapy for a total of 9 months.
SP0079 When and How To Manage The Secondary Raynaud's Phenomenon and Related Conditions
Authors: A.L. Herrick
Journal: Annals of the Rheumatic Diseases (2016)

When Raynaud9s phenomenon (RP) occurs secondary to an underlying condition (most importantly to rheumatologists to connective tissue disease), it can be very severe and progress to tissue injury, for example digital ulceration.  Most work on secondary RP has been in systemic sclerosis (SSc)-related RP and this will be the focus of this lecture, in order to exemplify the key principles of management. The main aims are to reduce symptoms and to prevent progression to digital ulceration and/or critical ischaemia. The first step in management is to establish the diagnosis, and decide whether there are any aggravating or triggering factors (e.g. smoking) amenable to intervention. Non-drug therapy, drug therapy and surgical options will all be discussed, with a focus on recent advances. Although calcium channel blockers remain first-line drug therapy for RP, phosphodiesterase inhibitors are being increasingly used. In those patients who progress to digital ulceration and/or critical ischaemia, intravenous prostanoids and (for patients with recurrent digital ulcers) endothelin receptor antagonism should be considered. For those patients refractory to medical management, surgical debridement of ulcers and/or digital sympathectomy may be beneficial. Botulinum toxin (Botox) injections are given in some centres, but further research is required to establish their efficacy. Studies are ongoing investigating new approaches to therapy.  Upon completion of the session, participants should be able to:  1. Describe the general approach to management of secondary RP, including of digital ulceration and critical ischaemia.  2. Discuss the indications for “advanced therapies” (intravenous prostanoids, endothelin receptor antagonism, surgery).  Disclosure of Interest  A. L. Herrick Grant/research support from: Actelion, Consultant for: Actelion and Apricus
SAT0545 The Effect of anti-TNF Alpha Treatment on Erosion Progression in Rheumatoid Arthritis by High-Resolution Peripheral Quantitative Computed Tomography: Three-Month Data
Authors: H. Choi, U. Heilmeier, M. Tanaka, A.J. Burghardt, N. Chanchek, T.M. Link, J. Graft, J.B. Imboden, X. Li
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Objectives</h3> High-resolution peripheral quantitative computed tomography (HR-pQCT) is an emerging imaging technology for early detection of erosion in rheumatoid arthritis (RA). In this study, we use HR-pQCT to investigate the effects of anti-tumor necrosis factor alpha (anti-TNFa) treatment on erosion progression for three months follow-up period. <h3>Methods</h3> Fourteen patients with clinically diagnosed RA (Group I, n=4, on methotrexate (MTX) treatment only, and Group II, n=10, on combined anti-TNFa and MTX treatment) underwent clinical evaluation using disease activity score (DAS) score and HR-pQCT scan. Scans were performed on metacarpophalangeal (MCP) joint at baseline (prior to anti-TNF initiation for Group II) in all patients and in twelve patients (4 in group I, 8 in group II) after three months. The maximum width and depth of erosions at the second, third MCP and proximal phalangeal bases (PB) were measured manually in accordance with SPECTRA definition. Erosion volume was calculated as width depth. Mann-Whitney U, Chi-square and paired T-test were done to compare measurements between and within groups from baseline to 3-month. <h3>Results</h3> The mean of ages was 71.8±3.8 in group I vs. 49.4±13.6 years in groups II (p=0.019), and of disease duration was 9.0±6.3 vs. 4.0± 4.5 years (p=0.076). The mean of total erosion volume at baseline was 8.0±10.4 mm<sup>3</sup> in group I vs. 24.5±42.1 mm<sup>3</sup> in group II (p=0.733). In group I, erosions were on PB2 (n=1), MCP2 (n=2), PB3 (n=0), and MCP3 (n=2); in group II, it was 2, 6, 2, and 5 respectively. There was a significant decrease of disease activity in group II (DAS28-CRP, p=0.001 and DAS28–ESR, p=0.002) from baseline to 3-month. Although there was no statistically significance between or within the groups, the mean changes of erosion volume by HR-pQCT tended to increase in group II (0.0±2.5 in group I vs. 4.0±6.8 in group II). <h3>Conclusions</h3> At 3 months follow-up, we observed a dissociation between clinical response and erosion volume measured by HR-pQCT in patients on anti-TNFa treatment. The significant decrease in DAS28-CRP, DAS28-ESR and tendency of increase in erosion volumes suggest that although anti-TNFa treatment suppresses inflammation within a 3-month treatment period, the structural joint damage may require more time to be hold. HR-pQCT is a sensitive tool for evaluating erosion volume changes even within a time window of 3-month. Now we are waiting one-year data of our cohort. <h3>Disclosure of Interest</h3> None declared
MicroRNA Are Useful Biomarkers for Prediction of Response to Therapy and Survival of Patients with Diffuse Large B-Cell Lymphoma.
