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Woerden, Netherlands

Fransen J.,Radboud University Nijmegen | Popa-Diaconu D.,Radboud University Nijmegen | Hesselstrand R.,Lund University | Carreira P.,Hospital de 12 Octobre | And 17 more authors.
Annals of the Rheumatic Diseases

Objective: Systemic sclerosis (SSc) is associated with a signifi cant reduction in life expectancy. A simple prognostic model to predict 5-year survival in SSc was developed in 1999 in 280 patients, but it has not been validated in other patients. The predictions of a prognostic model are usually less accurate in other patients, especially from other centres or countries. A study was undertaken to validate the prognostic model to predict 5-year survival in SSc in other centres throughout Europe. Methods: A European multicentre cohort of patients with SSc diagnosed before 2002 was established. Patients with SSc according to the preliminary American College of Rheumatology classifi cation criteria were eligible for the study when they were followed for at least 5 years or shorter if they died. The primary outcome was 5-year survival after diagnosis of SSc. The predefi ned prognostic model uses the following baseline variables: age, gender, presence of urine protein, erythrocyte sedimentation rate (ESR) and carbon monoxide diffusing capacity (DLCO). Results: Data were available for 1049 patients, 119 (11%) of whom died within 5 years after diagnosis. Of the patients, 85% were female, the mean (SD) age at diagnosis was 50 (14) years and 30% were classifi ed as having diffuse cutaneous SSc. The prognostic model with age (OR 1.03), male gender (OR 1.93), urine protein (OR 2.29), elevated ESR (1.89) and low DLCO (OR 1.94) had an area under the receiver operating characteristic curve of 0.78. Death occurred in 12 (2.2%) of 509 patients with no risk factors, 45 (13%) of 349 patients with one risk factor, 55 (33%) of 168 patients with two risk factors and 7 (30%) of 23 patients with three risk factors. Conclusion: A simple prognostic model using three disease factors to predict 5-year survival at diagnosis in SSc showed reasonable performance upon validation in a European multicentre study. Source

Fransen J.,Radboud University Nijmegen | Johnson S.R.,University of Toronto | Van Den Hoogen F.,Maartenskliniek | Baron M.,McGill University | And 33 more authors.
Arthritis Care and Research

Objective. Classification criteria for systemic sclerosis (SSc; scleroderma) are being updated. Our objective was to select a set of items potentially useful for the classification of SSc using consensus procedures, including the Delphi and nominal group techniques (NGT). Methods. Items were identified through 2 independent consensus exercises performed by the Scleroderma Clinical Trials Consortium and the European League Against Rheumatism Scleroderma Trials and Research Group. The first-round items from both exercises were collated and redundancies were removed, leaving 168 items. A 3-round Delphi exercise was performed using a 1-9 scale (where 1 = completely inappropriate and 9 = completely appropriate) and a consensus meeting using NGT was conducted. During the last Delphi round, the items were ranked on a 1-10 scale. Results. In round 1, 106 experts rated the 168 items. Those with a median score of <4 were removed, resulting in a list of 102 items. In round 2, the items were again rated for appropriateness and subjected to a consensus meeting using NGT by European and North American SSc experts (n = 16), resulting in 23 items. In round 3, SSc experts (n = 26) then individually scored each of the 23 items in a last Delphi round using an appropriateness score (1-9) and ranking their 10 most appropriate items for the classification of SSc. Presence of skin thickening, SSc-specific autoantibodies, abnormal nailfold capillary pattern, and Raynaud's phenomenon ranked highest in the final list that also included items indicating internal organ involvement. Conclusion. The Delphi exercise and NGT resulted in a set of 23 items for the classification of SSc that will be assessed for their discriminative properties in a prospective study. © 2012, American College of Rheumatology. Source

Hoogeboom T.J.,Sint Maartenskliniek | Hoogeboom T.J.,Maastricht University | Kwakkenbos L.,Sint Maartenskliniek | Rietveld L.,Maartenskliniek | And 3 more authors.
BMJ Open

