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NEWSLETTER
Fall, 2019

Videos from the 2019 Cure JM Medical Conference

In June, healthcare professionals and JM researchers from around the world convened at Ann & Robert H. Lurie Children's Hospital of Chicago for Cure JM's 2019 Update on Juvenile Myositis Care and Research. Attendees included pediatricians, rheumatologists, dermatologists, and neurologists.

Learning objectives included:

  • Identifying best practices in diagnosing and treating juvenile myositis (JM) patients.
  • Applying latest developments in myositis research to clinical care.
  • Examining specialized management issues relevant to JM patients.
  • Discussing JM case studies to make treatment plans to optimize care.

Cure JM Medical Conference YouTube Rlaylist screen

This YouTube playlist has videos from the day’s presentations, including: Immunology of JDM, Skin Disease and JDM, Natural Killer Cells in JDM, Radiologic Aspects of JDM, Exercise as Medicine for Childhood Myositis, Therapy of JDM, Past, Present, and Emerging, and two case studies.


JAK Inhibitors: Beyond Rheumatoid Arthritis

Dr. Lucy Wedderburn

Lucy R. Wedderburn's PhD., MRCP

At the recent American College of Rheumatology’s Annual Conference, Lucy Wedderburn, PhD., MRCP, a member of Cure JM’s Medical Advisory Board and Director of the Center for Adolescent Rheumatology at University College London, presented to a packed lecture hall on "JAK Inhibitors: Beyond Rheumatoid Arthritis." Dr. Wedderburn presented on the over a decade of research detailing juvenile myositis (JM) as an interferonopathy, and she and her colleagues on the discussion panel made a strong case for the potential of JAK inhibitors in treating rare pediatric autoimmune diseases such as lupus and JM.

JAK inhibitors are a relatively new class of drug known as biologics, such as baricitinib (Olumiant), tofacitinib (Xeljanz), abatacept (Orencia), and several others, often approved for rheumatoid arthritis. These drugs are generally well tolerated and have minimal side effects.

Dr. Wedderburn cited the effectiveness of tofacitinib in two patients with anti-MDA-5 autoantibodies who saw moderate improvement and lower disease activity in their muscles, skin, and other target organs. Both of these patients had remarkable improvement in lung disease measures, and one patient also saw improvements in peripheral calcinosis. Both patients demonstrated significant decrease in IFN scores (overexpression of interferons) to levels comparable to healthy controls. The overexpression of interferons leads to muscle weakness.

Both of these patients were able to reduce or discontinue prednisone therapy and other immunosuppressive medications in the course of treatment. The first author of the tofacitinib poster cited by Dr. Wedderburn is Sara Sabbagh, D.O., whose fellowship is being supported by Cure JM. The principal investigator is Cure JM Medical Advisory Board Chair, Lisa Rider, MD.


Top Five Signs That Lead to a Diagnosis of Juvenile Dermatomyositis

The main form of juvenile myositis is juvenile dermatomyositis (JDM). Up to 85% of all patients with juvenile myositis have JDM, so it’s important to recognize the major symptoms of JDM, so it can be properly diagnosed.

Here are the top five indicators that the patient has probable JDM. The presence of three of the five findings indicates that myositis is the probable diagnosis, and at least four of five findings indicates definite myositis. Dermatomyositis requires skin changes (#5).

Top 5 Signs of JDM

 TWEET THIS GRAPHIC 

Sources: Lisa G. Rider, MD, Lauren M. Pachman, MD, Frederick W. Miller, PhD, MD, Harriet Bollar, "Myositis and You: A Guide to Juvenile Dermatomyositis for Patients, Families, and Healthcare Providers." 2007. Chapter, "The First Signs of Illness" by Lauren M. Pachman, MD, and Carol B. Lindsley, MD.
Bohan A, Peter JB. Polymyositis and dermatomyositis. New England Journal of Medicine. 1975 Feb 13;292(7):344–47.