tbuckley/cabot-search huggingface dataset:
AB0490 Refractory Primary SjÖGren's Syndrome Successfully Treated with Bortezomib
Authors: J. Jakez-Ocampo, Y. Atisha-Fregoso, L. Llorente
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Primary Sjögren9s Syndrome (PSS) is a chronic autoimmune disease characterized by sicca complex, systemic manifestations and increased risk of lymphoma. Although it is well accepted the use of steroids for the treatment of systemic manifestations, there is scarce information available regarding the use of targeted therapy for refractory cases. Herein we describe a case of a severe PSS patient refractory to conventional treatment as well as, with a notable response to bortezomib, a proteasome inhibitor commonly used for the treatment of multiple myeloma. <h3>Objectives</h3> To describe a clinical case of a patient with refractory PSS successfully treated with bortezomib <h3>Methods</h3> A 42 years old woman with a history of 15 years of PSS was evaluated for persistent and refractory hyperglobulinemic purpura and severe fatigue that causes her serious disability. Her globulin levels were 9.6 g/L and the serum viscosity of 3.1 AU, and no response was observed with conventional immunosuppressive therapy namely, prednisone and azathioprine. Initially, the patient received two cycles of rituximab with only partial response in the laboratory parameters. No changes were observed neither in purpuric skin lesions nor in fatigue, even though the globulins level decreased to 3.2 g/L. Considering the persistence of clinical manifestations and of the risk of lymphoma and taking into account the theoretical principle of the activation of B and plasma cells by activation of proteasome in Sjögren9s syndrome, the patient received bortezomib (1.3 mg/m<sup>2</sup>) once a week for two months. The treatment options and her condition at that moment were discussed with the patient who agreed with the therapeutic modality. <h3>Results</h3> Bortezomib administration resulted in a notable improvement of general symptoms, a decrease in serum globulin levels from 8.5 to 4.9 g/dL (IgG from 7157 to 3013 mg/dL; IgA from 3069 to 784 mg/dL and IgM from 1353 to 387 mg/dL), as well as in serum viscosity from 3.0 to 2.2 AU. Hyperglobulinemic purpura disappears and prednisone tapering succeeds. After one year of follow-up, the patient is on 7.5 mg/day of prednisone and still without fatigue and skin lesions, although serum globulin levels remain high (4.9 g/dL). <h3>Conclusions</h3> As far as we know, this is the first report on the use of bortezomib in a refractory case of PSS. The rationale for its use resembles that of multiple myeloma, where an excessive production of an immunoglobulin by a plasma cell is the characteristic feature of its pathogenesis. Even when PSS is not a hematological neoplasia of plasma cell origin, the hyper viscosity syndrome in our patient were necessarily caused by a B and plasma cell hyperactivity. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.5549
Growth Hormone in Relation to Diabetic Retinopathy
Authors: Elinor D. U. Powell, Andrew G. Frantz, Mitchell T. Rabkin, Richard A. Field
Journal: New England Journal of Medicine (1966)

ARREST of diabetic retinopathy following spontaneous1 2 3 or induced4 5 6 hypopituitarism has frequently been demonstrated in the past thirteen years. Conjecture regarding the mechanism involved has focused on growth hormone in view of its action in aggravating diabetes. The radioimmunoassay of this hormone in blood by the method originated by Hunter and Greenwood7 and Glick et al.8 and the provocation of release by insulin induced hypoglycemia as described by the latter group9 have permitted quantitation of the hormone. In the present study these technics have been used to assess the influence of growth hormone in the progression and regression of diabetic retinopathy . . .
