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Clinical and Experimental Rheumatology

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https://www.readbyqxmd.com/read/28628471/haematological-involvement-associated-with-a-mild-autoinflammatory-phenotype-in-two-patients-carrying-the-e250k-mutation-of-pstpip1
#1
Elena Belelli, Chiara Passarelli, Manuela Pardeo, Dirk Holzinger, Fabrizio De Benedetti, Antonella Insalaco
OBJECTIVES: Hyperzincaemia/hypercalprotectinemia (Hz/Hc) syndrome is a recently described condition caused by a specific de novo mutation (E250K) affecting PSTPIP1 gene. It has a phenotype distinct from classical pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome that includes severe systemic and cutaneous inflammation, hepatosplenomegaly, arthritis without sequelae, pancytopenia and failure to thrive. METHODS: We describe an 8-year-old boy who presented recurrent right knee swelling mimicking septic arthritis and persistent bone marrow involvement, without cutaneous involvement...
June 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628466/evaluation-of-cancer-associated-myositis-and-scleroderma-autoantibodies-in-breast-cancer-patients-without-rheumatic-disease
#2
Ami A Shah, Antony Rosen, Laura K Hummers, Betty J May, Alpana Kaushiva, Richard B S Roden, Deborah K Armstrong, Fredrick M Wigley, Livia Casciola-Rosen, Kala Visvanathan
OBJECTIVES: Systemic sclerosis (scleroderma) and dermatomyositis are two prototypic autoimmune diseases that are strongly associated with malignancy. While specific autoantibodies in these diseases are markers of an increased risk of cancer at scleroderma and dermatomyositis onset, it is not known whether these autoantibodies are biomarkers of cancer risk in patients without rheumatic disease. METHODS: In a matched case-control study of women without rheumatic disease, identified from a familial breast cancer cohort, 50 breast cancer cases and 50 controls were assayed for 3 autoantibodies that are known markers of cancer-associated scleroderma and dermatomyositis: anti-RNA polymerase III, anti-NXP2, and anti-TIF1γ...
June 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628472/ifn-%C3%AE-cxcl16-upar-potential-biomarkers-for-systemic-lupus-erythematosus
#3
Si Wen, Fang He, Xuejing Zhu, Shuguang Yuan, Hong Liu, Lin Sun
OBJECTIVES: IFN-γ, CXCL16 and uPAR have recently been regarded as potential biomarkers in systemic lupus erythematosus (SLE). However, few researches have focused on the comparison of these three markers in SLE. We conducted this study to evaluate their role as biomarkers of disease activity and renal damage. METHODS: We enrolled 50 SLE patients with or without lupus nephritis (LN) and 15 healthy control subjects. The levels of IFN-γ, CXCL16, uPAR in serum, urine and renal tissues were detected by ELISA or immunohistochemistry...
June 16, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628470/vascularity-of-nail-bed-by-ultrasound-to-discriminate-psoriasis-psoriatic-arthritis-and-healthy-controls
#4
Sibel Zehra Aydin, Concepción Castillo-Gallego, Zoe R Ash, Helena Marzo-Ortega, Richard Wakefield, Dennis McGonagle
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June 16, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628469/six-joint-ultrasound-in-rheumatoid-arthritis-a-feasible-approach-for-implementing-ultrasound-in-remission
#5
Sibel Zehra Aydin, Esen Kasapoglu Gunal, Merve Ozata, Havva Keskin, Ayse Bilge Ozturk, Paul Emery, Maria Antonietta D'Agostino
OBJECTIVES: Subclinical disease activity in rheumatoid arthritis (RA) detected by imaging methods is predictive for flares and damage. Lack of time is the major limitation for not screening for subclinical disease in routine practice. We aimed to determine the most feasible protocol to screen patients with no clinical disease activity by ultrasound (US). METHODS: A hundred consecutive RA patients with no clinical activity according to the physician had an US scan for 38 joints...
June 16, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628468/dapsa-das28-and-mda-predict-long-term-treatment-regime-in-psoriatic-arthritis-the-swedish-early-psoriatic-arthritis-cohort
#6
Ulla Lindqvist, Mona-Lisa Wernroth, Tomas Husmark, Per Larsson, Mats Geijer, Annika Teleman, Elke Theander, Gerd-Marie Alenius
OBJECTIVES: To describe treatment patterns in the Swedish early psoriatic arthritis cohort (SwePsA) of the mono-/oligo-arthritic (M/O) and polyarthritis (P) and identify early predictive factors for treatment with disease-modifying anti-rheumatic (DMARD), non-steroidal anti-inflammatory drugs (NSAID), and tumour necrosis factor inhibition (TNFi) after 5 years. METHODS: Data for 198 M/O and P PsA were obtained within the programme for SwePsA. Multinomial and binary logistic regression analyses were used to assess the association between early predictive factors and treatment after 5 years adjusted for age at inclusion...
