DermatomyositisNCT00017914
Adult and Juvenile Myositis
An observational study of Dermatomyositis and Polymyositis, run by National Institute of Environmental Health Sciences (NIEHS) at 4 sites in United States.
Trial 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. Read more about this study
- Who is it for?
- Ages 2 to 100 yearsHealthy volunteers can take part
- Conditions the study is for
- Dermatomyositis, Polymyositis, Inclusion Body Myositis
- How many people
- 1,200 participants
- Where
- 4 sitesUnited States
- Study dates
- 7 Jun 1995to not given
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 2 to 100 years
- All sexes
- Healthy volunteers accepted
- Child, Adult, Older adult
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/non-inflammatory myopathies, mimicking and related skin disorders, disorders of 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- Close relatives of patients with at least one first-degree relative affected with IIIM (affected or unaffected siblings, children, parents, grandparents) can participate in the genetics portion of the protocol; 4- Healthy control subjects.
Who may be able to join
- All patients should have age range 2-100 years.
- 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:
- Patient has documented evidence that he/she meets criteria for an idiopathic inflammatory myopathy
- Possible, probable or definite adult or juvenile polymyositis or dermatomyositis by Bohan and Peter criteria
- Possible, probable or definite inclusion body myositis by Griggs and/or ENMC criteria
- 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.
Show all 23Show fewer
- 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
- 3. Ability of patient or parent/guardian to give informed consent to all or part of the study after full information has been provided.
- First or more distant relative of a proband with myositis, for genetics studies.
- Ability of patient or parent/guardian to give informed consent to all or part of the study after full information has been provided.
- Healthy volunteer subjects for biomarker studies will be gender and age-matched (within 5 years) with a myositis patient, as reasonably close as possible.
- Volunteer is not related to a myositis patient who is enrolled in the study
- Volunteer is in good health, without a recognized systemic rheumatic disorder, autoimmune disease, immune medicated disease, or cancer, and is not taking any anti-inflammatory medicines, including nonsteroidal anti-inflammatory drugs (NSAIDS) or corticosteroids
- Volunteer or volunteer s parent/guardian could give assent/informed consent to all or part of the study after full information has been provided
Who cannot join
- Patients <2 years or >100 years will be excluded.
- 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.
- For females of childbearing potential who are pregnant, they will be permitted to enroll, but would be excluded from radiographic procedures involving radiation or greater than minimal risk procedures, including obtaining biopsies.
- Relatives of Patients with Myositis:
- 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. For patients in which two or more first degree relatives are affected with myositis, any available close relatives (affected or unaffected parents, siblings or children, as well as grandparents and grandchildren, or other close relatives, when available) are eligible to participate in the genetics portion of the protocol.
- Patients <2 years or >100 years will be excluded.
Show all 27Show fewer
- 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.
- Pregnancy (females of childbearing potential). Verbal confirmation they are not pregnant.
- Healthy Volunteers:
- If Any "Yes" answer patient is not eligible for study
- Volunteer has undergone surgery or had a major trauma within the past 2 months
- Volunteer used anti-inflammatory medications within the past 8 weeks of enrollment, including not currently receiving nonsteroidal anti-inflammatory drugs or corticosteroids
- Volunteer has been diagnosed with a chronic infectious illness, inflammatory disease, malignancy, cardiac or pulmonary disease, muscle disorder, clotting disorder, metabolic disorder, systemic rheumatic or autoimmune disease, or immune-mediated disease
- Volunteer is pregnant (verbally screened for females of childbearing potential)
- Volunteer has a medical illness that in the judgement of the investigators does not allow safe blood draws or other clinical evaluations needed for study participation
- Volunteer is cognitively impaired
- Volunteer and or the volunteer's parents/legal guardian is unable or unwilling to give informed consent/assent
- Volunteer has functional limitations
- Volunteer has joint or muscle problems
- In IMAGE Survey participants must confirm in the survey that they have a medical diagnosis of myositis.
