A panel of 19 pediatric rheumatologists and two exercise physiology and physical therapy professionals involved in a European initiative called the Single Hub and Access Point for Pediatric Rheumatology in Europe used evidence-based research to develop a plan for the assessment and management of juvenile dermatomyositis, according to a recently published report.
The researchers identified investigations related to juvenile dermatomyositis (JDM) once in June 2013 and again in February 2015 from the PubMed/MEDLINE, Embase and Cochrane databases and developed a summary of the evidence to the panel. Forty-five articles related to therapy, 70 articles for diagnosis and three articles for both for JDM were selected for evaluation.
Included among the treatment recommendations were the use of the Childhood Myositis Assessment Scale and Manual Muscle Test, MRI to assess muscle inflammation, encouraging a safe exercise program, involvement of a physiotherapist and specialist nurse, and encouraging the use of sunblock. The panel wrote corticosteroids should be tapered as the patient improves, and the addition of methotrexate (preferably subcutaneous) and cyclosporine A can be beneficial.
In the case of inadequate response to treatment within 12 weeks, treatment intensification should be considered, according to the group, and IV immunoglobulin may be considered, particularly with high skin involvement. Topical tacrolimus may be helpful for skin involvement, and mycophenolate mofetil may be helpful in the absence of a response to other disease-modifying rheumatic drugs.
As an adjunctive therapy, B cell depletion may be considered, according to the group, as well as tumor necrosis factor inhibitors in patients with refractory disease, and IV cyclophosphamide for patients with ulcerated skin. While interstitial lung disease (ILD) is present in around 8% of patients, according to the research group, patients should be assessed for ILD with high-resolution CT. -by Shirley Pulawski
Disclosures: Enders reports funding from Valeria e Ettore Bossi Foundation. Please see the full study for a list of all other authors’ relevant financial disclosures.