June 16, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#7
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28628465/cardiopulmonary-involvement-in-takayasu-s-arteritis
#8
David N Brennan, Kenneth J Warrington, Cynthia S Crowson, Jean Schmidt, Matthew J Koster
OBJECTIVES: To evaluate cardiopulmonary (CP) involvement in patients with Takayasu's arteritis (TAK) and assess the impact on disease outcomes. METHODS: A retrospective cohort of patients with newly diagnosed TAK from 1984 to 2009 was assembled. Demographics, baseline disease characteristics, relapse events, surgeries and mortality were abstracted from direct medical record review. Angiograms, advanced imaging and cardiac studies were reviewed for evidence of CP involvement...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598782/serum-thyroxine-levels-are-associated-with-increased-systolic-pulmonary-arterial-pressure-in-systemic-sclerosis
#9
Gian Luca Bagnato, Rosaria Maddalena Ruggeri, Rosaria Certo, Maurizio Cusma' Piccione, Scipione Carerj, Willian Neal Roberts
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June 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598780/the-liver-in-familial-mediterranean-fever-is-it-involved
#10
REVIEW
Eldad Ben-Chetrit, Hasan Yazici
OBJECTIVES: Familial Mediterranean fever (FMF) is characterised by recurrent attacks of fever and serositis. It may affect the peritoneum, pleura, synovia and the skin. Usually the liver is intact in FMF. Recently, this concept was challenged by some groups which claimed that hepatitis is a feature of FMF and that non-alcoholic liver disease (NAFLD) and cryptogenic cirrhosis are more common among FMF patients. Scope of this paper is to critically review the relevant literature and to answer the question whether or not the liver is involved in FMF...
June 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598778/uncommon-presentation-of-granulomatosis-with-polyangiitis-in-association-with-subclinical-coeliac-disease
#11
Paraskevi V Voulgari, Fotini Apostolou, Evangelia Dounousi, George Liapis, Moises Elisaf, Alexandros A Drosos
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June 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598773/beh%C3%A3-et-s-syndrome-and-health-related-quality-of-life-influence-of-symptoms-lifestyle-and-employment-status
#12
Amal A Senusi, Dennis Ola, Jan Mather, John Mather, Farida Fortune
OBJECTIVES: Behçet's syndrome (BS) is a chronic multisystemic disorder. The complex pattern of BS symptoms can effect negatively on patients' quality of life. The aim of this study is to evaluate the influence of BS symptoms, oral health related lifestyles and employment status on Health Related Quality of Life (HRQoL). METHODS: A questionnaire was mailed to a cohort of 641 adult members of the Behçet's Syndrome Society (BSS) and patients attending a Behçet's syndrome centre in the UK...
June 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598784/a-pilot-study-using-high-frequency-ultrasound-to-measure-digital-ulcers-a-possible-outcome-measure-in-systemic-sclerosis-clinical-trials
#13
Michael Hughes, Tonia Moore, Joanne Manning, Graham Dinsdale, Ariane L Herrick, Andrea Murray
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June 7, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598787/severe-adverse-drug-reactions-to-biological-disease-modifying-anti-rheumatic-drugs-in-elderly-patients-with-rheumatoid-arthritis-in-clinical-practice
#14
Leticia Leon, Alejandro Gomez, Cristina Vadillo, Esperanza Pato, Luis Rodriguez-Rodriguez, Juan Angel Jover, Lydia Abasolo
OBJECTIVES: Biological DMARDs are widely used in the treatment of rheumatoid arthritis (RA) but their relationship with adverse drug reaction (ADR) is important. RA is now known to increase in incidence and prevalence with age. Our objective was to assess the incidence of severe ADR in the long term, compare safety between the different bDMARDs and identify other possible risk factors for severe ADR in elderly RA patients. METHODS: A 14-year retrospective longitudinal study was performed...
