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https://www.readbyqxmd.com/read/28217959/anti-rnpc3-antibodies-as-a-marker-of-cancer-associated-scleroderma
#1
Ami A Shah, George Xu, Antony Rosen, Laura K Hummers, Fredrick M Wigley, Stephen J Elledge, Livia Casciola-Rosen
INTRODUCTION: Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with RNA polymerase III (POL) autoantibodies and in patients negative for anti-centromere (CENP), anti-topoisomerase-1 (TOPO), and anti-POL antibodies (referred to as CENP/TOPO/POL (CTP)-Negative). In a recent study of 16 CTP-negative scleroderma patients with coincident cancer, we found that 25% had autoantibodies to RNPC3, a member of the minor spliceosome complex. In this investigation, we validated the relationship between anti-RNPC3 antibodies and cancer and examined the associated clinical phenotype in a large sample of scleroderma patients...
February 19, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28203114/longitudinal-analysis-of-quality-of-life-in-patients-with-undifferentiated-connective-tissue-diseases
#2
Michele Iudici, Rosaria Irace, Antonella Riccardi, Giovanna Cuomo, Serena Vettori, Gabriele Valentini
INTRODUCTION/OBJECTIVES: To prospectively assess the quality of life (QoL) of patients affected by undifferentiated connective tissue diseases (UCTDs) and to identify factors associated with changes over time. PATIENTS AND METHODS: A total of 46 consecutive UCTD patients completed the Short-Form 36 (SF-36) questionnaire at presentation and then yearly. At each 6-month visit, all patients underwent a detailed history taking and a laboratory and physical assessment, in order to follow the evolution of the disease over time and to assess the the co-existence of fibromyalgia...
2017: Patient related Outcome Measures
https://www.readbyqxmd.com/read/28197938/clinical-efficacy-and-safety-of-bathing-with-chinese-medicine-taohong-siwu-decoction-for-treatment-of-diffuse-cutaneous-systemic-sclerosis-a-randomized-placebo-controlled-trial
#3
Jing Zhou, Dong Yang, Shu-Hong Zhou, Jin-Ping Wang, Yue-Shu Liu, Shu-Lan Wang
OBJECTIVE: To examine the efficacy and safety of bathing therapy with Taohong Siwu Decoction (, TSD) in the treatment of early-stage, mild-moderate diffuse cutaneous systemic sclerosis (dcSSc). METHODS: This randomized, placebo-controlled trial enrolled 148 men and women (18-60 years) with dcSSc (disease duration ≤12 months) and baseline modified Rodnan skin score (MRSS) ≥10. Patients were randomized into a TSD group (71 cases bathing with TSD plus oral prednisone) or control group (71 cases bathing with placebo plus oral prednisone)...
February 15, 2017: Chinese Journal of Integrative Medicine
https://www.readbyqxmd.com/read/28188718/-hematopoietic-stem-cells-transplant-in-patients-with-common-variable-immunodeficiency-is-a-therapeutic-option
#4
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Patricia López-Pérez, Aurora Chávez-García, Marco Antonio Yamazaki-Nakashimada
BACKGROUND: Patients with common variable immunodeficiency show higher incidence of sinopulmonary and gastrointestinal infections, as well as lymphoproliferative and autoimmune diseases. The treatment of choice is replacement therapy with human gamma-globulin. Hematopoietic stem cell transplantation is a non-conventional therapeutic modality. CASE REPORT: Twenty-six-year old woman with no family or hereditary history of primary immune deficiencies or consanguinity, with repeated episodes of otitis, sinusitis, gastroenteritis and bronchitis since childhood...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28147261/clinical-follow-up-predictors-of-disease-pattern-change-in-anti-jo1-positive-anti-synthetase-syndrome-results-from-a-multicenter-international-and-retrospective-study
#5
REVIEW
Elena Bartoloni, Miguel A Gonzalez-Gay, Carlo Scirè, Santos Castaneda, Roberto Gerli, Francisco Javier Lopez-Longo, Julia Martinez-Barrio, Marcello Govoni, Federica Furini, Trinitario Pina, Florenzo Iannone, Margherita Giannini, Laura Nuño, Luca Quartuccio, Norberto Ortego-Centeno, Alessia Alunno, Christopher Specker, Carlomaurizio Montecucco, Konstantinos Triantafyllias, Silvia Balduzzi, Walter Alberto Sifuentes-Giraldo, Giuseppe Paolazzi, Elena Bravi, Andreas Schwarting, Raffaele Pellerito, Alessandra Russo, Carlo Selmi, Lesley-Ann Saketkoo, Enrico Fusaro, Simone Parisi, Nicolò Pipitone, Franco Franceschini, Ilaria Cavazzana, Rossella Neri, Simone Barsotti, Veronica Codullo, Lorenzo Cavagna
OBJECTIVE: Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients...