A Controlled Trial of Trimethoprim–Sulfamethoxazole or Aerosolized Pentamidine for Secondary Prophylaxis of <i>Pneumocystis carinii</i> Pneumonia in Patients with the Acquired Immunodeficiency Syndrome
Authors: W. David Hardy, Judith Feinberg, Dianne M. Finkelstein, Maureen E. Power, Weili He, Carolyn Kaczka, Peter T. Frame, Martin Holmes, Hetty Waskin, Robert J. Fass, William G. Powderly, Roy T. Steigbigel, Abigail Zuger, Robert S. Holzman
Journal: New England Journal of Medicine (1992)

Pneumocystis carinii pneumonia (PCP) continues to be the most common index diagnosis in the acquired immunodeficiency syndrome (AIDS), but it is not clear which of several available agents is the most effective in preventing a recurrence of PCP.
Abstract 2975: Transgenic mouse model of Twist1-induced metastasis reveals genes highly prognostic for human hepatocellular carcinoma
Authors: David I. Bellovin, Phuoc T. Tran, Stacey J. Adam, Andrew J. Gentles, Xin W. Wang, Dean W. Felsher
Journal: Cancer Research (2012)

Abstract Hepatocellular carcinoma (HCC) is a top global cancer health concern due to its high rate of mortality and increasing incidence. HCC lethality is largely attributable to its poor response to existing therapy and frequent diagnosis at late stage of disease, consequently resulting in HCC that progresses to local invasion and distant metastasis. Expression of Twist1 has been correlated with cancer invasion and metastasis, but a causal role has yet to be established for autochthonous tumors. Here, we report a new conditional transgenic mouse model of Twist1- and MYC-induced primary hepatocellular carcinoma (HCC). Expression of MYC alone produced primary tumors, but failed to induce metastasis, while Twist1 co-expression with MYC caused extra-hepatic metastases to the lymph nodes, spleen, peritoneum, and lungs. Moreover, Twist1 expression caused an increase in circulating tumor cells (CTCs). Combined Twist1 and MYC inactivation resulted in sustained regression of both primary and metastatic HCC as shown by X-ray computed tomography (micro-CT) and bioluminescence imaging (BLI) and the suppression of metastasis as measured by a loss of CTCs. Importantly, gene expression profiling illustrated that our mouse model of Twist1/MYC-induced liver cancer was representative of human HCC. Moreover, we exploited the Twist1-induced progression to identify a gene signature in Twist1/MYC tumors that is highly predictive of metastasis in human HCC patients, as well as prognostic for overall survival. Hence, we show that Twist1 expression can causally induce stepwise disease progression to metastasis in primary HCC, allowing for the identification of a gene signature that is highly predictive of clinical outcome in human liver cancer. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 103rd Annual Meeting of the American Association for Cancer Research; 2012 Mar 31-Apr 4; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2012;72(8 Suppl):Abstract nr 2975. doi:1538-7445.AM2012-2975
AB1169-HPR The Relationship between Pain and Health-Related Quality of Life in Patients with Juvenile-Onset Systemic Lupus Erythematosus
Authors: E. Tarakci, S.N. Baydogan, O. Kasapcopur, K. Barut
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Juvenile-onset systemic lupus erythematosus (JSLE) is an autoimmune disorder with multisystem involvement, leading to inflammatory damage to the joints, kidney, central nervous system, and hematopoietic system affecting children and adolescents. According to these multisystem deteriorations lead reduction in health-related quality of life (HRQL) in patients with JSLE. <h3>Objectives</h3> The aim of this study was to assess the relationship between HRQL and pain in patients with JSLE. <h3>Methods</h3> 19 patients (female=16, male=3) with JSLE and their parents were enrolled in the study. The subjects were recruited in a pediatric rheumatology clinic. They were diagnosed with JSLE by a