News

SHARE initiative releases consensus-based JDM management recommendations

View on the News

Accurate patient characterization is needed

The consensus-based recommendations for the management of juvenile dermatomyositis published in Annals of Rheumatic Diseases again highlight the ability of the European and Canadian investigators to work together and pool the information from a large number of centers. Similar efforts to pool data and encourage the development of protocols to optimize care are occurring in the United States under the auspices of CARRA (the Childhood Arthritis and Rheumatology Research Alliance).

These working groups represent an important first step toward standardizing optimal care for children with rheumatic diseases. However, the protocols put forth by both groups suffer from failure to address the diversity of presentations within their diseases and a resultant lack of specificity in their recommendations. The authors of these guidelines make specific recommendations regarding diagnosis, but these fail to encompass the range of weakness that may be present initially. Is it truly appropriate to bolus every child diagnosed with dermatomyositis with high-dose corticosteroids and begin methotrexate? No mention is made of the hypertension, pancreatitis, or systemic infection that might result.

The guidelines and protocols being promulgated in pediatric rheumatology continue to suffer from the grouping of children with diverse disease presentations and probably diverse diseases under a single diagnosis. Charles Spencer and colleagues described the highly variable course of juvenile dermatomyositis and the presence of distinct subsets of patients more than 30 years ago (J Pediatr. 1984 Sep;105[3]:399-408), but this diversity is not reflected in the current protocol. Methods for better characterizing our patients based on gene activation and cytokine profiles have been developed. More effort should be placed on accurate characterization of our patients with many diseases before we attempt to treat them all the same way.

Thomas J.A. Lehman, MD, is chief of the division of pediatric rheumatology at the Hospital for Special Surgery, and professor of clinical pediatrics at Weill Medical Center, Cornell University, New York. He has no relevant disclosures.


 

FROM ANNALS OF THE RHEUMATIC DISEASES

References

“It will now be important to broaden discussion and test acceptability of these to the wider community,” they wrote.

The SHARE initiative is funded by a grant from the European Agency for Health and Consumers. Dr. Enders disclosed a relationship with the Valeria e Ettore Bossi Foundation. Her coauthors reported financial or other relationships with Roche/Chugai, AbbVie, Pfizer, Novartis, Bristol-Myers Squibb, SOBI, Medac, The Myositis Association, Neopharm, GlaxoSmithKline, and/or Genzyme.

sworcester@frontlinemedcom.com

Pages

Recommended Reading

Updated Behçet’s disease recommendations expand biologic treatment
MDedge Rheumatology
European ANCA-associated vasculitis guidance gets first makeover since 2009
MDedge Rheumatology
Actemra may reduce scleroderma skin thickening
MDedge Rheumatology
VIDEO: TNF inhibitors improved refractory skin disease in juvenile dermatomyositis
MDedge Rheumatology
HCQ eye toxicity needs experience to assess
MDedge Rheumatology
Mechanism proposed for microvascular thrombosis in thrombotic thrombocytopenic purpura
MDedge Rheumatology
FDA accepting comments on draft guidelines on compounding law
MDedge Rheumatology
Psoriasiform eruptions in Kawasaki disease reveal distinct phenotype
MDedge Rheumatology
Teams boost confidence in IPF diagnoses
MDedge Rheumatology
Benefits, challenges emerge in evolution of rheumatology-dermatology clinics
MDedge Rheumatology