Authors: Alvaro J. Alencar, Raquel Malumbres, Ranjana Advani, Neha Talreja, Ragini Shyam, Javier Briones, Yaso Natkunam, Randy D. Gascoyne, Robert Tibshirani, Izidore S. Lossos
Journal: Blood (2009)
A Population-Based Nested Case-Control Study of Prediagnostic Serum Antibodies to JC Virus and BK Virus and Risk of Non-Hodgkin’S Lymphoma.
Authors: Dana E. Rollison, Neal A. Halsey, Keerti V. Shah, Raphael P. Vascidi, Kathy J. Helzlsouer
Journal: Blood (2004)
PWE-103 Implementation and impact of a care bundle for the management of patients with decompensated liver disease: Abstract PWE-103 Table 1
Authors: JK Dyson, S Paremal, CA Lamb, M Hudson, S McPherson
Journal: Gut (2015)

<h3>Introduction</h3> The 2013 National Confidential Enquiry into Patient Outcome and Death (NCEPOD) report ‘<i>Measuring the Units</i>’<sup>1</sup>and a regional audit<sup>2</sup>suggested significant deficiencies in the management of patients with decompensated liver disease. As a result, on behalf of the BSG and BASL, we developed a ‘care bundle’ to optimise and standardise the care of patients with decompensated liver disease in the first 24 h.<sup>3</sup>Our aim was to audit the implementation of the care bundle in our Trust. <h3>Method</h3> A retrospective audit was conducted on the management of consecutive patients admitted with decompensated liver disease to our general medical admissions unit over 4 time periods (1 pre- and 3 post-introduction of the care bundle) to assess the effect of bundle implementation. Direct admissions to the tertiary liver unit were excluded as twice daily consultant ward rounds might confound results for those patients. <h3>Results</h3> Since the care bundle was introduced in March 2014, there has been a significant stepwise increase in completion rates (6/25 (24%) for Jun/Jul 14; 5/11 (36%) for Nov 14 and 7/8 (88%) for Dec 14/Jan 15). Table 1shows a comparison of patient management prior to bundle implementation and post-implementation in those with and without a completed bundle. Overall, patients with a completed care bundle were more likely to have appropriate management compared with patients pre-implementation and subjects post-implementation without a completed bundle. The most striking improvement was in performing a diagnostic tap to exclude spontaneous bacterial peritonitis (SBP) which increased to 100% in patients with a completed bundle compared with 46% in those without a bundle completed (p = 0.008). <h3>Conclusion</h3> Uptake of the care bundle was initially slow, but with perseverance completion rates have increased from 24% to 88% over 9 months. The ‘care bundle’ appears to improve patient care, particularly by increasing the proportion of patients having a diagnostic ascitic tap. Ongoing audit will assess the impact of the care bundle on other aspects, such as management of variceal haemorrhage, length of hospital stay and mortality. <h3>Disclosure of interest</h3> None Declared. <h3>References</h3> http://www.ncepod.org.uk/2013arld.htm Dyson, <i>et al</i>. PTU-113. BSG, 2014 McPherson, <i>et al</i>. Frontline Gastroenterol. 2014 doi:10.1136/flgastro-2014-100491
Coagulation Markers Levels Measured during Acute Infection May Predict the Development of Long Term Cardio- and Cerebro-Vascular Events in Elderly.
Authors: Asher Winder, Tatiana Bruzgol, Inna Lichman, Amir Herman, Eyal Leibovitz, Eliezer A. Rachmilewitz, Dov Gavish
Journal: Blood (2007)
OC-059 Screening for Preventable and Treatable Opportunistic Infections in a Large Cohort of IBD Patients: Time to Take Note?
