Determining Disease Activity Biomarkers in Individuals With Churg-Strauss Syndrome
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Purpose
Churg-Strauss Syndrome (CSS) 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 CSS, 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 CSS.
| Condition |
|---|
|
Churg-Strauss Syndrome |
| Study Type: | Observational |
| Study Design: | Observational Model: Cohort Time Perspective: Prospective |
| Official Title: | Longitudinal Protocol for Churg-Strauss Syndrome |
Blood (serum and plasma), urine, and DNA
| Estimated Enrollment: | 250 |
| Study Start Date: | April 2006 |
| Estimated Study Completion Date: | April 2016 |
| Estimated Primary Completion Date: | April 2016 (Final data collection date for primary outcome measure) |
CSS, also known as allergic granulomatosis angiitis, is a systemic vasculitis. CSS 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 CSS, causing pain, tingling, numbness, and muscle wasting in the hands and feet. Because CSS 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 CSS patients. These biomarkers may be used to help direct clinical care for CSS 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.
Eligibility| Genders Eligible for Study: | Both |
| Accepts Healthy Volunteers: | No |
| Sampling Method: | Non-Probability Sample |
Individuals with Churg-Strauss Syndrome. 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 Churg-Strauss syndrome:
- Asthma
- Peak peripheral blood eosinophilia of greater than 10% of total white blood cell count
- Peripheral neuropathy attributable to vasculitis
- Transient pulmonary infiltrates on chest imaging studies
- Paranasal sinus abnormalities or nasal polyposis
- 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's disease
- Cryoglobulinemic vasculitis
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Mixed connective tissue disease or any overlapping autoimmune syndrome
Contacts and Locations| United States, Maryland | |
| The Johns Hopkins Vasculitis Center | Active, not recruiting |
| Baltimore, Maryland, United States, 21224 | |
| United States, Massachusetts | |
| Boston University School of Medicine | Recruiting |
| Boston, Massachusetts, United States, 02118 | |
| Contact: Daniel Finkel 617-414-2509 | |
| Principal Investigator: Paul A. Monach, MD, PhD | |
| United States, Minnesota | |
| Mayo Clinic | Recruiting |
| Rochester, Minnesota, United States, 55905 | |
| Contact: Sara Biorn 507-284-4862 biorn.sara@mayo.edu | |
| Principal Investigator: Ulrich Specks, MD | |
| Principal Investigator: Steven R. Ytterberg, MD | |
| United States, Ohio | |
| Cleveland Clinic Foundation | Recruiting |
| Cleveland, Ohio, United States, 44195 | |
| Contact: Katie Gartner 216-445-1397 gartnek@ccf.org | |
| Principal Investigator: Carol A. Langford, MD, MHS | |
| United States, Pennsylvania | |
| University of Pennsylvania | Recruiting |
| Philadelphia, Pennsylvania, United States, 19104 | |
| Contact: Alex Giardino 215-614-4407 Alexandra.Giardino@uphs.upenn.edu; | |
| Principal Investigator: Peter Merkel, MD, MPH | |
| University of Pittsburgh | Recruiting |
| Pittsburgh, Pennsylvania, United States, 15260 | |
| Contact: Dawn McBride, RN 412-586-3545 dlmc@pitt.edu | |
| Principal Investigator: Larry Moreland, MD | |
| United States, Utah | |
| University of Utah | Recruiting |
| Salt Lake City, Utah, United States, 84112 | |
| Contact: Julieanne Nielsen 801-585-0798 Julieanne.Nielsen@hsc.utah.edu | |
| Principal Investigator: Curry Koening, MD, MHS | |
| Canada, Ontario | |
| St. Joseph's Healthcare | Recruiting |
| Hamilton, Ontario, Canada | |
| Contact: Sandra Messier 905-522-1155 ext 35873 smessier@stjoes.ca | |
| Principal Investigator: Nader A. Khalidi, MD | |
| Mount Sinai Hospital | Recruiting |
| Toronto, Ontario, Canada | |
| Contact: Julia Farquharson 416-586-8616 JFarquharson@mtsinai.on.ca | |
| Principal Investigator: Simon Carette, MD | |
| Study Chair: | Peter A. Merkel, MD, MPH | University of Pennsylvania |
More Information
Additional Information:
Publications:
| Responsible Party: | Peter Merkel, Professor, University of Pennsylvania |
| ClinicalTrials.gov Identifier: | NCT00315380 History of Changes |
| Other Study ID Numbers: | RDCRN 5506, U54AR057319 |
| Study First Received: | April 14, 2006 |
| Last Updated: | October 23, 2012 |
| Health Authority: | United States: Federal Government |
Keywords provided by University of Pennsylvania:
|
CSS |
Additional relevant MeSH terms:
|
Churg-Strauss Syndrome Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Systemic Vasculitis Vasculitis Vascular Diseases Cardiovascular Diseases |
Granuloma Lymphoproliferative Disorders Lymphatic Diseases Autoimmune Diseases Immune System Diseases |
ClinicalTrials.gov processed this record on May 16, 2013