Consensus treatment plans developed for juvenile dermatomyositis with persistent rash

Cathy Patty-Resk, MSN, RN, CPNP

CARRA should be applauded for coming together to provide guidance to other pediatric rheumatology providers, as well as non-pediatric and non-rheumatology providers who are caring for these children in severely underserved areas.  CARRA has identified a pertinent sub-group of JDM in need of further research. This is the group of patients has residual skin disease after resolution of muscle involvement and fatigue. A less common pediatric small vessel vasculitis, JDM has a lack of proven effective treatments. It is possible this sub-group of patients with residual skin disease may need a different treatment regimen for undiscovered reasons. By more clearly stratifying JDM into three subclasses with CTPs, the goal is to help further advance research for a better understanding of this disease with its three most common presentations, particularly the group with residual skin disease.  There has been little progress in understanding this disease with regard to biologic targets and thus, no new biologics have proven effective. CARRA has set the stage for more organized and meaningful research into currently used JDM treatment protocols, leaving room for an experimental arm to study possible new treatments. These CTPs would mean children have access to better treatments leading to improved treatment outcomes.  The three CTPs are ones that have been used by many pediatric rheumatologists for years, but not necessarily with the group experiencing residual active skin disease. This is the group that may be undertreated. By setting the CTPs, it is more likely providers will continue to seek effective treatment for the residual skin disease instead of ignoring it. However, this is the group of patients who may be tougher to get parental “buy in” for more aggressive pharmaco-management. They often do not see the residual skin disease as problematic or threatening of health status as they did when fatigue, muscle pain and weakness was present.  Another area that may become more problematic is obtaining insurance authorization for off-label use of pharmaceuticals not FDA approved for JDM. In pediatric rheumatology, providers are experiencing this phenomenon more each day. Unfortunately, many of the drugs used in pediatric rheumatology are used off-label or with FDA orphan designation and have been used for years safely with great success. Humira for uveitis in children is a prime example of this because it is only approved for children with JIA and Crohn’s disease.  CARRA did not address the issue of criteria for treatment failure, with this paper stating it will leave it to the provider to determine. This may lead to early treatment withdrawal or not changing the treatment plan when failure has occurred. More experienced practitioners and subspecialty trained practitioners will likely have similar thresholds of failure. However, it is the less experienced, rural or non-rheumatology providers whose failure thresholds will likely differ most. Thus, would most likely affect the children who do not have access to one of the only 300 pediatric rheumatologists in the United States and possibly lead to worse outcomes.