pediatric rheumatologist based on the clinical diagnostic criteria. Patients with recent diagnoses of JSLE and those with mental deficits were excluded. Visual Analog Scale (VAS) was used to evaluate pain (during rest, activity and exercise) and well being. Functional ability was evaluated with Childhood Health Assessment Questionaire (CHAQ). Also, the Turkish version of PedsQL 3.0 Arthritis Module was used to evaluate HRQL. <h3>Results</h3> The mean age was 14.16±2.66 years (range 12–18 years). The mean disease duration was 3.42±2.76 years (range 0.08–15 years). The mean of number of affected joint was 2.16±2.92. Significant relationships were found between score of VAS-pain (during activity) and number of affected joint (r=0.801 p=0,000) and score of PedsQL-patient9s report (r=-0.655 p=0,002) and score of PedsQL-parent9s report (r=-0.670 p=0,002) and score of VAS-well being (r=0.759 p=0,000). <h3>Conclusions</h3> This study demonstrated that there is significant relationship between increased pain and reduction health-related quality of life in patients with JSLE. Due to number of affected joint increasing pain may results impairment daily functions and reduction health-related quality of life in patients with JSLE. Therefore, we concluded that reduction of pain has substantial role on improving of health-related quality of life in treatment of patients with JSLE. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.6056
The Effects of Supraphysiologic Doses of Testosterone on Muscle Size and Strength in Normal Men
Authors: Shalender Bhasin, Thomas W. Storer, Nancy Berman, Carlos Callegari, Brenda Clevenger, Jeffrey Phillips, Thomas J. Bunnell, Ray Tricker, Aida Shirazi, Richard Casaburi
Journal: New England Journal of Medicine (1996)

Athletes often take androgenic steroids in an attempt to increase their strength. The efficacy of these substances for this purpose is unsubstantiated, however.
AB0737 The resting state and task based functional magnetic resonance imaging study in non-neuropsychiatric systemic lupus erythematosus
Authors: Y. Fang, Q. Zou
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Objectives</h3> To find evidence for occult cerebral functional damage resides prior to clinical symptoms by using resting state and task based functional magnetic resonance imaging(fMRI) in systemic lupus erythematosus(SLE) patients without neuropsychological symptoms. <h3>Methods</h3> 41 non-neuropsychiatric systemic lupus erythematosus(non-NPSLE) patients and 28 similar control participants were enrolled, performed paced visual serial addition test (PVSAT), and performed both resting state and PVSAT task based fMRI scanning. Data were processed and analysed by SPM8 software package, resting state data were post processed with method called regional homogeneity (ReHo). Difference of ReHo values between patients and controls were compared by independent t test. The activated brain areas of PVASAT tasks were compared in SPM8, and functional connectivity of these areas were tested in resting state data. <h3>Results</h3> During resting state, compared to the controls, non-NPSLE subjects displayed significantly decreased ReHo values in multiple areas including the bilateral posterior lobes of the cerebellum (predominantly in crus I and II of the neocerebellum), the vermis, left inferior frontal gyrus, left precuneus, right limbic lobe and cingulate gyrus; ReHo was increased in bilateral cuneus and in the calcarine gyrus Both groups had no significant difference in PVSAT test. In PVSAT task based fMRI, in control subjects, the activations were situated in left superior and inferior parietal lobe, and left inferior frontal gyrus. While for non-NPSLE patients, significantly extended activations were found in left hemisphere in superior and inferior parietal lobe, superior, middle and inferior frontal gyrus. Brain areas activated in healthy controls were selected as regions of interest (ROIs) to test for the functional connectivity strength between patients and controls. Compared with controls, non-NPSLE patients exhibited a significantly higher magnitude of connectivity strength between areas activated during working memory task. <h3>Conclusions</h3> During resting state, non-NPSLE patients exhibited alteration of major areas of default mode network; while significantly expanded areas of activation in task based fMRI, both resutls demonstrated that cerebral damage existed prior to the presentation of any neuropsychological symptoms. <h3>Disclosure of Interest</h3> None Declared
