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Adult and Juvenile Myositis

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT00017914
Recruitment Status : Recruiting
First Posted : June 20, 2001
Last Update Posted : February 25, 2020
Sponsor:
Information provided by (Responsible Party):
National Institutes of Health Clinical Center (CC) ( National Institute of Environmental Health Sciences (NIEHS) )

Brief Summary:

This study will evaluate subjects with adult- and childhood-onset myositis to learn more about their cause and the immune system changes and medical problems associated with them. Myositis is an inflammatory muscle disease that can damage muscles and other organs, resulting in significant disability.

Children or adults with polymyositis or dermatomyositis or a related condition may be evaluated under this study. Healthy children or adults will also be enrolled as "controls," for comparison of test results.

All patients will undergo a complete history (including completing some questionnaires) and physical examination, review of medical records, and blood and urine tests. Patients may then choose to participate in an additional 1- to 5-day evaluation, which will include some or all of the following diagnostic, treatment or research procedures:

  1. Standardized muscle strength testing, range of motion of joints and walking (gait) analysis by a physiotherapist; completion of a questionnaire regarding ability to perform daily tasks
  2. Skin assessment, possibly including photographs of lesions and a skin biopsy (removal of a small skin sample under local anesthetic)
  3. Magnetic resonance imaging (scans that use magnetic fields to visualize tissues) of leg muscles
  4. Swallowing studies, including a physical examination and questionnaire on swallowing ability, studies of tongue strength, and ultrasound imaging during swallowing, and possibly, a modified barium swallow
  5. Voice and speech assessment, possibly including computerized voice analysis and laryngoscopy-analysis of the larynx (voice box) using a small rigid scope with a camera placed in the mouth to view and record vocal cord function
  6. Pulmonary function tests (measurement of air moved into and out of the lungs, using a breathing machine) to evaluate lung function and, possibly, chest X-ray
  7. Electrocardiogram (measurement of the electrical activity of the heart) and, possibly, echocardiogram (ultrasound imaging of the heart)
  8. Endocrine evaluation
  9. Eye examination, in patients with vision loss or other eye symptoms
  10. Nutrition assessment to evaluate muscle mass and muscle wasting, including tape measurements or bioelectric impedance testing, a painless procedure in which wires are attached to the extremities with a sticky paste.
  11. Muscle ultrasound.
  12. Electromyography (record of the electrical activity of muscles)
  13. Muscle or skin biopsy (removal of a small piece of muscle tissue for microscopic examination)

All patients may have only a one-time evaluation or may return for one follow-up evaluations (either the 1-day or 3- to 5-day evaluation) over a 1-year period.

Healthy children will undergo a medical history and brief physical examination; blood and urine tests; speech and swallowing studies including questionnaires and physical examination, tongue strength, and ultrasound study; and bioelectric impedance testing. Children 8 to 18 years old may also have exercise testing.


Condition or disease
Dermatomyositis Polymyositis Inclusion Body Myositis

Detailed Description:

This study will evaluate subjects with adult- and childhood-onset myositis to learn more about their cause and the immune system changes and medical problems associated with them. Myositis is an inflammatory muscle disease that can damage muscles and other organs, resulting in significant disability.

Children or adults with polymyositis or dermatomyositis or a related condition may be evaluated under this study. Healthy children or adults will also be enrolled as "controls," for comparison of test results.

All patients will undergo a complete history (including completing some questionnaires) and physical examination, review of medical records, and blood and urine tests. Patients may then choose to participate in an additional 1- to 5-day evaluation, which will include some or all of the following diagnostic, treatment or research procedures:

  1. Standardized muscle strength testing, range of motion of joints and walking (gait) analysis by a physiotherapist; completion of a questionnaire regarding ability to perform daily tasks
  2. Skin assessment, possibly including photographs of lesions and a skin biopsy (removal of a small skin sample under local anesthetic)
  3. Magnetic resonance imaging (scans that use magnetic fields to visualize tissues) of leg muscles
  4. Swallowing studies, including a physical examination and questionnaire on swallowing ability, studies of tongue strength, and ultrasound imaging during swallowing, and possibly, a modified barium swallow
  5. Voice and speech assessment, possibly including computerized voice analysis and laryngoscopy-analysis of the larynx (voice box) using a small rigid scope with a camera placed in the mouth to view and record vocal cord function
  6. Pulmonary function tests (measurement of air moved into and out of the lungs, using a breathing machine) to evaluate lung function and, possibly, chest X-ray
  7. Electrocardiogram (measurement of the electrical activity of the heart) and, possibly, echocardiogram (ultrasound imaging of the heart)
  8. Endocrine evaluation
  9. Eye examination, in patients with vision loss or other eye symptoms
  10. Nutrition assessment to evaluate muscle mass and muscle wasting, including tape measurements or bioelectric impedance testing, a painless procedure in which wires are attached to the extremities with a sticky paste.
  11. Muscle ultrasound.
  12. Electromyography (record of the electrical activity of muscles)
  13. Muscle or skin biopsy (removal of a small piece of muscle tissue for microscopic examination)

All patients may have only a one-time evaluation or may return for one follow-up evaluations (either the 1-day or 3- to 5-day evaluation) over a 1-year period.

Healthy children will undergo a medical history and brief physical examination; blood and urine tests; speech and swallowing studies including questionnaires and physical examination, tongue strength, and ultrasound study; and bioelectric impedance testing. Children 8 to 18 years old may also have exercise testing.