January 29, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28092215/therapeutic-implications-from-the-pathogenesis-of-raynaud-s-phenomenon
#6
Ariane L Herrick
Raynaud's phenomenon (RP) can be either primary (idiopathic) or secondary to a number of different diseases/conditions, when vasopasm can be superimposed upon structural vascular abnormality or a hyperviscosity state and may then lead to severe ischaemia with tissue damage. Treatment must be tailored to the individual. Areas covered: This review discusses how increased understanding of the pathogenesis of RP has driven and is driving new approaches to therapy, and how we are now better able to predict which patients presenting with RP are likely to have an underlying disease requiring specific intervention...
January 16, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28089066/evaluation-and-prevention-of-the-negative-matrix-effect-of-terpenoids-on-pesticides-in-apples-quantification-by-gas-chromatography-tandem-mass-spectrometry
#7
Géraldine Giacinti, Christine Raynaud, Sophie Capblancq, Valérie Simon
The sample matrix can enhance the gas chromatography signal of pesticide residues relative to that obtained with the same concentration of pesticide in solvent. This paper is related to negative matrix effects observed in coupled gas chromatography-mass spectrometry ion trap (GC/MS(2)) quantification of pesticides in concentrated extracts of apple peel prepared by the Quick Easy Cheap Effective Rugged and Safe (QuEChERS) method. It is focused on the pesticides most frequently used on the apple varieties studied, throughout the crop cycle, right up to harvest, to combat pests and diseases and to improve fruit storage properties...
December 21, 2016: Journal of Chromatography. A
https://www.readbyqxmd.com/read/28069166/evaluation-and-prevention-of-the-negative-matrix-effect-of-terpenoids-on-pesticides-in-apples-quantification-by-gas-chromatography-tandem-mass-spectrometry
#8
Géraldine Giacinti, Christine Raynaud, Sophie Capblancq, Valérie Simon
The sample matrix can enhance the gas chromatography signal of pesticide residues relative to that obtained with the same concentration of pesticide in solvent. This paper is related to negative matrix effects observed in coupled gas chromatography-mass spectrometry ion trap (GC/MS(2)) quantification of pesticides in concentrated extracts of apple peel prepared by the Quick Easy Cheap Effective Rugged and Safe (QuEChERS) method. It is focused on the pesticides most frequently used on the apple varieties studied, throughout the crop cycle, right up to harvest, to combat pests and diseases and to improve fruit storage properties...
February 3, 2017: Journal of Chromatography. A
https://www.readbyqxmd.com/read/28065485/systemic-sclerosis-sine-scleroderma-in-mexican-patients-case-reports
#9
Olga Vera-Lastra, Christian Alexis Sauceda-Casas, María Del Pilar Cruz Domínguez, Sergio Alberto Mendoza Alvarez, Jesús Sepulceda-Delgado
: Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. CONCLUSION: In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc...
January 3, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28053878/microparticle-subpopulations-are-potential-markers-of-disease-progression-and-vascular-dysfunction-across-a-spectrum-of-connective-tissue-disease
#10
E M McCarthy, D Moreno-Martinez, F L Wilkinson, N J McHugh, I N Bruce, J D Pauling, M Y Alexander, B Parker
OBJECTIVE: Microparticles (MPs) are membrane-bound vesicles derived from vascular and intravascular cells such as endothelial cells (EMPs) and platelets (PMPs). We investigated EMP and PMP numbers across a spectrum of autoimmune rheumatic diseases (AIRDs) with the aim of comparing the levels of, and relationship between, EMPs and PMPs. METHODS: Patients with Systemic Lupus Erythematosus (SLE) (n = 24), Systemic Sclerosis (SSc) (n = 24), Primary Raynauds Phenomenon (RP) (n = 17) and "other CTD" (n = 15) (Primary Sjogrens Syndrome, UCTD or MCTD) as well as 15 healthy controls were recruited...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28053559/systemic-lupus-erythematosus-and-pulmonary-arterial-hypertension-links-risks-and-management-strategies
#11
REVIEW
Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz
Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28029677/an-interesting-case-of-basal-cell-carcinoma-with-raynaud-s-phenomenon-following-chronic-arsenic-exposure
#12
S Gulshan, M J Rahman, R Sarkar, S Ghosh, R Hazra
Arsenic is commonly known to be associated with squamous cell carcinoma. Among the lesser known associations is basal cell carcinoma and even rarer is its effect on blood vessels causing peripheral vascular disease. Here we present a case of a 55 yr old man with ulceroproliferative lesions on scalp and forehead along with several hyperpigmented patches on trunk and extremities. He had symptoms suggestive of Raynaud's phenomenon that eventually led to digital gangrene. FNAC was done which was suggestive of basal cell carcinoma...