Authors: S Myers, K Ashton, E Whitehead, S Sebastian
Journal: Gut (2016)

<h3>Introduction</h3> Patients with IBD are exposed to a number of preventable and treatable opportunistic infections. Some of these infections can have potential serious and occasionally life threatening consequences and hence screening for these infections to assess and modify this when possible is recommended. ECCO consensus guidelines promote screening soon as possible following diagnosis of IBD and if feasible vaccination or treatments particularly before considering immunomodulatory treatment. However the uptake of these guidelines by clinicians and indeed patients in real world practice is limited. <h3>Methods</h3> We collected data from a structured screening programme in a large cohort of IBD patients who were to be started on anti TNF therapy. Baseline characterestics were recorded. For this analysis we focussed on Varicella Zoster, Hepatitis B, hepatitis C, HIV, EBV and Tuberculosis. VZV IgG negative or equivocal patients were considered non-immune. We collected data on self reported varciella exposure in VZV IgG negative patients. All patients with indeterminate Quantiferon were subjected to a T-SPOT test. <h3>Results</h3> 254 adult and paediatric onset IBD patients (138 females, 116 males) who received or considered for biologics were included in this study. Median age at diagnosis was 28 years (range 6–71). Majority of patients (189) had Crohn’s disease. 19 patients (7.5%) were Varicella non immune at screening and all had self reported history of Varicella zoster or uncertain history. 3 of these patients who did not receive vaccination (2 patient preference) had disseminated Varicella Zoster infection. Abnormal TB screening was identified in 16 patients (6.2%). Indeterminate Quantiferon with negative T-SPOT test was noted in 12 patients. 3 patients had positive Quantiferon and T-SPOT and received anti tuberculous chemoprophylaxis. I patient with Indeterminate Quantiferon and Indeterminate T-SPOT was considered low risk . EBV negative status was identified in 7 patients (2.8%) and 3 of these were young males and Thiopurines were not used or stopped in these patients. I patient each were Hepatitis C and HIV positive without clear preidentified risk profile and both received anti viral treatment. I patient identified with hepatitis B is awaiting treatment <h3>Conclusion</h3> A significant number of preventable and treatable opportunistic infections were identified at screening in a large cohort of IBD patients receiving anti TNF therapy. Clinical assessment of risk alone without approapriate serology will overlook a number of these patients.This study highlights the need for early targeted screening in IBD patients. Cost effectiveness of this structured targetted screening needs further evaluation. <h3>Disclosure of Interest</h3> None Declared
TMOD-11HBEGF ACTIVATES EGFR AND AXL TO FORM MALIGNANT GLIOMAS IN THE CONTEXT OF Ink4a/Arf AND Pten LOSS
Authors: Clifford Shin, Sheri Holmen
Journal: Neuro-Oncology (2015)

Heparin-binding EGF-like growth factor (HBEGF) is a ligand for epidermal growth factor receptor (EGFR), the most frequently amplified receptor-tyrosine kinase in malignant gliomas. About 30% of high-grade gliomas express HBEGF, and it is also commonly upregulated in tumors that express the EGFR mutant, EGFRvIII. HBEGF has been associated with the progression of many solid tumors including breast, ovarian, bladder, and pancreatic. To date, HBEGF has not been evaluated for its ability to promote the development of high-grade gliomas in vivo. Using a retroviral method for somatic cell gene transfer, we show that HBEGF cooperates with loss of tumor suppressors Ink4a/Arf and Pten to form malignant gliomas in vivo. These mouse tumors share many characteristics found in human high-grade gliomas including necrosis and nuclear atypia. No tumors were seen with loss of two of the most common tumor suppressors altered in malignant gliomas, Ink4a/Arf and Pten. Our preliminary studies in astrocytes in vitro suggest that HBEGF activates not only EGFR but also AXL, a member of the TAM (Tyro3, AXL, Mer) family of RTKs. This cross activation may explain some of the inefficacy of targeted EGFR therapies that have been tested in malignant gliomas – even in those that have amplification of EGFR which would otherwise predict increased sensitivity to EGFR inhibition. Although unreported in the context of HBEGF or gliomas, a role for AXL in mediating EGFR signaling has been cited as a mechanism of resistance against targeted EGFR therapies in other solid tumors. Our ongoing research seeks to define the mechanism by which HBEGF activates AXL and EGFR to initiate high-grade gliomas.
