Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/96276
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Type: Journal article
Title: Potential role of the lectin pathway of complement in the pathogenesis and disease manifestations of systemic sclerosis: a case-control and cohort study
Author: Osthoff, M.
Ngian, G.
Dean, M.
Nikpour, M.
Stevens, W.
Proudman, S.
Eisen, D.
Sahhar, J.
Citation: Arthritis Research and Therapy, 2014; 16(1):480-1-480-11
Publisher: BioMed Central
Issue Date: 2014
ISSN: 1478-6354
1478-6362
Statement of
Responsibility: 
Michael Osthoff, Gene-Siew Ngian, Melinda M Dean, Mandana Nikpour, Wendy Stevens, Susanna Proudman, Damon P Eisen, and Joanne Sahhar
Abstract: INTRODUCTION: Repetitive episodes of ischemia and reperfusion (I/R) are a cardinal feature of the pathogenesis of systemic sclerosis (SSc), which precedes tissue fibrosis. The complement system is a key mediator of tissue damage after I/R, primarily by activation of the lectin pathway. This study investigated whether serum levels and polymorphisms of mannose-binding lectin (MBL) and ficolin-2 (FCN2), two pattern recognition receptors of the lectin pathway, are associated with the predisposition to and clinical features of SSc. METHODS: A case-control study was undertaken involving 90 patients with SSc from a single SSc outpatient clinic and 90 age- and sex-matched blood donors. MBL and FCN2 levels and polymorphisms were measured in both groups, and in cases correlated with clinical data. RESULTS: MBL levels and genotypes were equally distributed in cases and controls while there were some significant differences in FCN2 polymorphisms. Median MBL levels were higher in SSc cases with diffuse disease compared with controls (2.6 versus 1.0 μg/ml, P <0.001). CONCLUSIONS: Overall, predisposition to SSc was not influenced by the lectin pathway of complement in our matched case-control study. However, our preliminary data suggest that MBL, and to a lesser extent FCN2, may modulate disease manifestations of SSc, particularly in diffuse cutaneous disease.
Keywords: Humans; Scleroderma, Systemic; Mannose-Binding Lectin; Biological Markers; Case-Control Studies; Cohort Studies; Cross-Sectional Studies; Adult; Aged; Middle Aged; Complement System Proteins; Female; Male
Rights: © 2014 Osthoff 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/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
RMID: 0030024399
DOI: 10.1186/s13075-014-0480-6
Appears in Collections:Medicine publications

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