June 24, 2019
2 min read

Glucocorticoid discontinuation plan recommended for juvenile-onset dermatomyositis

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A group of international researchers writing in Pediatric Rheumatology have proposed a plan for tapering and discontinuing glucocorticoids in patients with juvenile-onset dermatomyositis, based on changes in core set measures of disease activity in the first 6 months of treatment.

“Therapeutic approaches for adult patients with [dermatomyositis] are not standardized, while those for children are essentially based on consensus and literature revision,” Gabriella Giancane, MD, of the IRCCS Istituto Giannina Gaslini, Pediatric Clinic in Genoa, Italy, and colleagues wrote. “While [glucocorticoids] still remain the mainstay of initial and long-term treatment in new-onset [juvenile dermatomyositis] despite their known adverse effects, a still open question is how to taper and discontinue [glucocorticoids] in [juvenile dermatomyositis] patients.”

To develop an evidence-based plan for tapering and discontinuing glucocorticoids in patients with juvenile dermatomyositis, and to identify predictors of discontinuation and clinical remission, Giancane and colleagues analyzed data from the international, multicenter, randomized PRINTO trial. A total of 139 patients with new onset juvenile dermatomyositis were randomized into one of three arms, where they were treated with either prednisone alone, prednisone in combination with methotrexate or prednisone with cyclosporine.

Researchers writing in Pediatric Rheumatology have proposed a plan for tapering and discontinuing glucocorticoids in patients with juvenile-onset dermatomyositis.
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The researchers compared juvenile dermatomyositis core set measures from PRINTO, the American College of Rheumatology and EULAR, as well as median absolute and relative percent changes over time, across three groups. Group one included 30 patients who achieved clinical remission and ceased prednisone treatment with no major therapeutic changes. Considered the reference group, patients in group one were compared with those who failed to achieve remission, which included 43 participants with major therapeutic changes in group two, and 66 without changes in group three.

According to the researchers, based on median changes in core set measures reported in patients in group one, prednisone could be tapered from 2 mg/kg to 1 mg/kg per day in the first 2 months from disease onset if any of the measures decreased by 50% to 94%. In addition, tapering can occur from 1 mg/kg to 0.2mg/kg per day in the following 4months if any core set measures further decrease by 8% to 68%. This can be followed by discontinuation over the next 18months.

The researchers also found that PRINTO 50-70-90 responses after two months of treatment, an age at onset of older than9years and combination therapy with prednisone and methotrexate increase the likelihood of clinical remission in juvenile dermatomyositis (P <.05).

“This study provides a steroid tapering plan in new-onset [juvenile dermatomyositis] patients,” Giancane and colleagues wrote. “In particular, we propose evidence-based specific quantitative cut-offs for glucocorticoids tapering/discontinuation based on the change in the [core set measure] in the initial 6months of treatment as well as in the overall response to treatment and we identify early predictors of remission, to be used in daily practice and in future clinical trials by pediatric rheumatologists.” – by Jason Laday

Disclosures: Giancane reports no relevant financial disclosures. Please see the full study for additional author disclosures.