THU0357 Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus - A Single Center Retrospective Clinical Analysis from India
Authors: S.K. Appani, P.K. Devarasetti, R.V. Irlapati, L. Rajasekhar
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Diffuse Alveolar hemorrhage (DAH) a rare life threatening manifestation of Systemic Lupus Erythematosus (SLE) with high mortality.<sup>1,2</sup> <h3>Objectives</h3> To characterize the profile of SLE patients with DAH in our cohort. <h3>Methods</h3> Medical charts of patients with SLE and DAH from 2008 to 2015 in our institute were reviewed. Clinical, laboratory, treatment, outcome details were noted. Statistical analysis carried out with SPSS software (version 21). Independent sample T test was done to compare survivors and non-survivors. <h3>Results</h3> Nineteen (1.32%) of 1438 SLE patients had DAH. Their mean age was 24.2±7.8 years, mean duration of hospital stay 16.1±7.5 days, mean SLE disease activity index (SLEDAI) (19.2 ±8.7). Seven had DAH at initial diagnosis of lupus, 4 had recurrent episodes of DAH. Ten (52.6%) patients died. Clinical manifestations included fever (84.2%), cough (78.9%), dyspnea (100%), hemoptysis (73.7%). Organ involvement include nephritis in (14/19) 73.7%, CNS involvement (6/19) 31.6%, arthritis (9/19) 47.4%, mucocutaneous (13/19) 68.4%, myocarditis (5/19) 26.3%, pulmonary arterial hypertension (7/19) 36.8%, serositis (8/19) 42.1%, renal failure (8/19) 42.1%, autoimmune hemolytic anemia in (2/19) 10.5% patients. Leucopenia (10/19) 52.6%, thrombocytopenia (11/19) 57.9%, low complements in 14/15 patients, dsDNA positive in (16/19) 84.2%, aCL antibody positivity in 4/14, raised serum procalcitonin in 3/14. Mean erythrocyte sedimentation rate (ESR) was 52.9±41.6 mm first hour, mean creatinine 1.6±1.5 mg/dl. Concomitant infection was seen in 7 (36.8%) patients. Respiratory tract infection was seen in 5 (4 had bacterial, 1 had strongyloides) and 2 had systemic infection. Eleven (57.9%) required mechanical ventilation. All patients received intravenous methylprednisolone pulses, cyclophosphamide in (16/19) 84.2%, IVIg in 2, Rituximab in 1, plasmapheresis in 1 patient. Median follow up of survivors is 33 months, 2 lost to follow up. Univariate analysis showed age and high ESR as independent risk factors associated with DAH. Mortality was associated with requirement of mechanical ventilation. <h3>Conclusions</h3> DAH in SLE is rare, but associated with high mortality. It occurs in patients with high disease activity. Requirement of mechanical ventilation is associated with higher mortality. <h3>References</h3> Martínez-Martínez MU, Abud-Mendoza C. Predictors of mortality in diffuse alveolar haemorrhage associated with systemic lupus erythematosus. Lupus.2011;20:568–74. Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus: clinical presentation, histology, survival, and outcome. Medicine 1997; 76: 192–202. <h3>Disclosure of Interest</h3> None declared
Double somatic mutations in mismatch repair genes are frequent in colorectal cancer after Hodgkin's lymphoma treatment
Authors: Lisanne S Rigter, Petur Snaebjornsson, Efraim H Rosenberg, Peggy N Atmodimedjo, Berthe M Aleman, Jelle ten Hoeve, Willemina R Geurts-Giele, Thomas W van Ravesteyn, Johan Hoeksel, Gerrit A Meijer, Hein te Riele, Flora E van Leeuwen, Winand N Dinjens, Monique E van Leerdam
Journal: Gut (2016)

Objective Hodgkin's lymphoma survivors who were treated with infradiaphragmatic radiotherapy or procarbazine-containing chemotherapy have a fivefold increased risk of developing colorectal cancer (CRC). This study aims to provide insight into the development of therapy-related CRC (t-CRC) by evaluating histopathological and molecular characteristics. Design 54 t-CRCs diagnosed in a Hodgkin's lymphoma survivor cohort were analysed for mismatch repair (MMR) proteins by immunohistochemistry, microsatellite instability (MSI) and KRAS / BRAF mutations. MSI t-CRCs were evaluated for promoter methylation and mutations in MMR genes. Pathogenicity of MMR gene mutations was evaluated by in silico predictions and functional analyses. Frequencies were compared with a general population cohort of CRC (n=1111). Results KRAS and BRAF mutations were present in 41% and 15% t-CRCs, respectively. Compared with CRCs in the general population, t-CRCs had a higher MSI frequency (24% vs 11%, p=0.003) and more frequent loss of MSH2/MSH6 staining (13% vs 1%, p&lt;0.001). Loss of MLH1/PMS2 staining and MLH1 promoter methylation were equally common in t-CRCs and the general population. In MSI CRCs without MLH1 promoter methylation, double somatic MMR gene mutations (or loss of heterozygosity as second hit) were detected in 7/10 (70%) t-CRCs and 8/36 (22%) CRCs in the general population (p=0.008). These MMR gene mutations in t-CRCs were classified as pathogenic. MSI t-CRC cases could not be ascribed to Lynch syndrome. Conclusions We have demonstrated a higher frequency of MSI among t-CRCs, which results from somatic MMR gene mutations. This suggests a novel association of somatic MMR gene mutations with prior anticancer treatment.