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Determining Disease Activity Biomarkers in Individuals With Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)

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. Identifier: NCT00315380
Recruitment Status : Recruiting
First Posted : April 18, 2006
Last Update Posted : May 9, 2019
Office of Rare Diseases (ORD)
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Rare Diseases Clinical Research Network
Information provided by (Responsible Party):
Peter Merkel, University of Pennsylvania

Brief Summary:
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) is a rare immune system disorder that causes asthma, an excessive number of eosinophils (a type of white blood cell) in the blood, and the inflammation of blood vessels, or vasculitis. In order to properly treat EGPA, it is critical that the level of disease activity can be determined over the course of the disease. The purpose of this study is to determine new biological markers, or biomarkers, that may be used to assess the severity of this disease in people with EGPA.

Condition or disease
Eosinophilic Granulomatosis With Polyangiitis Churg-Strauss Syndrome

Detailed Description:

EGPA, also known as allergic granulomatosis angiitis, is a systemic vasculitis. EGPA is marked by three distinct symptoms: asthma; eosinophilia, evidenced by an excessive number of eosinophils in the blood and tissues; and vasculitis involving the skin, lungs, nerves, kidneys, and other organs. Nerve involvement may also occur in EGPA, causing pain, tingling, numbness, and muscle wasting in the hands and feet. Because EGPA patients may not show any visible signs of active disease, current methods of monitoring disease progression usually represent a period of extended inflammation and disease activity. Thus, patients may go untreated during a period of undetectable disease when damage might be preventable. This study will use novel scientific methods to identify new biomarkers that can be used to monitor disease activity in EGPA patients. These biomarkers may be used to help direct clinical care for EGPA patients and assist in future drug development.

Study visits will occur monthly for the first year, then every 3 months thereafter for the remainder of the study. Blood and urine collection will occur at every visit. A physical exam and medical and medication history will occur every 3 months; also, participants will be asked to complete several questionnaires to assess disease activity, health status, and tobacco, alcohol, and drug use. Participants may have additional study visits if a disease flare or disease-related complications occur during the study.

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Study Type : Observational
Estimated Enrollment : 500 participants
Observational Model: Cohort
Time Perspective: Prospective
Official Title: Longitudinal Protocol for Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)
Study Start Date : April 2006
Estimated Primary Completion Date : April 2020
Estimated Study Completion Date : April 2020

Primary Outcome Measures :
  1. Discover biomarkers in EGPA capable of measuring disease activity and response to treatment [ Time Frame: Study completion ]

Secondary Outcome Measures :
  1. Measure the predictive value of biomarkers for clinical outcome in EGPA [ Time Frame: Study completion. ]

Biospecimen Retention:   Samples With DNA
Blood (serum and plasma), urine, and DNA

Information from the National Library of Medicine

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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Individuals with eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Enrollment will be sequential and participants will have disease in various stages and of different duration.

Inclusion Criteria:

  • Documented evidence of small vessel vasculitis and at least 4 of the following 6 American College of Rheumatology (ACR) criteria for the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss):

    1. Asthma
    2. Peak peripheral blood eosinophilia of greater than 10% of total white blood cell count
    3. Peripheral neuropathy attributable to vasculitis
    4. Transient pulmonary infiltrates on chest imaging studies
    5. Paranasal sinus abnormalities or nasal polyposis
    6. Eosinophilic inflammation on tissue biopsy
  • Parent or guardian willing to provide informed consent, if applicable

Exclusion Criteria:

  • Granulomatosis with polyangiitis (Wegener's)
  • Microscopic polyangiitis
  • Drug-induced vasculitis
  • Hypereosinophilic syndrome
  • Sarcoidosis
  • Infectious forms of vasculitis
  • Takayasu's arteritis
  • Giant cell arteritis
  • Cogan's syndrome
  • Behcet's disease
  • Kawasaki disease
  • Cryoglobulinemic vasculitis
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Mixed connective tissue disease or any overlapping autoimmune syndrome

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 identifier (NCT number): NCT00315380

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United States, Massachusetts
Boston University School of Medicine Recruiting
Boston, Massachusetts, United States, 02118
Contact: Naomi Amudala, NP    617-414-2512   
Principal Investigator: Paul A. Monach, MD, PhD         
United States, Minnesota
Mayo Clinic Recruiting
Rochester, Minnesota, United States, 55905
Contact: Sam Hughes   
Principal Investigator: Ulrich Specks, MD         
Principal Investigator: Steven R. Ytterberg, MD         
United States, Ohio
Cleveland Clinic Foundation Recruiting
Cleveland, Ohio, United States, 44195
Contact: Elizabeth Kisela   
Principal Investigator: Carol A. Langford, MD, MHS         
United States, Pennsylvania
University of Pennsylvania Recruiting
Philadelphia, Pennsylvania, United States, 19104
Contact: B.Minal Hatwar   
Principal Investigator: Peter Merkel, MD, MPH         
University of Pittsburgh Recruiting
Pittsburgh, Pennsylvania, United States, 15260
Contact: Laurie Hope   
Principal Investigator: Larry Moreland, MD         
United States, Utah
University of Utah Recruiting
Salt Lake City, Utah, United States, 84112
Contact: Jessica gonzalez   
Principal Investigator: Curry Koening, MD, MHS         
Canada, Ontario
St. Joseph's Healthcare Recruiting
Hamilton, Ontario, Canada
Contact: Sandra Messier    905-522-1155 ext 35873   
Principal Investigator: Nader A. Khalidi, MD         
Mount Sinai Hospital Recruiting
Toronto, Ontario, Canada
Contact: Judy Vendramini   
Principal Investigator: Simon Carette, MD         
Sponsors and Collaborators
University of Pennsylvania
Office of Rare Diseases (ORD)
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Rare Diseases Clinical Research Network
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Study Chair: Peter A. Merkel, MD, MPH University of Pennsylvania

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Responsible Party: Peter Merkel, Professor, University of Pennsylvania Identifier: NCT00315380     History of Changes
Other Study ID Numbers: VCRC5506
U54AR057319 ( U.S. NIH Grant/Contract )
First Posted: April 18, 2006    Key Record Dates
Last Update Posted: May 9, 2019
Last Verified: May 2019
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

Keywords provided by Peter Merkel, University of Pennsylvania:

Additional relevant MeSH terms:
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Granulomatosis with Polyangiitis
Systemic Vasculitis
Churg-Strauss Syndrome
Vascular Diseases
Cardiovascular Diseases
Lung Diseases, Interstitial
Lung Diseases
Respiratory Tract Diseases
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Autoimmune Diseases
Immune System Diseases
Lymphoproliferative Disorders
Lymphatic Diseases