This protocol applies to patients under 18 with juvenile dermatomyositis who do not have major organ involvement or extensive ulcerative skin disease, and in whom the initial treatment course did not achieve the defined clinical targets.
Age under 18 years. Juvenile dermatomyositis without major organ involvement and without extensive ulcerative skin disease. Inadequate response to the initial therapy line at the 12-week assessment point.
The initial line — high-dose corticosteroids combined with methotrexate (or an alternative DMARD where methotrexate was not tolerated) — did not achieve sufficient clinical improvement. Three of the following four targets were not met at 12 weeks:
Failure to fulfil at least three of four clinically inactive-disease criteria (CPK ≤150 U/L, CMAS ≥48, MMT8 ≥78, PGA ≤0.2) triggers escalation to this next-line protocol.
The clinical targets remain the same measurable benchmarks: meaningful gains in CMAS and MMT8 scores, reduction in serum CPK, and improvement in physician-rated disease activity. Clinically inactive disease requires meeting at least three of four defined criteria. Response timing varies by agent — some may require up to 26 weeks before full effect is seen.
DOI: 10.1136/annrheumdis-2016-209247
Intravenous immunoglobulin may be a useful adjunct for resistant disease, particularly when skin features are prominent.
MMF may be a useful therapy for muscle and skin disease (including calcinosis).
B cell depletion therapy (rituximab) can be considered as an adjunctive therapy for those with refractory disease.
Anti-TNF therapies can be considered in refractory disease; infliximab or adalimumab are favoured over etanercept.
Clinicians should be aware that rituximab can take up to 26 weeks to work.
In 2012, PRINTO published criteria defining clinically inactive disease; necessitating fulfilment of three out of four variables from CPK ≤150 U/L, CMAS ≥48, MMT8 ≥78 and PGA score of overall disease activity ≤0.2.
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