Objectives: To evaluate the feasibility and potential effectiveness of a 12-week, non-pharmacological multidisciplinary intervention in patients with generalised osteoarthritis (GOA). Design: A randomised, concurrent, multiple-baseline single-case design. During the baseline period, the intervention period and the postintervention period, all participants completed several health outcomes twice a week on Visual Analogue Scales. Setting: Rheumatology outpatient department of a specialised hospital in the Netherlands. Participants: 1 man and four women (aged 51-76 years) diagnosed with GOA. Primary outcome measures: To assess feasibility, the authors assessed the number of dropouts and adverse events, adherence rates and patients' satisfaction. Secondary outcome measures: To assess the potential effectiveness, the authors assessed pain and self-efficacy using visual data inspection and randomisation tests. Results: The intervention was feasible in terms of adverse events (none) and adherence rate but not in terms of participants' satisfaction with the intervention. Visual inspection of the data and randomisation testing demonstrated no effects on pain (p=0.93) or self-efficacy (p=0.85). Conclusions: The results of the present study indicate that the proposed intervention for patients with GOA was insufficiently feasible and effective. The data obtained through this multiple-baseline study have highlighted several areas in which the therapy programme can be optimised. Source

Tsoi K.L.,University Utrecht | Custers M.,Maartenskliniek | Bij De Vaate L.,Zuwe Hofpoort Ziekenhuis | Jacobs J.W.G.,University Utrecht
BMJ Case Reports

A 57-year-old woman presented with malaise and heaviness in her extremities. At first there were no clues of an infammatory disease, but the patient developed slowly progressive oedema of her arms and legs with induration of the skin. Blood tests showed eosinophilia. Additional analysis revealed generalised lymphadenopathy. After excluding an infectious or malignant cause, the clinical diagnosis of eosinophilic fasciitis was made, this was confirmed by the results of a full thickness skin biopsy. Copyright 2012 BMJ Publishing Group. All rights reserved. Source

Arts E.E.A.,Radboud University Nijmegen | Popa C.,Radboud University Nijmegen | Den Broeder A.A.,Maartenskliniek | Semb A.G.,Diakonhjemmet Hospital | And 4 more authors.
Annals of the Rheumatic Diseases

Objective: This study was undertaken to assess the predictive ability of 4 established cardiovascular (CV) risk models for the 10-year risk of fatal and non-fatal CV diseases in European patients with rheumatoid arthritis. Methods: Prospectively collected data from the Nijmegen early rheumatoid arthritis (RA) inception cohort was used. Discriminatory ability for CV risk prediction was estimated by the area under the receiver operating characteristic curve. Calibration was assessed by comparing the observed versus expected number of events using Hosmer-Lemeshov tests and calibration plots. Sensitivity and specificity were calculated for the cut-offvalues of 10% and 20% predicted risk. Results: Areas under the receiver operating characteristic curve were 0.78-0.80, indicating moderate to good discrimination between patients with and without a CV event. The CV risk models Systematic Coronary Risk Evaluation (SCORE), Framingham risk score (FRS) and Reynolds risk score (RRS) primarily underestimated CV risk at low and middle observed risk levels, and mostly overestimated CV risk at higher observed risk levels. The QRisk II primarily overestimated observed CV risk. For the 10% and 20% cut-offvalues used as indicators for CV preventive treatment, sensitivity ranged from 68-87% and 40-65%, respectively and specificity ranged from 55-76% and 77-88%, respectively. Depending on the model, up to 32% of observed CV events occurred in patients with RA who were classified as low risk (<10%) for CV disease. Conclusions: Established risk models generally underestimate (Systematic Coronary Risk Evaluation score, Framingham Risk Score, Reynolds risk score) or overestimate (QRisk II) CV risk in patients with RA. © 2014 BMJ Publishing Group Ltd & European League Against Rheumatism. Source

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