- Note that the following screening activities may be performed to determine subject eligibility:
- Email, written, or telephone communications with prospective subjects
- Review of existing medical records
- Review of existing imaging studies
- Review of existing photographs or videos
- Review of existing pathology specimens/reports from a specimen obtained for diagnostic purposes
- If a participant is ineligible and willing, their contact information- including name, phone numbers, emails, best times to reach, and potential diagnosis- may be shared with other NIH investigators if the participant may be eligible to participate in those studies.
Trial locations and contacts
4 sites in 1 country. Contact the study team or a site near you to ask about taking part.
United States4 sites, 2 recruiting
- Completed
Emory University
Atlanta, Georgia, 30322
- Recruiting
National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
- Completed
Texas Scottish Rite Hospital
Dallas, Texas, 75219
- Recruiting
NIEHS Clinical Research Unit (CRU)
Research Triangle Park, North Carolina, 27709
About this study
Official title: Studies in the Natural History and Pathogenesis of Childhood-Onset and Adult-Onset Idiopathic Inflammatory Myopathies
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.
Read more
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:
- 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
- Skin assessment, possibly including photographs of lesions and a skin biopsy (removal of a small skin sample under local anesthetic)
- Magnetic resonance imaging (scans that use magnetic fields to visualize tissues) of leg muscles
- 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
- 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
- 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
- Electrocardiogram (measurement of the electrical activity of the heart) and, possibly, echocardiogram (ultrasound imaging of the heart)
- Endocrine evaluation
- Eye examination, in patients with vision loss or other eye symptoms
- 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.
- Muscle ultrasound.
- Electromyography (record of the electrical activity of muscles)
- 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.
Read the 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:
- 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
- Skin assessment, possibly including photographs of lesions and a skin biopsy (removal of a small skin sample under local anesthetic)
- Magnetic resonance imaging (scans that use magnetic fields to visualize tissues) of leg muscles
- 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
- 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
- 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
- Electrocardiogram (measurement of the electrical activity of the heart) and, possibly, echocardiogram (ultrasound imaging of the heart)
- Endocrine evaluation
- Eye examination, in patients with vision loss or other eye symptoms
- 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.
- Muscle ultrasound.
- Electromyography (record of the electrical activity of muscles)
- 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.
- Conditions
- Dermatomyositis, Polymyositis, Inclusion Body Myositis
- Keywords
- Adult and Pediatric Disease, Autoimmunity Pathogenesis, Disease Assessment, Genetic and Environmental Risk Factors, Myositis, Natural History
- Medical subject headings
- Dermatomyositis, Polymyositis, Myositis, Inclusion Body, Myositis
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| 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/autoimmune diseases/complications similar to myositis patients. Close relatives of IIM patients (affected or unaffected siblings, children, parents, grandparents) |
What is being measured
The primary outcome is the main question the study is designed to answer.
Physician Global Assessment Form
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.
Measured: At enrollment and each study visit
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted20 Jun 2001 (estimated)
- Study start7 Jun 1995
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (8)Papers the sponsor has linked to this study.
- Other trials for DermatomyositisStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Other
- Enrolment
- 1,200 participants (estimated)
Sponsor and investigators
- Lead sponsor
- National Institute of Environmental Health Sciences (NIEHS)
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- Lisa G Rider, M.D., Principal investigator, National Institute of Environmental Health Sciences (NIEHS)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00017914
- Sponsor's protocol ID
- 940165
- Other IDs
- 94-E-0165
- Status last verified
- 22 Sep 2026
- First submitted
- 19 Jun 2001
- Last updated
- 24 Sep 2026
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Miller FW, Lamb JA, Schmidt J, Nagaraju K. Risk factors and disease mechanisms in myositis. Nat Rev Rheumatol. 2018 Apr 20;14(5):255-268. doi: 10.1038/nrrheum.2018.48.
