AI Article Synopsis

  • Biological treatments targeting IL-1β or IL-6 are key for managing systemic juvenile idiopathic arthritis (sJIA) in children, but many still experience ongoing inflammation and growth issues due to high glucocorticoid use.
  • A long-term strategy has shown promise by using a combination of hormonal therapies: delaying puberty with a gonadotropin-releasing hormone analog and promoting growth with growth hormone after inflammation is controlled.
  • This approach aims to normalize adult height in sJIA children by managing puberty and optimizing growth opportunities following serious treatment phases.

Article Abstract

Background: Biologicals targeting the interleukin (IL)-1β or IL-6 pathway are becoming prime choices for the treatment of children with systemic juvenile idiopathic arthritis (sJIA). Up to 1 in 3 sJIA children receiving such treatment continues to have inflammatory activity and to require supra-physiological glucocorticoid doses which may reduce growth velocity for years and may lead to an extremely short stature for age, if not for life. Currently, there is no long-term proposal to normalize the adult height of these children with sJIA.

Methods And Results: We present long-term (up to 10 years), proof-of-concept evidence that the adult stature and adipose body composition of short sJIA children can be normalized with a hormonal combination strategy: (i) pubertal onset is postponed with a gonadotropin-releasing hormone analog (triptorelin) until a minimum height is reached, or until prepubertal growth is exhausted, and (ii) height gain is promoted with growth hormone (≈50 μg/kg/day), once inflammation is under control and high glucocorticoid doses are no longer needed. The latter treatment takes advantage of the window of relative glucocorticoid deficiency, which is known to open after prolonged glucocorticoid administration, and to be uniquely favorable to height gain.

Conclusion: A long-term combination of biological and hormonal treatments for short sJIA children can be guided by a simple concept that involves (i) postponement of pubertal development and (ii) growth-promoting therapy after the episodes of major inflammation and high-dose glucocorticoid treatment. Limited long-term experience in short sJIA children suggests that this strategy leads consistently - albeit late - to a normal adult stature.

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Source
http://dx.doi.org/10.1159/000489778DOI Listing

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