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Juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is an autoimmune disease causing vasculitis that manifests itself in children; it is the pediatric counterpart of dermatomyositis. In JDM, the body's immune system attacks blood vessels throughout the body, causing inflammation called vasculitis. In the United States, the incidence rate of JDM is approximately 3 cases per million children per year, leading to 300 to 500 new cases annually and affecting an estimated 3,000 to 5,000 children. Other forms of juvenile myositis are juvenile polymyositis (JPM) and juvenile inclusion-body myositis (JIBM), which are extremely rare and are not as common in children as in adults. more...

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Juvenile dermatomyositis

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Cause

The underlying cause of JDM is unknown. It most likely has a genetic component, as other auto-immune disease tend to run in the families of patients. The disease is usually triggered by a condition that causes immune system activity that does not stop as it should, but the trigger is almost certainly not the cause in most cases. Common triggers include immunizations, infections, injuries, and sunburn.

Symptoms

The vasculitis caused by JDM manifests itself predominantly in two ways:

One is a distinctive rash. The rash often affects the face, eyelids, and hands, and sometimes the skin above joints, including the knuckles, knees, elbows, etc. The color of the rash is a pinkish purple, and is called Heliotrope (after a flower of the same name with approximately this color). On the hands and face, the rash very closely resembles allergies, eczema, fifth disease, or other more common skin condition, but the heliotrope color is unique to the inflammatory process of JDMS. Some children develop calcinosis, which are calcium deposits under the skin. The rash is the source of the "dermato-" part of the name of the disease.

The second symptom caused by vasculitis is muscle inflammation. This symptom is the source of the "-myositis" part of the name of the disease ("myo" = muscle, "-itis" = inflammation of). Muscle Inflammation causes muscle weakness, which can cause fatigue, clumsiness, not keeping up physically with peers, and eventually inability to perform tasks like climbing stairs, lifting objects, and performing other manual tasks. Other signs may include falling, dysphonia, or dysphagia. The muscle weakness often causes a medical misdiagnosis of muscular dystrophy or other muscle disease. Some patients develop contractures, when the muscle shortens and causes joints to stay bent; exercise can prevent this. The muscles first affected tend to be proximal (i.e., neck, shoulders, back, and abdominal). About half of children with JDM also have pain in their muscles.

Progression

The speed of the progression of JDM is highly variable. Nearly all JDM patients have some skin involvement. The JDM rash usually occurs as the initial symptom. Sometimes it is so slight as not to be recognized for what it is until muscle symptoms appear. Sometimes muscle symptoms never appearing at all or occur very gradually over the course of months, and sometimes going from normal strength to being unable to walk within days. Usually, muscle symptoms appear weeks to months after the onset of the rash.

Diagnosis

JDM is diagnosed by a combination of patient/parent observations, clinical examination, and laboratory blood tests.

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Promising Study Results of Protein Therapeutics' Oralgam in Juvenile Rheumatoid Arthritis to be Reported at American College of Rheumatology Meeting
From Business Wire, 9/22/03

Business Editors/Health/Medical Writers

BIOWIRE2K

TUCSON, Ariz.--(BUSINESS WIRE)--Sept. 22, 2003

A Phase II clinical trial to evaluate the potential of Oralgam(TM) (oral human immunoglobulin)as an add-on therapy to treat juvenile rheumatoid arthritis (JRA) in children 3 to 16 years of age found that the longer patients remained on Oralgam, the more improvement was seen in multiple measures of disease activity. Study results will be presented by Ilona Szer, M.D., at the ACR Annual Meeting held Orlando, Fla., Oct. 24-28.

JRA, a severe and crippling condition, is the most common rheumatic disease in children, resulting in approximately 30,000 to 50,000 patients in the United States, according to the Arthritis Foundation. Drugs initially developed and approved for use in adults are currently being used in children with varying degrees of safety and efficacy. A significant number of children with JRA, however, fail to achieve adequate control of disease symptoms using standard therapies, including methotrexate, prednisone and anti-TNF or anti-IL1 biological response modifiers.

The 40 children with JRA in the Phase II clinical trial sponsored by Protein Therapeutics were selected based on having at least four active joints and inadequate response to standard treatment. Response to treatment was evaluated using the ACR Pediatric 30 criteria.

Findings of the study include:

-- Two-thirds of the children who remained on the drug for at

least four months met the definition of improvement in disease

activity by measures recognized by the American College of

Rheumatology.

-- Regression analysis predicted a 57 percent improvement in

daily disability scores (Childhood Health Assessment

Questionnaire), an 88 percent improvement in active joint

scores, a 69 percent improvement in Patient Global Assessment

scores and a 76 percent improvement in Physician Global

assessment scores over a 12-month period compared to baseline.

-- Steroid use was reduced by 71 percent of the patients

receiving daily or weekly dosing with steroids.

-- Seven patients were able to eliminate steroids completely

while participating in the trial.

-- Seventy-seven percent of the patients who reduced or

eliminated steroid use during the trial met the ACR definition

of response while steroid use was being tapered or eliminated.

The study concluded that the safety of the treatment and the response of patients who previously had been inadequately controlled with multiple conventional drugs are encouraging and suggest that Oralgam should be evaluated further in longer-term, dose-ranging clinical trials.

The complete abstract of the study results, "Oral Human Immunoglobulin in Juvenile Rheumatoid Arthritis (JRA)," is available at www.rheumatology.org/annual/abstracts.

Protein Therapeutics has an approved Investigational New Drug (IND) application with the U.S. Food and Drug Administration for the use of Oralgam in the treatment of juvenile rheumatoid arthritis. The FDA also has granted orphan drug designation to Oralgam to help facilitate its development for the treatment of JRA. Currently, Oralgam is the only drug being developed specifically to treat JRA. The established safety of human immunoglobulin allows the drug to be tested in children without first having to be tested in adults.

Oral administration of human immunoglobulin, as Oralgam, is being studied as a new and unique approach to treating JRA and other autoimmune diseases. Instead of suppressing the body's immune system as current therapies do, oral immunoglobulin, prepared from pooled normal human donor plasma, supplements the body's immune system.

The ACR/ARHP Annual Scientific Meeting, at which the study results will be presented, is the premier scientific meeting devoted to rheumatic diseases. The American College of Rheumatology (ACR) and the Association of Rheumatology Health Professionals (ARHP), a division of ACR, are professional organizations whose members are dedicated to healing, preventing disability and curing more than 100 types of arthritis and related disorders.

About Protein Therapeutics Inc.

Protein Therapeutics, a privately held pharmaceutical company, is dedicated to the development of effective therapies for autoimmune diseases. The company's focus is on antibody products that are safe for the patient, free of adverse reactions and easily administered. Clinical trials are planned or in process for Crohn's disease, ulcerative colitis, autistic enterocolitis, dermatomyositis and rheumatoid arthritis. Additional information about Protein Therapeutics is available at www.protein-therapeutics.com.

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