October 2016: JNMA; Journal of the Nepal Medical Association
https://www.readbyqxmd.com/read/28024309/stroke-as-initial-manifestation-of-adenosine-deaminase-2-deficiency
#13
Miriam Elbracht, Michael Mull, Norbert Wagner, Christiane Kuhl, Angela Abicht, Ingo Kurth, Klaus Tenbrock, Martin Häusler
Deficiency of adenosine deaminase 2 (ADA2) due to homozygous or compound heterozygous mutations in the cat eye syndrome chromosome region, candidate 1 (CECR1) gene causes an autoimmune phenotype with systemic vasculitis affecting the skin, inner organs, and the central nervous system. Typically, stroke has been reported to follow systemic inflammatory disease and predominantly affects posterior and central brain areas. Here, we describe one of the rare patients in whom acute mesencephalic stroke preceded systemic inflammation and presented as initial clinical symptom...
December 26, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/28018840/hand-impairment-in-systemic-sclerosis-various-manifestations-and-currently-available-treatment
#14
Amber Young, Rajaie Namas, Carole Dodge, Dinesh Khanna
Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud's phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often co-exist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life...
September 2016: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/27998297/difficulties-in-diagnosis-and-treatment-of-severe-secondary-raynaud-s-phenomenon-in-a-cameroonian-woman-a-case-report
#15
Valirie Ndip Agbor, Tsi Njim, Leopold Ndemnge Aminde
BACKGROUND: Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27996340/geographic-variation-as-a-risk-factor-for-digital-ulcers-in-systemic-sclerosis-patients-a-multicentre-registry
#16
Ejr Souza, C S Muller, Amc Horimoto, R A Rezende, I Guimarães, H A Mariz, A T Dantas, I P Da Costa, Apt Del-Rio, J Sekiyama, C B Kahwage, C Kayser
OBJECTIVE: To evaluate the influence of geographic variation on the risk of digital ulcer (DU) development in systemic sclerosis (SSc) patients. METHODS: This cross-sectional, multicentre study evaluated patients with SSc from centres located in different geographic regions of Brazil (subtropical and tropical climate zones). Demographic and clinical data were collected. RESULTS: The study included 141 patients with SSc (26 from the subtropical and 115 from the tropical zone)...
December 20, 2016: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/27987518/-clinical-characteristics-of-4-cases-of-scleritis-associated-with-systemic-lupus-erythematosus
#17
L Wang, Y Yang, Y Jia, H Miao, Y S Zhou, X Y Zhang
Episcleritis and scleritis are relatively rare ocular diseases, which are commonly associated with rheumatic diseases including systemic lupus erythematosus (SLE). To investigate clinical and laboratory features of SLE-associated episcleritis and scleritis, we now report 4 cases of inpatients who were diagnosed with episcleritis or scleritis secondary to SLE from September 2005 to July 2016 in the Department of Rheumatology and Immunology in Peking University People's Hospital. Demographic, clinical and laboratory characteristics were summarized together with the treatment regimen and the prognosis; the literature was reviewed...
December 18, 2016: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/27979657/undifferentiated-connective-tissue-disease-and-interstitial-lung-disease-trying-to-define-patterns
#18
María Laura Alberti, Francisco Paulin, Heidegger Mateos Toledo, Martín Eduardo Fernández, Fabián Matías Caro, Jorge Rojas-Serrano, Mayra Edith Mejía
OBJECTIVES: To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior. METHODS: Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: 'highly specific' connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1: 320), and 'specific' ANA staining patterns (centromere, cytoplasmic and nucleolar patterns)...
December 12, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27931961/imaging-of-connective-tissue-diseases-beyond-visceral-organ-imaging
#19
REVIEW
Maurizio Cutolo, Nemanja Damjanov, Barbara Ruaro, Ana Zekovic, Vanessa Smith
Connective tissues diseases (CTDs) can also be diagnosed early by "external" and safe imaging methods beyond the visceral organ analysis. This study aims to explore various imaging techniques used in diagnosing CTDs. Skin impairment in systemic sclerosis (SSc) may be recognized and studied by the modified Rodnan skin score (mRSS), which has some drawbacks, whereas high-frequency ultrasound (US) seems advantageous for the early identification of skin involvement. Salivary gland involvement in Sjögren syndrome (SS) can be assessed using standard tests such as unstimulated salivary flow test, salivary gland scintigraphy or contrast sialography...
August 2016: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/27917635/-raynaud-s-phenomenon
#20
Michal Tomčík
Raynaud's phenomenon (RP) is a very common sign which can usually be seen across all medical specialties. It is characterized by episodic color changes of acral parts of the body (palor, cyanosis, rubor) lasting from a few minutes to hours, which are usually triggered by cold temperature and/or stress. The primary RP occurs alone, without concomitant diseases, is usually benign and has favorable prognosis. Secondary RP occurs in a variety of diseases with a very variable progression and prognosis, mostly unfavorable one due to the development of ischemic tissue necrosis and gangrene...
2016: Casopís Lékar̆ů C̆eských
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