Abstract 4579: Effect of pre-adipocyte and differentiated adipocyte conditioned media on galectin-3 expression in mouse intestinal epithelial cells
Authors: Jong-Sang Kim, Ji-Sun Lim, Annalene Garcia, Ji-Yeon Seo, Jia Park, Dae Hwan Nam, Mi Kyung Sung, Young-Rok Seo
Journal: Cancer Research (2010)

Abstract We hypothesized that obesity cause inflammation which, in turn, promotes colon carcinogenesis. To test the hypothesis mouse intestinal IEC-6 cells were incubated in the presence of pre-conditioned media collected from culturing LPS-stimulated macrophage, followed by comparing protein expression profile with control cells. Our proteomic study revealed 45 differentially regulated proteins in mouse intestinal IEC-6 cells under state of inflammation. Among others, galectin-3, a beta-galactoside-binding protein, was up-regulated. Galectin-3 has been reported to play anti-apoptotic role in tumor progression and metastasis, and its over-expression has been observed in several types of cancer such as colon cancer. In order to determine relationship between obesity-induced inflammation and colon carcinogenesis, conditioned media (CM) from LPS-stimulated 3T3-L1 pre-adipocytes or differentiated adipocytes were utilized to culture IEC-6 cells. The CM was analyzed for levels of nitric oxide (NO), TNF-α and IL-6, and IEC lysates were analyzed for galectin-3 expression by western blot. Results showed that enhanced generation of NO, TNF-α and IL-6 resulted in upregulation of galectin-3 with respect to incubation time. Also, cytokine concentration was correlated to level of galectin-3, as verified by incubating IEC with increasing concentration of TNF-α or IL-6. These results indicate involvement of pro-inflammatory mediators from pre-adipocytes and adipocytes in colon carcinogenesis, in which galectin-3 may play a crucial role. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 101st Annual Meeting of the American Association for Cancer Research; 2010 Apr 17-21; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2010;70(8 Suppl):Abstract nr 4579.
Gallbladder Bed Photopenic Halo on Hepatobiliary Scintigraphy: A Sign of Biliary Leakage
Authors: NICOLAAS I. BOHNEN, MILTON D. GROSS, BRAHM SHAPIRO
Journal: Clinical Nuclear Medicine (1999)

A 34-year-old woman who had received a kidney transplant underwent Tc-99m mebrofenin hepatobiliary scintigraphy to evaluate suspected biliary leakage several days after laparoscopic cholecystectomy for chronic symptomatic cholelithiasis. The study showed an unusual circumferential photopenic rim around the gall-bladder bed on hepatobiliary scintigraphy that preceded obvious scan evidence of biliary leakage. Severe inflammatory changes in the soft tissues around the gallbladder with local fluid collections are likely to be associated with this "reverse halo sign." This photopenic halo sign is different from the well-known "rim" sign that is often seen in acute gangrenous cholecystitis.
Case 14-2014
Authors: Kalpathy S. Krishnamoorthy, Florian Eichler, Otto Rapalino, Matthew P. Frosch
Journal: New England Journal of Medicine (2014)

An 11-month-old girl was seen in the neurology clinic because of developmental delay. Development to 6 months was normal; by 11 months, she had difficulty sitting and had stopped reaching for objects and feeding herself. An examination and diagnostic tests were performed.
