RANKL: Osteoprotegerin ratio and bone mineral density in children with untreated juvenile dermatomyositis

Kelly A. Rouster-Stevens, Craig B. Langman, Heather E. Price, Roopa Seshadri, Richard M. Shore, Kathy Abbott, Lauren M. Pachman*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


Objective. To determine bone mineral density (BMD) in patients at the time of diagnosis of juvenile dermatomyositis (DM), to compare the RANKL: osteoprotegerin (OPG) ratio in patients with juvenile DM with that in healthy control subjects, and to evaluate whether BMD is associated with the RANKL:OPG ratio and the duration of untreated disease. Methods. Thirty-seven children with juvenile DM were enrolled. Dual x-ray absorptiometry (DXA) was performed before treatment, and Z scores for the lumbar spine (L1-L4) were determined. The duration of untreated disease was defined as the period of time from the onset of rash or weakness to the time at which DXA was performed. Serum specimens obtained at the time of DXA were analyzed for concentrations of RANKL and OPG, using enzyme-linked immunosorbent assay. The RANKL:OPG ratio was also determined in 44 age-matched healthy control subjects. Results. At the time of diagnosis of juvenile DM, patients had a significantly increased RANKL: OPG ratio compared with that in healthy children (mean ± SD 2.19 ± 3.03 and 0.13 ± 0.17, respectively; P < 0.0001). In patients with a lumbar spine BMD Z score of -1.5 or lower, the RANKL:OPG ratio was significantly higher than that in patients with a lumbar spine BMD Z score higher than -1.5 (P = 0.038). Lumbar spine BMD Z scores (mean ± SD -0.13 ± 1.19 [range -2.10 to 2.85]) were inversely associated with the duration of untreated disease (R = -0.50, P = 0.003). Conclusion. Children with juvenile DM have an elevated RANKL:OP6 ratio at the time of diagnosis, resulting in expansion of the number of osteoclasts and activation of the bone resorptive function. This may lead to a lack of normal bone mineral accretion and a subsequent reduction in the lumbar spine BMD Z score. Patients with a longer duration of untreated juvenile DM have reduced lumbar spine BMD Z scores. These data suggest that early diagnosis could reduce the likelihood of reduced lumbar spine BMD in these patients by prompting intervention strategies at an early stage.

Original languageEnglish (US)
Pages (from-to)977-983
Number of pages7
JournalArthritis and rheumatism
Issue number3
StatePublished - Mar 2007

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Pharmacology (medical)

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