Juvenile Myositis

Juvenile Dermatomyositis (JDM) and Juvenile Polymyositis (JPM)

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Overview

Juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) are rare autoimmune diseases that affect muscle and skin as well as other organ systems. Duke pediatric rheumatologists are experts in treating children affected with these diseases and are actively conducting research into these conditions. We help children live normal, active lives.

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Tests

Tests Overview

A prompt, precise diagnosis is key to getting juvenile dermatomyositis and juvenile polymyositis under control and preventing debilitating complications such as muscle weakness and loss of mobility. Your child's pediatric rheumatologists will perform a variety of tests to rule out other illnesses, confirm the presence of the autoimmune disorder, and assess muscle function.

Physical Exam

Description

Your child’s doctor will look for signs of inflammatory muscle disease, such as muscle weakness and rashes on the eyelids, knuckles, elbows, and knees. The physical exam findings may differ between JDM and JPM; however, most cases of myositis in children are due to JDM, with skin and muscle involvement.

Blood Tests

Description

Blood tests can detect whether the immune system is attacking itself and whether inflammation is present. They can also identify whether enzymes that signal muscle damage are elevated and whether there is additional organ involvement.

Swallowing Study

Description

A swallow study is performed if muscle weakness affects your child’s ability to swallow properly or safely.

Pulmonary Function Tests (PFTs)

Description

Muscles involved in breathing can be weakened by inflammatory disease. PFTs can measure the degree of weakness.

Electromyography

Description

Also known as EMG, this electrical test evaluates muscle damage.

MRI

Description

This imaging exam can be useful in evaluating muscle inflammation when considering a diagnosis of juvenile myositis.

Muscle Biopsy

Description

A small piece of muscle is removed and examined under a microscope to look for signs of inflammation and damage.

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Duke Health offers locations throughout the Triangle. Find one near you.

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Treatments

Treatments Overview

Your child may need one or more medications to control their body’s immune response, relieve symptoms, or treat complications. Many of these medications suppress the immune system. Our goal is to put your child’s disease into remission and to maintain that remission for a prescribed period of time. We then wean your child to a lower dose or off medication while ensuring that we maintain the disease remission.

Standard Therapy

Description

Corticosteroids and methotrexate are standard therapy in treating JDM and JPM. Corticosteroids are used to calm the immune system at the onset of the disease; your child is weaned once remission is achieved. Methotrexate is a long-term maintenance medication.

Intravenous Immunoglobulin (IVIg) and Rituxan

Description

When standard therapy is not enough to attain remission, additional medications can be effective in calming disease activity and progression. Intravenous immunoglobulin (IVIg) and Rituxan are proteins that are given intravenously. IVIg is immunoglobulin -- antibodies in the blood, collected from healthy donors -- that will bind to and remove proteins (antibodies) made by your child's body that are causing disease. Rituxan is a man-made antibody that temporarily prevents your child's body from producing the antibodies that are causing disease.

Additional medications are available. We will explain the risks and benefits of any treatment and answer your questions and concerns before starting your child on a treatment plan.

Pediatric Physical and Occupational Therapists (PT/OT)

Description

Physical and occupational therapists work with your child to improve their strength and mobility. They also help your child learn ways to perform everyday tasks while they recover from their weakness.

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855-855-6484

Why Choose Duke

A Designated Cure JM Center of Excellence
Our decades of experience managing juvenile dermatomyositis, juvenile polymyositis, and other inflammatory myopathies is recognized by our designation as a Cure JM Center of Excellence.

A Team of Pediatric Specialists
In addition to a pediatric rheumatologist, your child will benefit from the care provided by a team of experts that includes metabolic and genetic specialists, a neuromuscular neurologist, physical and occupational therapists, pulmonary specialists, dermatologists, and others. Together, they will diagnose and manage your child’s condition and the complications associated with the disease.

Research Leaders
Our team includes internationally recognized researchers whose work focuses on better understanding the cause of these diseases and identifying factors that can determine which children will respond to standard therapy and which may not.

Transition to Adult Care
Adolescents participate in a transition program that introduces them to the self-care skills they will need as they become young adults with a chronic disease. At the same time, they will transition their care to an adult rheumatologist.

Best Children's Hospital in NC

Duke Children's Hospital & Health Center is proud to be nationally ranked in 10 pediatric specialties.

This page was medically reviewed on 03/07/2019