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Study Type : Observational
Estimated Enrollment : 1200 participants
Observational Model: Cohort
Time Perspective: Other
Official Title: Studies in the Natural History and Pathogenesis of Childhood-Onset and Adult-Onset Idiopathic Inflammatory Myopathies
Actual Study Start Date : June 8, 1994


Group/Cohort
Healthy Volunteer
Healthy subject who has not received anti-inflammatory medications and should not has undergone surgery or any major trauma within the 8 weeks prior to enrollment.
Myositis Patient
Patient should have documented evidence that he/she meets criteria for an idiopathic inflammatory myopathy (IIM)
Non-Myositis Patient
Patients with other myopathies, other autoimmune diseases, other complications similar to myositis patients. First degree relatives of IIM patients (affected or unaffected)



Primary Outcome Measures :
  1. Physician global assessment form [ Time Frame: At enrollment and each study visit ]
    1- A secure internet accessible IMACS Outcomes Data Repository of core set disease activity, damage and quality of life measures has been established for adult and juvenile myositis patients as part of IMACS. 2- Compare the clinical and immunopathogenic features of childhood and adult IIM to determine the differences of IIM in these two populations referred to NIH. 3- investigate the immunopathogenesis and immunogenetics of IIM.



Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.


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Ages Eligible for Study:   up to 100 Years   (Child, Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
1- Any patient who has documented evidence that he/she meets criteria for an idiopathic inflammatory myopathy;@@@2- Any patient who has been diagnosed with inflammatory and non-inflammatory myopathies, mimicking and related skin disorders, disorders of the complications of myositis, overlapping autoimmune diseases that may be associated with myositis, patients with muscle and/or skin inflammation and documented environmental exposures, patients with myositis or complications of myositis and suspected genetic disorders, patients with undifferentiated connective tissue disease, patients with signs or symptoms of myositis or laboratory abnormalities who do not have an established diagnosis of myositis for them to be evaluated to establish a diagnosis; @@@3- All available first-degree relatives (affected and unaffected)of patients with at least one first-degree relative affected with IIIM, are eligible to participate in the genetics portion of the protocol; @@@4- Healthy control subjects.
Criteria
  • INCLUSION CRITIERA:

All Patients admitted to the study must satisfy at least one of the following criteria among item 1 (A or B or C) OR item 2 (A or B or C) AND item 3:

  1. Patient has documented evidence that he/she meets criteria for an idiopathic inflammatory myopathy

    A. Possible, probable or definite adult or juvenile polymyositis or dermatomyositis by Bohan and Peter criteria

    B. Possible, probable or definite inclusion body myositis by Griggs and/or ENMC criteria

    C. Has an idiopathic inflammatory myopathy that does not meet these criteria, including common or rarer forms of myositis such as cancer- associated, focal, orbital, eosinophilic myositis, macrophagic, proliferative, etc.

  2. Has one of the following conditions:

2A. Have a disorder that is related to an idiopathic inflammatory myopathy, that may include:

  • Inflammatory and non-inflammatory myopathies:
  • Mimicking and related skin disorders:
  • Disorders of the complications of myositis (including interstitial lung disease, calcifying disorders, cardiomyopathies, etc.):
  • Overlapping autoimmune diseases that may be associated with myositis
  • Patients with muscle and/or skin inflammation and documented environmental exposures
  • Patients with myositis or complications of myositis and suspected genetic disorders
  • Patients with undifferentiated connective tissue disease
  • Patients with signs or symptoms of myositis (such as weakness, skin rashes, interstitial lung disease) or laboratory abnormalities (such as elevated CK or muscle biopsy with myopathic features) who do not have an established diagnosis of myositis for them to be evaluated to establish a diagnosis

    2B. For patients with at least one first-degree relative affected with IIIM, all available first-degree relatives (affected and unaffected) are eligible to participate in the genetics portion of the protocol.

    2C. Healthy control subject needed for sub-studies in the protocol.

    3. Ability of patient or parent/guardian to give informed consent to all or part of the study after full information has been provided

EXCLUSION CRITERIA:

Any conditions in which the drawing of the amount of blood required or undergoing procedures needed for the study is not deemed medically appropriate by the treating physician or the principal investigator.


Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00017914


Contacts
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Contact: Lisa G Rider, M.D. (301) 451-6272 riderl@mail.nih.gov

Locations
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United States, Maryland
National Institutes of Health Clinical Center, 9000 Rockville Pike Recruiting
Bethesda, Maryland, United States, 20892
Contact: For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR)    800-411-1222 ext TTY8664111010    prpl@cc.nih.gov   
United States, North Carolina
NIEHS Clinical Research Unit (CRU) Recruiting
Research Triangle Park, North Carolina, United States
Sponsors and Collaborators
National Institute of Environmental Health Sciences (NIEHS)
Investigators
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Principal Investigator: Lisa G Rider, M.D. National Institute of Environmental Health Sciences (NIEHS)

Additional Information:
Publications:
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Responsible Party: National Institute of Environmental Health Sciences (NIEHS)
ClinicalTrials.gov Identifier: NCT00017914    
Obsolete Identifiers: NCT00001374
Other Study ID Numbers: 940165
94-E-0165
First Posted: June 20, 2001    Key Record Dates
Last Update Posted: February 25, 2020
Last Verified: January 6, 2020
Keywords provided by National Institutes of Health Clinical Center (CC) ( National Institute of Environmental Health Sciences (NIEHS) ):
Adult and Pediatric Disease
Autoimmunity Pathogenesis
Disease Assessment
Genetic and Environmental Risk Factors
Myositis
Additional relevant MeSH terms:
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Dermatomyositis
Myositis
Polymyositis
Myositis, Inclusion Body
Muscular Diseases
Musculoskeletal Diseases
Neuromuscular Diseases
Nervous System Diseases
Connective Tissue Diseases
Skin Diseases