PubMed 29674613Miller FW, Chen W, O'Hanlon TP, Cooper RG, Vencovsky J, Rider LG, Danko K, Wedderburn LR, Lundberg IE, Pachman LM, Reed AM, Ytterberg SR, Padyukov L, Selva-O'Callaghan A, Radstake TR, Isenberg DA, Chinoy H, Ollier WE, Scheet P, Peng B, Lee A, Byun J, Lamb JA, Gregersen PK, Amos CI; Myositis Genetics Consortium. Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes. Genes Immun. 2015 Oct;16(7):470-80. doi: 10.1038/gene.2015.28. Epub 2015 Aug 20.
PubMed 26291516Rider LG, Aggarwal R, Machado PM, Hogrel JY, Reed AM, Christopher-Stine L, Ruperto N. Update on outcome assessment in myositis. Nat Rev Rheumatol. 2018 May;14(5):303-318. doi: 10.1038/nrrheum.2018.33. Epub 2018 Apr 12.
PubMed 29651119Sparling AC, Ward JM, Sarkar K, Schiffenbauer A, Farhadi PN, Smith MA, Rahman S, Zerrouki K, Miller FW, Li JL, Casey KA, Rider LG. Neutrophil and mononuclear leukocyte pathways and upstream regulators revealed by serum proteomics of adult and juvenile dermatomyositis. Arthritis Res Ther. 2024 Nov 11;26(1):196. doi: 10.1186/s13075-024-03421-7.
PubMed 39529136Burbelo PD, Huapaya JA, Khavandgar Z, Beach M, Pinal-Fernandez I, Mammen AL, Chiorini JA, Noroozi Farhadi P, Miller FW, Schiffenbauer A, Sarkar K, Warner BM, Rider LG. Quantification of autoantibodies using a luminescent profiling method in autoimmune interstitial lung disease. Front Immunol. 2024 Oct 25;15:1462242. doi: 10.3389/fimmu.2024.1462242. eCollection 2024.
PubMed 39524452Sherman MA, Noroozi Farhadi P, Pak K, Trieu EP, Sarkar K, Targoff IN, Neely ML, Mammen AL, Rider LG; Childhood Myositis Heterogeneity Collaborative Study Group. Myositis-Associated Autoantibodies in Patients With Juvenile Myositis Are Associated With Refractory Disease and Mortality. Arthritis Rheumatol. 2024 Jun;76(6):963-972. doi: 10.1002/art.42813. Epub 2024 Mar 12.
PubMed 38272842Ward JM, Ambatipudi M, O'Hanlon TP, Smith MA, de Los Reyes M, Schiffenbauer A, Rahman S, Zerrouki K, Miller FW, Sanjuan MA, Li JL, Casey KA, Rider LG. Shared and Distinctive Transcriptomic and Proteomic Pathways in Adult and Juvenile Dermatomyositis. Arthritis Rheumatol. 2023 Nov;75(11):2014-2026. doi: 10.1002/art.42615. Epub 2023 Aug 13.
PubMed 37229703Sherman MA, Yang Q, Gutierrez-Alamillo L, Pak K, Flegel WA, Mammen AL, Rider LG, Casciola-Rosen LA; Childhood Myositis Heterogeneity Collaborative Study Group. Clinical Features and Immunogenetic Risk Factors Associated With Additional Autoantibodies in Anti-Transcriptional Intermediary Factor 1gamma Juvenile-Onset Dermatomyositis. Arthritis Rheumatol. 2024 Apr;76(4):631-637. doi: 10.1002/art.42768. Epub 2024 Jan 30.
