Clinical Trial: Immune Abnormalities in Sporadic Inclusion Body Myositis

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Study of Immune Dysregulation in Patients With Sporadic Inclusion Body Myositis (s-IBM)

Brief Summary:

This study will examine the abnormal immune response in patients with sporadic inclusion body myositis (s-IBM)-the most common inflammatory muscle disease in people over the age of 50. s-IBM progresses steadily and may lead to severe weakness and wasting of arm and leg muscles. Patients may become unable to perform daily living activities and be confined to wheelchairs. s-IBM is thought to be an autoimmune disease, in which the body's own immune system attacks healthy muscles. This study will explore the causes of the muscle tissue inflammation that is responsible for destruction of muscle fibers and weakness in this disease. Information from the study may help in the development of an effective treatment for this disease.

Patients with s-IBM may be eligible for this study. Those who are unable to travel or who have severe cardiovascular, renal or other end-stage organ disease will be excluded. Candidates will be screened for eligibility with a medical history and physical and neurological examinations.

Participants will be seen at the NIH Clinical Center every six months over a 12-month period (visits at enrollment, 6 months and 12 months) either on an inpatient or outpatient basis, depending on their disease severity. Each 2- to 3-day visit will involve the following tests and evaluations:

  • Blood samples for routine laboratory tests are collected at every visit. Additional blood for research studies is collected at 12 months.
  • Quantitative muscle strength testing is done at every visit. The patient pulls against straps connected to dynamometers (devices that measure muscle power) to evaluate strength of the main muscle groups in the arms and legs.
  • Lymphapheresis is done at

    Detailed Summary: Sporadic Inclusion Body Myositis (s-IBM) is the most common acquired myopathy above the age of 50 years. It is a disabling disease that leads to wheelchair confinement. The cause is unknown but autoimmune mechanisms have been implicated. The primary goal of the study is to search for candidate autoantigen(s) that drive the autoimmune response and identify candidate pathogenic T-cell epitopes among the endomysial T-cells extracted from the patients' muscle biopsies. It is anticipated that the search for the antigens implicated in the clonal expansion of endomysial T-cells over time will elucidate the autoimmune pathogenesis of the disease and lead to the development of antigen-specific immunomodulatory therapeutic strategies. This is an investigative study intended to better define the pathogenesis of s-IBM. No new therapy will be provided except of standard care.
    Sponsor: National Institute of Neurological Disorders and Stroke (NINDS)

    Current Primary Outcome:

    Original Primary Outcome:

    Current Secondary Outcome:

    Original Secondary Outcome:

    Information By: National Institutes of Health Clinical Center (CC)

    Dates:
    Date Received: February 7, 2002
    Date Started: February 4, 2002
    Date Completion: December 10, 2007
    Last Updated: January 24, 2017
    Last Verified: December 10, 2007