June 6, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598777/no-histologic-evidence-of-foetal-cardiotoxicity-following-exposure-to-maternal-hydroxychloroquine
#15
Deborah Friedman, Leif Lovig, Marc Halushka, Robert M Clancy, Peter M Izmirly, Jill P Buyon
It is currently recommended that hydroxychloroquine (HCQ) be maintained during pregnancy in patients with systemic lupus erythematosus. Recent data suggest that this Toll-like receptor inhibitor may also reduce the recurrence rate of anti-SSA/Ro associated congenital heart block (CHB). This case report describes a unique situation in which a CHB-afflicted, HCQ-exposed pregnancy was electively terminated. The heart did not reveal any characteristic features of cardiotoxicity, providing further evidence supporting the safety of foetal exposure to HCQ...
June 6, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598776/expression-of-the-genes-facilitating-methotrexate-action-within-subcutaneous-rheumatoid-nodules
#16
Emma L Houlder, Melanie J Millier, John Highton, David Gwynne-Jones, Lisa K Stamp, Paul A Hessian
OBJECTIVES: We sought further understanding of the association between methotrexate (MTX) therapy and accelerated development of subcutaneous rheumatoid nodules. The objective was to establish expression of genes involved in the transport, metabolism, and mechanism of action of MTX within nodule tissue. We also examined for differences in gene expression between nodules from patients actively receiving MTX compared to those not receiving MTX. METHODS: Subcutaneous nodule tissues (n=23) were obtained from 21 patients with RA, undergoing elective surgery...
June 6, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598774/what-could-we-learn-from-the-sub-analysis-of-a-single-nation-cohort-in-a-worldwide-study-lessons-from-the-results-observed-in-the-italian-cohort-of-the-go-more-trial
#17
Roberto Giacomelli, Piero Ruscitti, Stefano Bombardieri, Giovanna Cuomo, Salvatore De Vita, Mauro Galeazzi, Monica Mecchia
OBJECTIVES: GO-MORE Trial investigated the use of Golimumab (GLM) in 3280 rheumatoid arthritis (RA) patients worldwide. At present, the burden of arthritis is greater in poorer countries than in developed countries due to socioeconomic disparities, thus suggesting the usefulness of subgroup investigations. We aimed to evaluate GLM as add-on therapy for RA patients in the Italian cohort of GO-MORE trial and compared the clinical characteristics between Italian patients and the enrolled patients worldwide...
June 6, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598786/prevalence-and-risk-factors-for-liver-fibrosis-detected-by-transient-elastography-or-shear-wave-elastography-in-inflammatory-arthritis-a-systematic-review
#18
Azin Rouhi, Glen Hazlewood, Abdel-Aziz Shaheen, Mark G Swain, Claire E H Barber
OBJECTIVES: Emerging technologies for monitoring subclinical liver fibrosis include transient elastography (TE) and shear wave elastography (SWE). A systematic review was conducted to assess the prevalence and report on predictors of liver fibrosis as detected by these technologies in inflammatory arthritis (IA) patients, including rheumatoid arthritis, spondyloarthritis and juvenile idiopathic arthritis. METHODS: MEDLINE, EMBASE and Web of Science were searched from inception to 06/27/2016 using search terms for IA or DMARDs and TE/SWE...
June 5, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598783/anti-homocitrullinated-protein-antibody-isotype-usage-in-rheumatoid-arthritis-and-their-unaffected-first-degree-relatives
#19
David A Bell, Sallie Elhayek, Ewa Cairns, Lillian Barra
OBJECTIVES: The majority of rheumatoid arthritis (RA) patients express anti-citrullinated protein antibodies (ACPA). Unaffected first-degree relatives of RA patients (FDR) also express ACPA, commonly of the IgA isotype. IgG anti-homocitrullinated/carbamylated protein antibodies (AHCPA) have been detected in both RA and FDR. It is unknown whether other isotypes are expressed. We aim to investigate the AHCPA isotype profile in unaffected FDR of RA patients. METHODS: The enrolled subjects were examined by a rheumatologist...
June 5, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28598781/health-care-utilisation-before-and-after-intensive-care-unit-admission-in-rheumatoid-arthritis
#20
Ruth Ann Marrie, Carol A Hitchon, Christine A Peschken, Hui Chen, Charles N Bernstein, Allan Garland
OBJECTIVES: We evaluated the incidence of intensive care unit (ICU) admission in a rheumatoid arthritis (RA) population according to health care utilisation and use of immune therapies in the year preceding admission. Also, we compared health care utilisation after ICU admission in persons with and without RA. METHODS: We identified all persons with RA in Manitoba, Canada using population-based administrative data, and controls matched by age, sex, and region of residence...
June 5, 2017: Clinical and Experimental Rheumatology
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