PubMed 38059274
Study at a glance
From the registry record, last updated by the sponsor on 24 Sep 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Studies in the Natural History and Pathogenesis of Childhood-Onset and Adult-Onset Idiopathic Inflammatory Myopathies
- ClinicalTrials.gov ID
- NCT00017914
- Acronym / sponsor ID
- 940165
- Conditions
- Dermatomyositis, Polymyositis, Inclusion Body Myositis
- Keywords
- Adult and Pediatric Disease, Autoimmunity Pathogenesis, Disease Assessment, Genetic and Environmental Risk Factors, Myositis, Natural History
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 1,200 participants (estimated)
- Lead sponsor
- National Institute of Environmental Health Sciences (NIEHS)US National Institutes of Health
- Study start
- 7 Jun 1995 (actual)
- First posted
- 20 Jun 2001 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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:
- 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
- Skin assessment, possibly including photographs of lesions and a skin biopsy (removal of a small skin sample under local anesthetic)
- Magnetic resonance imaging (scans that use magnetic fields to visualize tissues) of leg muscles
- 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
- 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
- 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
- Electrocardiogram (measurement of the electrical activity of the heart) and, possibly, echocardiogram (ultrasound imaging of the heart)
- Endocrine evaluation
- Eye examination, in patients with vision loss or other eye symptoms
- 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.
- Muscle ultrasound.
- Electromyography (record of the electrical activity of muscles)
- 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.
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:
- 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
- Skin assessment, possibly including photographs of lesions and a skin biopsy (removal of a small skin sample under local anesthetic)
- Magnetic resonance imaging (scans that use magnetic fields to visualize tissues) of leg muscles
- 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
- 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
- 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
- Electrocardiogram (measurement of the electrical activity of the heart) and, possibly, echocardiogram (ultrasound imaging of the heart)
- Endocrine evaluation
- Eye examination, in patients with vision loss or other eye symptoms
- 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.
- Muscle ultrasound.
- Electromyography (record of the electrical activity of muscles)
- 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.
Study design
- Observational model
- Cohort
- Time perspective
- Other
- Number of arms
- 3
| Arm | Type | Receives |
|---|---|---|
| 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/autoimmune diseases/complications similar to myositis patients. Close relatives of IIM patients (affected or unaffected siblings, children, parents, grandparents) |
Who may qualify
- Ages
- 2 to 100 years
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult, Older adult
- 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/non-inflammatory myopathies, mimicking and related skin disorders, disorders of 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- Close relatives of patients with at least one first-degree relative affected with IIIM (affected or unaffected siblings, children, parents, grandparents) can participate in the genetics portion of the protocol; 4- Healthy control subjects.
Key inclusion criteria (23 listed)
- All patients should have age range 2-100 years.
- 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:
- Patient has documented evidence that he/she meets criteria for an idiopathic inflammatory myopathy
- Has one of the following conditions:
- 2A. Have a disorder that is related to an idiopathic inflammatory myopathy, that may include:
Key exclusion criteria (27 listed)
- Patients <2 years or >100 years will be excluded.
- 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.
- For females of childbearing potential who are pregnant, they will be permitted to enroll, but would be excluded from radiographic procedures involving radiation or greater than minimal risk procedures, including obtaining biopsies.
- 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. For patients in which two or more first degree relatives are affected with myositis, any available close relatives (affected or unaffected parents, siblings or children, as well as grandparents and grandchildren, or other close relatives, when available) are eligible to participate in the genetics portion of the protocol.
- Patients <2 years or >100 years will be excluded.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
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.
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 24 Sep 2026.
- Background: PubMed 29674613Miller FW, Lamb JA, Schmidt J, Nagaraju K. Risk factors and disease mechanisms in myositis. Nat Rev Rheumatol. 2018 Apr 20;14(5):255-268. doi: 10.1038/nrrheum.2018.48.
- Background: PubMed 26291516Miller FW, Chen W, O'Hanlon TP, Cooper RG, Vencovsky J, Rider LG, Danko K, Wedderburn LR, Lundberg IE, Pachman LM, Reed AM, Ytterberg SR, Padyukov L, Selva-O'Callaghan A, Radstake TR, Isenberg DA, Chinoy H, Ollier WE, Scheet P, Peng B, Lee A, Byun J, Lamb JA, Gregersen PK, Amos CI; Myositis Genetics Consortium. Genome-wide association study identifies HLA 8.1 ancestral haplotype alleles as major genetic risk factors for myositis phenotypes. Genes Immun. 2015 Oct;16(7):470-80. doi: 10.1038/gene.2015.28. Epub 2015 Aug 20.
