Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/71740
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Type: Journal article
Title: Prevalence, correlates and clinical usefulness of antibodies to RNA polymerase III in systemic sclerosis: a cross-sectional analysis of data from an Australian cohort
Author: Nikpour, M.
Hissaria, P.
Byron, J.
Sahhar, J.
Micallef, M.
Paspaliaris, W.
Roddy, J.
Nash, P.
Sturgess, A.
Proudman, S.
Stevens, W.
Citation: Arthritis Research & Therapy, 2011; 13(6):1-13
Publisher: BioMed Central Ltd.
Issue Date: 2011
ISSN: 1478-6354
1478-6362
Statement of
Responsibility: 
Mandana Nikpour, Pravin Hissaria, Jillian Byron, Joanne Sahhar, Maree Micallef, William Paspaliaris, Janet Roddy, Peter Nash, Alan Sturgess, Susanna Proudman and Wendy Stevens
Abstract: INTRODUCTION: The prevalence of antibodies to RNA polymerase III (anti-RNAP) differs among systemic sclerosis (SSc) cohorts worldwide. Previously reported associations of anti-RNAP include diffuse cutaneous disease, tendon friction rubs and renal crisis, with recent reports suggesting a close temporal association between malignancy and SSc disease onset among patients with anti-RNAP. METHODS: Patients with SSc were tested for the presence of anti-RNAP at recruitment into the Australian Scleroderma Cohort Study. We used univariate and multivariable methods to identify and quantify clinical and laboratory correlates of anti-RNAP in SSc. Diagnostic testing procedures were used to determine the usefulness of these antibodies in estimating the likelihood of clinically important outcomes. RESULTS: There were 451 patients with mean ± standard deviation age and disease duration at recruitment of 58.1 ± 12.4 and 11.6 ± 10.0 years, respectively; 151 (33.5%) patients were recruited within 5 years of diagnosis of SSc. Overall, 69 (15.3%) patients had anti-RNAP. Univariate associations of anti-RNAP were diffuse disease (75.4% vs. 20.9%, P < 0.0001), joint contractures (73.9% vs. 30.1%, P < 0.0001), greater highest-recorded modified Rodnan skin score (20.6 ± 12.4 vs. 10.1 ± 7.9, P < 0.0001), synovitis (31.9% vs. 19.9%, P = 0.03), myositis (2.9% vs. 0.5%, P = 0.05), systemic hypertension (59.4% vs. 39.7%, P = 0.002), renal crisis (24.6% vs. 1.8%, P < 0.0001) and malignancy diagnosed within 5 years of onset of SSc skin disease (13.3% vs. 3.9%, P = 0.01). In multiple regression analysis, after adjustment for other covariates, anti-RNAP were independently associated with renal crisis (odds ratio (OR) 3.8, 95% confidence interval (CI) 1.2 to 11.5, P = 0.02; positive predictive value (PPV) 24.6%, negative predictive value (NPV) 98.2%), diffuse disease (OR 6.4, 95% CI 2.9 to 13.8, P < 0.0001; PPV 75.4%, NPV 20.9%), joint contractures (OR 2.5, 95% CI 1.2 to 5.3, P = 0.02; PPV 73.9%, NPV 69.9%) and malignancy diagnosed within 5 years of onset of SSc skin disease (OR 4.2, 95% CI 1.3 to 13.4, P = 0.01; PPV 13.3%, NPV 96.1%). CONCLUSIONS: Anti-RNAP status is a clinically useful prognostic marker in SSc and enables clinicians to identify patients at high risk of developing renal crisis, synovitis, myositis and joint contractures. Patients with anti-RNAP also have an increased risk of malignancy within a 5-year timeframe before or after onset of SSc skin changes.
Keywords: Skin; Humans; Scleroderma, Systemic; RNA Polymerase III; Autoantibodies; Enzyme-Linked Immunosorbent Assay; Prevalence; Multivariate Analysis; Logistic Models; Risk Assessment; Risk Factors; Cohort Studies; Cross-Sectional Studies; Aged; Middle Aged; Australia; Female; Male
Description: Extent: 13p.
Rights: © 2012 Nikpour et al.; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
RMID: 0020117105
DOI: 10.1186/ar3544
Appears in Collections:Medicine publications

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