Age-dependent inhibition of ectopic calcification: A possible role for fetuin-A and osteopontin in patients with juvenile dermatomyositis with calcinosis

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Abstract

Objectives. To assess if age and/or age-dependent variations in the levels of two major calcification regulatory proteins, fetuin-A and osteopontin, could be associated with an increased risk of calcinosis in children with juvenile dermatomyositis (JDM). Methods. The frequency of calcinosis was derived from a national UK database of 212 cases of JDM. Serum fetuin-A and plasma osteopontin levels were determined using ELISA in 15 JDM patients with calcinosis and 15 JDM patients without calcinosis. Healthy controls were 19 age-matched children, 24 adolescents and 13 adults. Sixteen patients with juvenile idiopathic arthritis (JIA) were additional paediatric disease controls. Results. Of the 212 JDM cases 10% had calcinosis. Calcinosis patients had younger age of disease onset than those without calcinosis (mean age of 5.3 yrs vs 7.1 yrs, respectively, P = 0.016). No significant difference in fetuin-A or osteopontin could be detected between the two JDM groups. Fetuin-A levels in all groups of children and the adolescent group were much lower than described previously in adults, and there was a significant positive correlation between age and fetuin-A level, and also between osteopontin levels in plasma and serum fetuin-A. Conclusions. Children who develop JDM at an younger age may have increased risk of developing calcinosis. Physiologically low levels of fetuin-A in young children combined with an additional negative acute-phase effect on fetuin-A due to chronic inflammation could explain in part the propensity to develop ectopic calcification observed in JDM patients, and why calcinosis is less frequent in adults with dermatomyositis. © The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.

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Marhaug, G., Shah, V., Shroff, R., Varsani, H., Wedderburn, L. R., Pilkington, C. A., & Brogan, P. A. (2008). Age-dependent inhibition of ectopic calcification: A possible role for fetuin-A and osteopontin in patients with juvenile dermatomyositis with calcinosis. Rheumatology, 47(7), 1031–1037. https://doi.org/10.1093/rheumatology/ken136

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