- Background: PubMed 29651119Rider LG, Aggarwal R, Machado PM, Hogrel JY, Reed AM, Christopher-Stine L, Ruperto N. Update on outcome assessment in myositis. Nat Rev Rheumatol. 2018 May;14(5):303-318. doi: 10.1038/nrrheum.2018.33. Epub 2018 Apr 12.
- Background: PubMed 39529136Sparling AC, Ward JM, Sarkar K, Schiffenbauer A, Farhadi PN, Smith MA, Rahman S, Zerrouki K, Miller FW, Li JL, Casey KA, Rider LG. Neutrophil and mononuclear leukocyte pathways and upstream regulators revealed by serum proteomics of adult and juvenile dermatomyositis. Arthritis Res Ther. 2024 Nov 11;26(1):196. doi: 10.1186/s13075-024-03421-7.
- Background: PubMed 39524452Burbelo PD, Huapaya JA, Khavandgar Z, Beach M, Pinal-Fernandez I, Mammen AL, Chiorini JA, Noroozi Farhadi P, Miller FW, Schiffenbauer A, Sarkar K, Warner BM, Rider LG. Quantification of autoantibodies using a luminescent profiling method in autoimmune interstitial lung disease. Front Immunol. 2024 Oct 25;15:1462242. doi: 10.3389/fimmu.2024.1462242. eCollection 2024.
- Background: PubMed 38272842Sherman MA, Noroozi Farhadi P, Pak K, Trieu EP, Sarkar K, Targoff IN, Neely ML, Mammen AL, Rider LG; Childhood Myositis Heterogeneity Collaborative Study Group. Myositis-Associated Autoantibodies in Patients With Juvenile Myositis Are Associated With Refractory Disease and Mortality. Arthritis Rheumatol. 2024 Jun;76(6):963-972. doi: 10.1002/art.42813. Epub 2024 Mar 12.
- Background: PubMed 37229703Ward JM, Ambatipudi M, O'Hanlon TP, Smith MA, de Los Reyes M, Schiffenbauer A, Rahman S, Zerrouki K, Miller FW, Sanjuan MA, Li JL, Casey KA, Rider LG. Shared and Distinctive Transcriptomic and Proteomic Pathways in Adult and Juvenile Dermatomyositis. Arthritis Rheumatol. 2023 Nov;75(11):2014-2026. doi: 10.1002/art.42615. Epub 2023 Aug 13.
- Derived from this study: PubMed 38059274Sherman MA, Yang Q, Gutierrez-Alamillo L, Pak K, Flegel WA, Mammen AL, Rider LG, Casciola-Rosen LA; Childhood Myositis Heterogeneity Collaborative Study Group. Clinical Features and Immunogenetic Risk Factors Associated With Additional Autoantibodies in Anti-Transcriptional Intermediary Factor 1gamma Juvenile-Onset Dermatomyositis. Arthritis Rheumatol. 2024 Apr;76(4):631-637. doi: 10.1002/art.42768. Epub 2024 Jan 30.
- NIH Clinical Center Detailed Web PageLink provided by the sponsor.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Lisa G Rider, M.D. principal investigator
National Institute of Environmental Health Sciences (NIEHS)
4 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Dermatomyositis and Polymyositis, run by National Institute of Environmental Health Sciences (NIEHS) at 4 sites in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 23 inclusion and 27 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 24 Sep 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 4 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Lisa G Rider, M.D. ((301) 451-6272, riderl@mail.nih.gov), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Physician Global Assessment Form (time frame: At enrollment and each study visit). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00017914, which also lists 8 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 24 Sep 2026.