AB0810 The 15% rule in scleroderma: The frequency of organ complications in systemic sclerosis (SSC). A systematic review:
Authors: C. Muangchan, M. Baron, J.E. Pope
Journal: Annals of the Rheumatic Diseases (2012)

<h3>Background</h3> The prevalence of each organ complication in scleroderma (SSc) varies by definition used. However, it is important to be aware of several complications that can be detected and treated. A simple rule of prevalence of organ complications in SSc can be helpful for clinicians. <h3>Objectives</h3> This study was done to determine the frequency of several SSc features including organ involvement (lung, heart, digital ulcers {DU}, scleroderma renal crisis {SRC}, pulmonary arterial hypertensions {PAH}). <h3>Methods</h3> A comprehensive literature search of the Medline-OVID/EMBASE, Pub Med, and Scopus databases from 1980 to November 30th, 2011 was conducted to identify relevant articles with at least 50 SSc patients. Search words were within organ systmes such as lung, heart, pulmonary artery, kidney, digital ulcers, arthritis, myopathy and secondary Sjogren's. Study quality was assessed using the STROBE checklist. Prevalence of each organ complication was extracted from studies. Pooled prevalence and odds ratios (ORs) were calculated using the random effects method, and between-study heterogeneity was quantified using the I-squared statistic. <h3>Results</h3> 5916 articles were identified (913 from Embase, 1009 from PubMed and 3994 from Scopus). 5665 were excluded of which 4912 were irrelevant, 237 did not report organ prevalence, 183 were case reports, 193 were reviews, 111 had less than 50 patients,24 were not in Egnlish and 5 were not obtained; leaving 251 articles for full text review with 80 included in the meta-analysis. Where available, frequencies were also included from the Canadian Scleroderma Reserach Group (a database with more than 1000 SSc patients). There were no GI complications at a prevalence of 15% (GERD and dysphagia are far higher) and RP is nearly 100%. Xray ILD was common but significant restrictive ILD on PFTs occurred in approximately 15%. <h3>Conclusions</h3> Many complications in SSc occur 15% of the time including low FVC, PAH, diastolic dysfunction, clincal and echo cardiac changes, arrhythmias, inflammatory arthritis, myopathy, Sjogren's and digital ulcers. 15% of digital ulcers have complications. SRC is uncommon but occurs in almost 15% of dcSSc. This is a helpful rule for frequency of organ involvement in SSc. <h3>Disclosure of Interest</h3> C. Muangchan: None Declared, M. Baron: None Declared, J. Pope Grant/Research support from: Funding for the CSRG is from the Canadian Institutes of Health Research (CIHR) and the Fonds de Recherché en Santé du Québec, as well as Scleroderma Society of Canada, Scleroderma Society of Ontario, Sclérodermie Québec and Cure Scleroderma Foundation
AB0814 Molecular profiling to diagnose a case of atypical dermatomyositis
Authors: K. Sarin, L. Chung, J. Kim, B.W. Higgs, B. Jallal, Y. Yao, D. Fiorentino
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> The diagnosis of dermatomyositis (DM) is often challenging as skin or muscle biopsy findings can be non-specific and the characteristic rash is quite heterogeneous in presentation. For example, DM skin lesions often have a distribution similar to that of psoriasis and both diseases share activation of some common inflammatory pathways. In some cases, psoriatic lesions have been reported to occur in patients with DM, although it remains unclear if these lesions represent a coincidental occurrence of psoriasis and DM in the same patient or instead represent an atypical cutaneous manifestation of DM (1,2). <h3>Objectives</h3> This clinical scenario was used to conduct the current proof-of-concept study to determine if molecular profiling could be used to aid the clinical diagnosis of DM in a patient in which traditional histopathology yielded inconclusive results. <h3>Methods</h3> A tissue sample was obtained from a clinical and histopathologic psoriatic-appearing lesion occurring in a patient with known DM. Using oligonucleotide microarrays, its molecular features were compared to lesional skin biopsies obtained from 34 confirmed DM and 33 psoriasis patients. <h3>Results</h3> We first identified the pathways enriched by differential transcripts which differentiate psoriasis from DM. We then demonstrated that the psoriasiform lesion displayed molecular features more similar to DM than psoriasis using both unsupervised hierarchical clustering and principal components analysis. <h3>Conclusions</h3> This case study suggests that lesions that appear clinically and histologically similar to psoriasis may instead represent an atypical manifestation of DM. Furthermore, this also highlights the clinical utility of molecular profiling in enhancing diagnosis in autoimmune skin diseases. <h3>References</h3> Pavlovic, M Zecevic RD, Zolotarevski L. Psoriasis in a patient with dermatomyositis. Vojnosanit Pregl 2004; 61(5):557-9. Kim NN, Lio PA, Morgan GA, Jarvis JN, Pachman LM. Double Trouble: Therapeutic Challenges in Patients With Both Juvenile Dermatomyositis and Psoriasis. Arch Dermatol 2011. <h3>Disclosure of Interest</h3> K. Sarin: None Declared, L. Chung: None Declared, J. Kim: None Declared, B. Higgs Shareholder of: Astra Zeneca, Employee of: MedImmune, B. Jallal Shareholder of: Astra Zeneca, Employee of: MedImmune, Y. Yao Shareholder of: Astra Zeneca, Employee of: MedImmune, D. Fiorentino: None Declared
THU0195 Incidence of Infliximab (IFX) Infusion Reactions with and Without Premedication
Authors: F. Bartoli, G. Fiori, F. Galluccio, F. Nacci, G. Salvadorini, M. Matucci Cerinic
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> Some patients may experience adverse drug reactions (ADR) during infusion with IFX and this could be due to immunogenicity. <h3>Objectives</h3> To evaluate the incidence of infusion reactions in rheumatoid arthritis (RA) patients, seronegative spondiloarthritis (SpA) and psoriatic arthritis (PsA) receiving IFX with and without premedication <h3>Methods</h3> The charts of 75 patients treated with infliximab from 2000 to 2012 were reviewed. From 2001 to 2006 patients did not receive any medication before IFX, while from 2007 to 2011 patients received premedication with paracetamole (500 mg) and iv with esomeprazole 40 mg iv, hydrocortisone 5 mg/Kg, clorfenamine 10 mg. From 2011 on, premedication was modified as follows: paracetamole 500 mg, Hydroxizine 25 mg and iv ranitidine 50 mg, 6-methilprednisolone 100 mg. <h3>Results</h3> From 2001 to 2006 when premedication was not yet used, 21/75 (28%) patients had infusional reactions to IFX. From 2007 to 2011 9/67 (13.4%) patients had infusional reactions after introduction of corticosteroids and antihistamines premedication. Moreover, 1/57 patient (1.7%) had infusional reaction in the last two years using premedication with 6-methilprednisolone iv and ranitidine iv. The number of infusion reactions was significantly lower using premedication with corticosteroids and antihistamines (p &lt;0.05). The incidence of infusional reactions was significantly reduced (p &lt;0.05) with paracetamole, hydroxyzine and 6-metilprednisolone 100 mg and ranitidine iv before IFX treatment. No one anaphylactic reactions was observed. <h3>Conclusions</h3> In our experience, the combination of drugs in a premedication protocol with paracetamole, hydroxyzine, 6-methilprednisolone and ranitidine iv reduced significantly the number and severity of infusional reactions to IFX. <h3>References</h3> Sany J, Kaiser MJ, Jorgensen C, Trape G. Study of the tolerance of infliximab infusions with or without betamethasone premedication in patients with active rheumatoid arthritis. Ann Rheum Dis. 2005 Nov;64(11):1647-9. Okuyama A, Nagasawa H, Suzuki K, Kameda H, Kondo H, Amano K, Takeuchi. Fcγ receptor IIIb polymorphism and use of glucocorticoids at baseline are associated with infusionreactions to infliximab in patients with rheumatoid arthritis.Ann Rheum Dis. 2011 Feb;70(2):299-304. Epub 2010 Oct 27. Lequerré T, Vittecoq O, Klemmer N, Goëb V, Pouplin S, Menard JF, Daragon A, Mejjad O, Le Loët X. Management of infusion reactions to infliximab in patients with rheumatoid arthritis or spondyloarthritis: experience from an immunotherapy unit of rheumatology.J Rheumatol. 2006 Jul;33(7):1307-14. Epub 2006 Jun <h3>Disclosure of Interest</h3> None Declared